Dear SirGastroschisis is an isolated structural anomaly rarely associated with other life-threatening anomalies. The most common associated gastrointestinal anomalies are intestinal atresia, Meckel's diverticulum and intestinal duplications. Intestinal atresia occurs in 10-15% of gastroschisis cases. [1] We herein report a case of gastroschisis with concomitant type III b jejuno-ileal atresia with perforation of the proximal dilated segment of jejunum with congenital short bowel successfully treated by primary anastomosis and primary abdominal closure.A 7-hours-old male infant born to a healthy 20-year-old mother was referred to our institution for definitive treatment by the regional hospital. Baby was delivered by emergency Caesarean section due to fetal bradycardia at 36 weeks gestation. An Apgar score 1/5 was >7, whereas birth weight was 3200 grams. Family history was showed no case of gastroschisis. The clinical examination showed a gastroschisis with the entire stomach and the mid-gut herniating through the full thickness abdominal wall defect, just right of the umbilicus . No other severe congenital anomalies were detected. Initial resuscitation in the form of ongoing fluid correction and heat losses of exposed bowel and subsequent metabolic disturbance was conducted preoperatively. Perforation of the dilated loop of proximal jejunum which ended blindly was observed during the surgery .Atresia of the jejunum was located 25 cm distal to duodeno-jejunal junction. Further exploration found ileal atresia 65 cm proximal to ileo-cecal valve. In our case of type III b jejuno-ileal or "apple peel" atresia, the total length of small intestine was 90 cm . Primary jejuno-ileal end to end anastomosis without liberal resection of the proximal bulbous jejunal segment and primary closure of the anterior abdominal wall with preservation of the umbilicus were performed. Postoperatively, patient was admitted in our NICU. The baby was treated with gastric decompression and total parenteral nutrition. After the restoration of enteral function, following the 13th postoperative day, low-volume enteral nasogastric tube feeding was initiated, with a gradual increase until full oral feeding was established in the twenty-sixth postoperative day. Precautionary, before each feeding gastric aspiration was performed. The baby was discharged on 31st day.Treatment of patients with gastroschisis associated with intestinal atresia is a huge and serious challenge. The choice of adequate surgical treatment is controversial and largely depends on the assessment of reactive change of the intestine. Ideal treatment of such anomalies would consist of safe primary closure of the an-terior abdominal wall with the establishment of the intestinal continuity by primary anastomosis. Unfortunately, this scenario is rarely feasible. [2] Alternative treatment options may consist of primary, delayed primary, or staged silo closure of the anterior abdominal wall by leaving the atresia undisturbed or creating a stoma with the correction of intestinal atresia after few weeks. [3] Restoration of intestinal continuity by primary anastomosis with primary abdominal closure in our case was technically feasible and supported by parenteral nutrition in the postoperative course, all of which provided an excellent outcome. Even without the liberal resection of the proximal bulbous jejunal segment, in order to preserve the length of the proximal jejunum, intestinal peristalsis was established within time-frame that was not sig-nificantly longer than the establishment of peristalsis in patients with gastroschisis without intestinal atresia.The management neonates with gastroschisis associated with intestinal atresia has improved in recent decades due to improvement of surgical technique, improved quality and organization of Neonatal Intensive-Care Unit (NICU), use of total parenteral nutrition (TPN) as well as the profiling of neonatal anesthesia as a separate sub-discipline. With the aforementioned appropriate treatment, the survival rate in infant with gastroschisis associated with intestinal atresia is higher than 90% but the average rate of morbidity in these patients is significantly higher.Source of Support: NilConflict of Interest: None


Ewing's sarcoma (ES) is an uncommon round cell tumor with an aggressive course affecting mainly children and young adults. Only 1% of cases is reported with jaw involvement and have mandibular predilection. Radiographic finding in ES reflect many destructive nature of the lesion, like osteolysis, cortical erosion, periostitis and soft tissue mass. A case of ES of the mandible is reported with special consideration to the radiological appearance.How to cite this article: Krishna KBB, Thomas V, Kattoor J, Kusumakumari P. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up. Int J Clin Pediatr Dent 2013;6(2):109-114. ES is a poorly differentiated tumor of uncertain histogenesis and aggressive biologic behavior, and has emerged as one of most common sarcoma of childhood.2 The basic radiographic finding in ES is predominantly permeative osteolysis with a soft tissue mass. ES can mimic most malignant and a few benign entities of bone and referred as ‘great imitator of bone pathology’. This paper reports a case of ES of the mandible in a 3.5 years old girl, highlighting pre- and post-treatment radiological features.Tumors exhibiting neuroectodermal differentiation occur throughout the body, and the diverse tissues of the head and neck gives rise to a wide assortment of these neoplasms. Neuroectodermal neoplasms may be divided into lesions showing primarily epithelial differentiation (group I) and a more diverse group of nonepithelial neoplasms with predominantly neural features (group II) includes Ewing's sarcoma (ES).A 3.5 years old female child was brought to department of oral medicine and radiology with a complaint of mobility of right lower back tooth of 1 week duration. There was no history of toothache, trauma or associated symptoms. Extraoral examination revealed an irregular lobulated, nontender bony hard swelling of size 3 × 3.5 cm with uniformly blending borders on buccal aspect of right side body and angle of mandible . Lower bIntraorally, right lower gingivobuccal sulcus and floor of mouth obliterated due to swelling from 84 region to retromolar region . An irreOcclusal and panoramic radiographs revealed ill-defined lytic lesions involving premolar-molar region of right side body of mandible, extending from 83 to angle of mandible. Cortex and periosteum was characterized by erosion, thinning and discontinuity on buccal cortex; radiating spicules on lingual cortex; irregular thinning of lower cortical plate seen without considerable expansion. Multiple irregular patchy radiolucent areas in internal structure of permeative and moth eaten pattern with size varying from 0.5 to 2 cm was noted in internal structure . ConsideTrucut needle biopsy showed spicules of bone with a cellular cytoplasm composed of round cells with scanty cytoplasm and pleomorphic round or oval nuclei in sheets and sinusoidal pattern . ImmunohPatient underwent radiotherapy and adjuvant chemotherapy treatment with vincristine, cyclophosphamide, etoposide and mesna in Regional Cancer Centre, Thiruvananthapuram, Kerala. One year follow-up clinical review showed bony hard nontender diffuse expansion of right side mandible without apparent soft tissue swelling extraorally . IntraorRadiological follow-up using occlusal and panoramic radiography after 1 year of treatment showed mixed radiodensity on premolar-molar region and anterior border of ramus of right side of mandible involving 82 to 47. Intact and visible uniform expansion with cortical regeneration was seen throughout the lower border of the mandible near the lesion. Cortical break in buccal aspect remodeled with overzealous cancellous bone apposition resulted in an increased buccolingual width. Periosteal reactions including vertical spiculations completely resolved and cortical out line re-established in lingual aspect . Conside3ES first described by James Ewing who was a pioneer in the field of cancer research, in 1921. ES is related to the PNET, sharing a common karyotype translocation t in approximately 90% of these tumors. This translocation results in juxtaposition of the ENS and the FLI1 genes. The current research found that both ES and PNET showed similar translocations and is considered to be the ends of a histological spectrum of ‘Ewing's family of tumors’ (EFT).47 ES has a predilection for the male sex . Peak incidence is during the second decade of life; although 20 to 30% of cases are diagnosed in the first decade. The age of the patient is important diagnostically. When confronted with patients older than 30 years, the clinician must first eliminate other small round-cell tumors, including small-cell carcinoma and large-cell lymphoma, before making a diagnosis of ES. In patients younger than 5 years, the possibility of metastatic neuroblastoma or acute leukemia needs to be ruled out.5 An extensive review of the literature shows only few cases of ES affecting children under 5 years of age.6 In the case, a 3.5 years old female child represented an uncommon case of ES with lower extreme of age at the time of diagnosis.ES accounts for approximately 6% of all malignant bone tumors. Approximately 4% of ES have arisen in the bones of the head and neck, with 1% occurring in the jaws. When the jaws are involved, the predilection is for the ramus of the mandible, with few cases reported in the maxilla. Posterior parts favored over anterior parts. Because ES has a propensity to metastasize to other bones, the possibility that jawbone involvement represents metastatic disease from another skeletal site should always be considered.7 Other systemic symptoms and signs include fever, anemia and nonspecific signs of inflammation, such as increases in ESR, moderate leukocytosis and an increase in serum LDH. Conventional blood, serum and urine tests cannot specifically identify ES.5 In this case, tooth mobility was the first symptom with a progressive painless swelling. Patient was afebrile and hematological findings showed a lymphocyte predominant smear and a mild eosinophilia.The common presenting signs and symptoms in maxillofacial region includes swelling, pain, loose teeth, paresthesia, ulceration, trismus and toothache. Extraoral features reported are exophthalmos, ptosis, epistaxis, otitis media and sinusitis.5 According to Hofer et al lytic malignant diseases in the jaws manifests mainly as moth eaten and permeative patterns. ES can mimic most malignant and a few benign entities of bone, such as osteomyelitis, eosinophilic granuloma, giant cell tumor, simple bone cyst, aneurysmal bone cyst. McCormack et al referred ES as the ‘great imitator of bone pathology’ due to its clinical presentations.8The basic radiographic finding in ES reflect the aggressive nature of the lesion, including osteolysis, cortical erosion, periostitis and soft tissue mass.16Features of ES of mandible reported by various authors in articles from year 2000 searched manually and electronically using PubMed are depicted in In our case, radiological features noted include multiple ill-defined lytic medullary lesions, cortical erosion and disruption without considerable expansion, effects on adjacent anatomical structures, periosteal reactions and an associated soft tissue mass. Tumor-related osteolysis and periosteal reactions suggest a diagnosis of primary malignant tumor.Permeative destruction is a major feature of ES seen in approximately 90% of cases and ranges from pin head sized holes, moth eaten, rotten wood, geographic or nearly purely lytic pattern with or without associated sunray spicules of the periosteal bone. Ill-defined lytic patterns seen in this case include moth eaten and permeative pattern which represent poorly defined areas of medullary destruction. Buccal cortical erosion extending from medullary destruction and disruption without considerable expansion in this case are seen almost exclusively in rapidly destructive malignant disease and in some cases of metastatic disease. Destructive osteolytic lesion of ES should be differentiated radiologically from osteosarcoma, neuroblastoma, lymphosarcoma, histiocytosis-X, osteomyelitis, rhabdo-myosarcoma and metastatic carcinoma.7 Periosteal reactions are the reactive osteogenesis of the periosteum, are caused by extraosseous extension of the tumor. Several types of periosteal reactions have been observed in ES: (i) an ‘onion-peel appearance’ is a multilayered reaction, (ii) a ‘sunburst’ or ‘spiculae’ pattern is a perpendicular reaction, while (iii) ‘Codman's triangle’ is a triangular lifting of the periosteum from the bone at the site of detachment. Sun-ray like vertical spiculations and supraperiosteal soft tissue mass were noted in this case.710Wood et al in a review of 105 cases reported laminar periosteal response like onion-peel appearance was not a common feature of ES in jaw bones.717 Current case revealed missing permanent tooth germ, displacement, root resorption of deciduous teeth and absence of inferior alveolar canal outline of premolar-molar region.Radiological findings of ES on adjacent anatomical structures include displacement or destruction of unerupted tooth follicles, advanced eruption, loss of lamina dura, widespread periodontal ligament space. Root resorption, too much eruption for amount of root formation, widened canal, osteosclerosis, antral clouding, etc. were also noted in literature.Presence of soft tissue mass, age of the patient, hematological investigations and radiology can facilitate differential diagnosis, histopathology and immuno-histochemistry enables final diagnosis.18EFT cells show membranous expression of CD99 or MIC2 on immunohistochemistry. Antibody against FLI1, which is centered in the nucleus of the tumor cells, has been shown to be specific for EFT. Depending on the degree of neuroectodermal differentiation, the tumor cells may also express neuron-specific enolase (NSE), synaptophysin and S-100 protein. Immunohistochemistry is essential as the family of small round cell tumors is rather large and includes non-Hodgkin lymphoma, neuroblastoma, rhabdo-myosarcoma, mesenchymal chondrosarcoma, retino-blastoma (Rb), and desmoplastic small round cell tumor (DSRCT). Other tumors can also show small round cells and include osteosarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor and melanoma. Although CD99 shows strong membrane positivity in EFT, it can also be positive in other tumors like lymphoblastic lymphoma, rhabdomyosarcoma, synovial sarcoma, mesenchymal chondrosarcoma, Wilms tumor and rarely in DSRCT. Hence, a panel of immunohistochemical stains is employed to arrive at a definitive diagnosis.15 Current case was treated with curative chemotherapy and review of the patient after 1 year did not show any abnormal findings.Treatment of ES has undergone significant changes by utilization of integrated therapies, including chemotherapy, radiation therapy and surgery, has led to an impressive improvement in prognosis. Poor prognostic factors are patients below 10 years of age, pelvic lesions, presence of metastasis, presence of systemic symptoms, large tumor volume, high mitotic rate, filigree pattern in histopathological sections and poor response to chemotherapy. ES of the mandible has got better prognosis than long bones since facial sites are diagnosed earlier.A case of ES in a young child highlighting diagnostic radiological features in jaws and after 1 year post-treatment follow-up was reported. Familiarity with radiological findings will enable the clinician to rule out more common inflammatory lesions and narrow down differential diagnosis. Early diagnosis and judicious management ensures good prognosis for this enigmatic malignancy.


Hepatocellular carcinoma (HCC) is a primary malignancy of the liver with up to half of cases suffering from extrahepatic metastasis in the later stages of the disease. Commonly reported and encountered metastatic sites include the lymph nodes, lung, bone, and adrenal glands. This is an effort to throw a spotlight on a rare case of metastatic HCC which presented to us as two distinct lesions in the nose. It focuses on the presentation and the steps that were taken to reach this rare and unusual diagnosis. It sparks interest from a clinical and histopathology perspective. Our cynosure is the findings of the case coupled with a probe on the possible routes of spread of HCC to sinonasal region. Sinonasal metastasis is extremely rare although it is reported several times in literature. However, in the case we present, the pathology was unknown and the diagnosis of a silent HCC in the liver came as a surprise to both the patient and the caregivers. Therefore, we aim to highlight the diagnostic dilemma of this case from clinical and histopathological point of view. We also hope to enlighten the readers about the approach that was taken by the histopathologist to confirm the diagnosis. We strongly felt that reporting this case with a literature review was warranted to include exploration of the existing theories of the modes of spread of HCC to the sinonasal region.A 58-year-old male presented to our otorhinolaryngology and head and neck surgery clinic with a recurring left sided nasal skin lesion and a right sided nasal septal mass. The two lesions were rapidly growing over the preceding 3 months and bled with minor trauma. It is pertinent to mention that the patient had no chronic illnesses and had no alarming social habits including smoking or excessive alcohol consumption. Prior to presenting at our department, the nasal skin lesion was treated multiple times with cryotherapy. On examination, the patient was conscious and alert with stable vitals and no signs of acute distress. General physical examination revealed no pallor, jaundice, cyanosis, clubbing, or edema. Cardiopulmonary examination was unremarkable. The abdomen was soft and nontender with no palpable hepatosplenomegaly or obvious stigmata of chronic liver disease. Neurological and musculoskeletal systems were intact. However, 0.5 × 0.5 cm sessile pinkish lesion  on the sHistopathology conducted on both the specimens showed a similar picture of malignant neoplasm composed of sheets and nest of cells divided by a delicate capillary network. Neoplastic cells were of medium-to-large size with abundant cytoplasm and fairly round uniform nuclei with prominent nucleoli. Intranuclear inclusions were also identified. Mitosis was frequently noted. The tumor had a circumscribed pushing margin covered by epidermis. Immunohistochemical study with appropriate controls was performed and showed that the neoplastic cells were positive for cytokeratin AE1/cytokeratin AE3, HepPar1, arginase, CD10 , CEA polyclonal , and CE138. The tumor was negative for TTF-1, Vimentin, CK20, CK7, p63, CK34BE12, HMB45, synaptophysin, S100, Melan-A, inhibin, PSA, CK19, alpha-1-fetoprotein, and RCC . Final iBased on the histopathological findings, extensive laboratory workup ensued. Complete blood count with peripheral smear, electrolytes, urine analysis, and renal function tests and lipid profile and most importantly liver function tests and serum alpha fetoprotein (AFP) were within normal limits. An abdominal ultrasound followed by MRI was requested. Results showed a cirrhotic liver with large 5 × 15 cm vascular lesion with MR characteristics suggestive of a neoplastic process, most likely hepatocellular carcinoma. The hepatic and portal vessels showed either partial or complete filling defects, all suggestive of tumor invasion. An ultrasound-guided liver biopsy revealed a diagnosis of well differentiated hepatocellular carcinoma on top of chronic hepatitis grade III/grade IV, stage III/stage IV. A whole body FDG PET scan was performed in search for distant metastasis. Multiple lung and subcutaneous nodules with moderate uptake were detected suggestive of metastasis. Interestingly, the primary HCC did not show uptake on the PET CT scan.A multidisciplinary team plan was set for hepatic artery embolization and systemic therapy with sorafenib as well as palliative radiotherapy to the nasal area. To date, 12 months after the initial nasal lesions were excised, the patient has had no signs on recurrence of the lesions in the nose. He is being managed by the oncology and palliative care teams with regular 3-monthly imaging surveillance. A good response to therapy of both the primary and the metastatic tumors has been noted primarily because the tumors have not increased in size and no new growths have been noted. The patient clinical and general condition remain stable.Hepatocellular carcinoma (HCC) remains a global health concern. It is believed to be the second most common cause of cancer-related mortality worldwide after lung cancer and responsible for approximately three-quarters of a million deaths annually . Cases a Aspergillus growing on crops, which is amongst the most carcinogenic substances known [In South Asia, the rate of HCC is 7.6 per 100,000 persons per year for males and 2.8 for females. Most of the patients present in their 5th and 6th decade. Hepatitis B virus (HBV) is the main causative agent for HCC in many Asian Pacific countries although in South Asia hepatitis C virus (HCV) is the definitive contributor, seen in 60–70% of all cases. This can be attributed to the prevalence of HCV in the population, with 4.8% of general population positive for anti-HCV antibody which is closely followed by the HBV surface antigen at 2.5%. The high prevalence of HCC is also attributed to high contamination rates with aflatoxin, a toxin produced byes known . In MiddApproximately 50% of HCC patients will suffer from extrahepatic metastasis, the most frequent sites being the lung, regional lymph nodes, adrenal glands, and bone . MetastaThe pathogenesis of distant metastasis of HCC to the head and neck region remains obscure but one proposed route is the spread via the hepatic veins to caval venous system through the pulmonary circulation and then into arterial vessels feeding the head and neck. Another explanation is by retrograde spread via the prevertebral and vertebral venous plexus. Increases in intra-abdominal pressure can squeeze the tumor cells into the prevertebral and vertebral venous plexus, which communicate at the foramen magnum with the venous systems of the skull base as the cavernous sinus and pterygoid plexus and eventually into sinonasal region , 7.For management of sinonasal metastatic lesions, various modalities have been reported. They include palliative radiotherapy, systemic chemotherapy, and transcatheter embolization to control nasal bleeding. Surgical intervention of metastatic septal lesion has been also reported as a simple modality to control local symptoms .This report aims to sensitize the otorhinolaryngologist and head and neck surgeon and histopathologist to keep metastatic HCC on their differential list while investigating and managing such a case. HCC has the ability to spread to any region of the body without prior warning of a liver disease or pathology. The histopathologist requires a high index of suspicion to diagnose a case of HCC with no supporting clinical information.


The use of point-of-care ultrasound for the diagnosis of bowel obstructions and hernias is becoming increasingly common in the emergency department (ED). Using a relatively rare case of an incisional port hernia, we demonstrate the ultrasound findings of a strangulated hernia causing a partial small bowel obstruction. A 46-year-old female presented four days following a laparoscopic surgery complaining of abdominal pain, nausea and lack of bowel movements. There was a palpable mass in the left lower quadrant under the 12mm trocar port incision. ED point-of-care ultrasound revealed herniated akinetic loops of bowel through her laparoscopy incision. This is the first case report to describe the use of point-of-care ultrasound for the diagnosis of a strangulated incisional port hernia at the bedside. Incisional hernias are a well described surgical complication and a common emergency department (ED) presentation. Despite the predominance of laparoscopic surgery, port hernias remain a rare complication with an incidence as low as 0.14% to 6%.The role of point-of-care ultrasonography (POCUS) in the ED management of abdominal pain, including hernias and small bowel obstruction is growing. Multiple studies have demonstrated sensitivities of 93.9–97.7% and specificities of 81.4–92.7% for the diagnosis of SBO using POCUS in the ED setting.A 46-year-old woman presented to the ED with left lower quadrant pain associated with nausea and anorexia worsening over the last 48 hours. The pain was constant and progressive and worsened with movement. Four days earlier the patient had undergone a laparoscopic Burch colposuspension for stress incontinence and pelvic organ prolapse. The surgical notes were unremarkable and her post-operative course was uncomplicated. She was discharged home on post-operative day one.On arrival, she was tachycardic at 110 beats per minute and normotensive at 117/75. She was afebrile, had a normal respiratory rate, but was quite pale and had considerable difficulty transferring from the chair to the examination table. Her abdomen was generally soft, but a firm, focally tender mass was noted in the left lower quadrant. It was unclear based on the physical examination whether this mass was a seroma, hematoma, or hernia. The patient reported she was passing gas but not stool.On bedside ultrasonography by the emergency physician, dilated fluid-filled loops of bowel were visible in the mass, herniating through a 12mm port site directly under the skin . Free flBased on these results, the general surgery team was called and the decision was made to take the patient to the operating room. An interval computed tomography (CT) confirmed a small bowel obstruction due to a laparoscopic port hernia with signs of early ischemic changes secondary to strangulation of small bowel. The patient underwent an urgent laparotomy that identified purple, yet still viable, strangulated small bowel loop without full thickness necrosis. The bowel was reduced and no bowel resection was necessary.Laparoscopic port incisional hernias are uncommon, and can be difficult to distinguish from benign fluid collections such as post-operative hematomas or seromas. While most hernias are easily palpable on exam, some cases of Spigelian hernias may not be palpable and ultrasonography has been an established tool in their diagnosis.To examine for a hernia using POCUS, a high-frequency linear transducer is placed over the region of swelling or pain. For evaluation of deeper hernias, a low-frequency curvilinear transducer should be used. The region should be evaluated systematically in two orthogonal planes. On ultrasound, hernias appear as loops of bowel trapped within an echogenic sac protruding through a defect in the abdominal wall.POCUS can be used to examine for signs of bowel obstruction and strangulation within the hernia sac. A study comparing abdominal radiographs to POCUS for bowel obstruction found that abdominal radiographs had a sensitivity of 46.2% and a specificity of 66.7% when diagnostic, but were non-diagnostic 36% of the time. POCUS on the other hand was found to be 91% sensitive and 84% specific, with no non-diagnostic scans.Point-of-care ultrasound has been shown to be an effective tool for the diagnosis of bowel obstruction and hernias. In this case, timely access to emergency POCUS allowed us to quickly identify a strangulated incisional port hernia. Presence of Doppler flow does not rule out strangulation, while absence of Doppler flow is a late finding. Further studies are needed to evaluate the accuracy of POCUS findings for strangulated hernias.


Tumor-induced osteomalacia (TIO) is a rare disorder, which is commonly found in craniofacial locations and in the extremities. To the best of our knowledge, only 16 cases have been described in the spine, and this is the first report to describe a case of patient with TIO in the thoracic spine combined with a mesenchymal hamartoma which had confused the therapeutic strategies to date.111In pentetreotide scintigraphy (octreotide scan) revealed an increased uptake at the right forearm. The tumor was resected totally, and the histopathology revealed a mesenchymal hamartoma, but we noticed that hypophosphatemia was not corrected after the tumor resection. Then a whole-body magnetic resonance imaging (WB-MRI) was performed and the results revealed tumorous tissues at the right T1 vertebral pedicle. The tumor was removed with an en bloc method, and the pathology showed phosphaturic mesenchymal tumor. Follow-up at 1 year after surgery revealed no recurrence, and the serum phosphorus level of the patient was normal.We report the case of a 60-year-old patient with hypophosphatemia and presented with limb weakness. Treating with phosphate did not correct the hypophosphatemia and an Tumor-induced osteomalacia is exceedingly rare with only 16 cases in spine published in the literature. It is difficult to find and leads to years of suffering debilitating complications. In this regard, the WB-MRI is a better method to locate the real tumor. Treating with phosphate can only relieve symptoms, and a complete surgical removal remains the gold standard treatment.The online version of this article (doi:10.1186/s12957-015-0589-3) contains supplementary material, which is available to authorized users. Tumor-induced osteomalacia (TIO), also known as oncogenic osteomalacia, is a rare paraneoplastic syndrome, which is characterized by hyperphosphaturia, hypophosphatemia, and increased levels of alkaline phosphatase ,2. CliniIn this report, we illustrate the clinical presentation and therapeutic strategy of a patient with a TIO in thoracic spine. He underwent an en bloc resection to attain oncological and metabolic control. To date, about 300 cases have been described in the world’s literature , and onl111In pentetreotide scintigraphy (octreotide scan) was performed, and the result revealed a markedly increased uptake at the right forearm. The tumor was resected totally, and the histopathology revealed a mesenchymal hamartoma, but we noticed that hypophosphatemia wasn’t corrected after the tumor resection. In 2013, the patient suffered paraplegia and then a PET/CT scan was performed. Unfortunately, PET/CT scan did not locate the tumor, and thus, he underwent to a whole-body magnetic resonance imaging (WB-MRI), which revealed tumorous tissues at the right T1 vertebral pedicle.The patient, a 60-year-old man, first noticed pain in his right foot in 2007. In 2008, he came to the primary hospital presenting with low back pain, gait disturbance, and muscle weakness in the right lower limb. Radiological examination did not reveal any pathologic change in the lumbar vertebrae, whereas a bone scan revealed markedly increased bone uptake at the whole skeleton, and positron emission tomography/computed tomography (PET/CT) revealed mild increased uptake at multiple ribs and right femoral trochanter. No regular treatment was performed. Then the patient’s symptoms were progressively worsened and he became disabled. In 2010, blood examination revealed hypophosphatemia , hypocalcemia , reduced 1,25-dihydroxy vitamin D , and elevated β-C-terminal telopeptide of type I collagen (CTX) . Osteomalacia was initially diagnosed and treated with phosphate, calcium, vitamin D, and calcitriol. Although blood examination still revealed hypophosphatemia (0.55 mg/dl), symptoms got better progressively. In 2012, an The 60-year-old man was presented to our spine tumor center for consultation regarding resection of the tumor in the right T1 vertebral pedicle. On presentation to our center, the patient had signs of severe osteomalacia, and the patient’s most outstanding complaints were diffuse bone pain throughout the whole skeleton, limb weakness, hypaesthesia, and disabled walking. Physical examination revealed that motor strength was graded as 3/5 in the upper limbs and 1/5 in the lower limbs. Sensation decreased slightly throughout, with hyporeflexia. The Hoffmann sign and the Babinski sign were negative.Blood tests revealed hypophosphatemia (0.51 mg/dl), hypocalcemia (2.15 mmol/L), elevated levels of parathyroid hormone (PTH) , normal serum alkaline phosphatase (ALP) levels , and normal levels of 1,25-dihydroxy vitamin D (21.01 pg/ml).Radiological exams demonstrated osteolytic changes of the T1 right vertebral pedicle and mild collapse of the T1 vertebral body. In addition, some fractures were detected in multiple ribs. MRI revealed a tumor in the T1 vertebral body and right vertebral pedicle , or may be associated with more common processes such as renal failure . TIO is TIO can lead to debilitating complications and years of suffering. The average age of 16 cases is 48.0 (range 14 to 72) years. It occurs in any age, with preponderance around the fifth decades. Characteristically, TIO affects adults without a predilection for gender, with nine female and seven male in all the spinal TIOs. Diffuse bone pain, caused by poor bone mineralization, is the most frequent symptom in these patients .Notably, one of the most challenging aspects of TIO is to find the tumor. The occult nature of TIO delays its recognition, and in our case, we employed 6 years to identify the real tumor which may attribute to the mesenchymal hamartoma in the same patient complicating the final diagnosis. In a literature review of all the TIOs , the aveTypical laboratory findings include hyperphosphaturia, elevated ALP, and low serum levels of vitamin D3, which account for 14/15, 10/15, and 5/15 in the literature, respectively . Hypopho111In pentetreotide scintigraphy (octreotide scan), a scanning technique that detects the expression of somatostatin receptors (SSTRs) [Radiographically, it presents with bone cortical thickness, osteoporosis, and fracture . Unfortu (SSTRs) ,27. Howe (SSTRs) ,29. Howe (SSTRs) . WB-MRI  (SSTRs) ,32.The ideal treatment for TIO is complete tumor resection, and this corrects the biochemical abnormalities and remineralizes the bone substance in most cases 12/16) . The par2/16 . ThTumor-induced osteomalacia is exceedingly rare with only 16 cases in spine published in the literature. It is difficult to find and leads to years of suffering debilitating complications. In this regard, WB-MRI is one of the important options to detect the real tumor. Treating with phosphate can only relieve symptoms, and completing surgical removal remains the modality of choice.Written informed consent was obtained from each patient for publication of this study and the accompanying images.


The present study reports two cases of mandibular metastasis from hepatocellular carcinoma (HCC), including the clinical presentation, and computed tomography (CT), histopathology and immunohistochemistry results. Space-occupying lesions occurred unilaterally as an initial mandibular manifestation. HCC metastasis was confirmed by post-surgical examination, and the primary tumor was found using CT scans. Hepatitis B virus infection history and positive results of hepatitis B surface antigen, hepatitis B e-antibody, hepatitis B core antibody and hepatitis B virus pre-S1 antigen further supported the pathogenesis of HCC. Based on the clinical findings, the characteristics of the CT scans and the histopathology and immunohistochemistry results, the mechanisms of HCC metastasis and its management are also discussed. Metastasis is a step in the development of malignant tumors that is influential with regard to cancer-related patient mortality. As one of the most frequently diagnosed cancers worldwide, hepatocellular carcinomas (HCCs) are prone to metastasize to other tissues . The mosWith a rich blood supply and a specific growth-promoting microenvironment, bone tissue is the third most common target for metastasis overall, after the liver and lungs . In maliThe present study reports two cases of HCC metastatic to the mandible, without any other metastases, and describes the treatment for the initial mandibular lesion. Written informed consent was obtained from the patients.A 57-year-old male was admitted to the Affiliated Hospital of Qingdao University  in July 2005, presenting with a painless swelling in the preauricular region and numbness of the lower lip for six months. Maxillofacial asymmetry was observed, and the immobile tumor could be localized by palpation in the right subaural and preauricular areas, in the absence of tenderness. Intraoral examination disclosed a soft and smooth mass extending from the right maxillary molar to the tuberosity. Computed tomography (CT) revealed an osteolytic lesion of 4×4.5×5 cm in the right mandibular ramus . In addi99mTc-methylene diphosphonate scans. The chest X-ray result was negative. No kidney or liver abnormalities were evident upon physical examination. Tests for HBsAg, HBeAb, HBcAb and hepatitis B virus pre-S1 antigen were all positive.A 37-year-old male was admitted to the Affiliated Hospital of Qingdao University in March 2007, presenting with a painless and progressive swelling in the right mandible, initially 1.0×1.0×1.0 cm in size, which had been apparent for three months, and numbness of the lower lip, which had persisted for almost one month. Prior to admission to the Affiliated Hospital of Qingdao University, the patient was fist admitted to the Dental Clinic of Longkou People’s Hospital  in Nov 2006 for a toothache in the right mandible for four months, which was identified as an accompanying symptom. The patient was first admitted to the Dental Clinic for a toothache in the right mandible, which was actually an accompanying symptom. A physical examination showed a solid 4×4×2.5-cm tumor in the ramus region. Thereafter, CT scans revealed a 6.0×4.8-cm osteolytic lesion in the upper segment of the right mandibular ramus, involving the masseter muscle and the lateral pterygoid . EnricheThe needle biopsy of the tumor revealed metastatic HCC in the connective tissues, positive for HAb18 in immunohistochemical staining, which was relatively highly expressed in the cytoplasm . CT scanHCC is considered to be the sixth most frequently diagnosed cancer, accounting for 5.7% of all new cancer cases and/or mortalities per year, 82% of which occur in developing countries  . Althouget al  and/or hepatitis C virus (HCV) infection is the primary risk factor for the development of HCC . FollowiThe major characteristics of mandibular metastasis are masses with or without toothache, numbness of the lower lip or the involved skin, and dysfunction of the temporomandibular joint for condyle involvement . BleedinAs the initial diagnosis in the present study was of a mandibular tumor, which was consequently diagnosed as a metastasis from HCC, the association and differences between primary and secondary tumors requires consideration and verification. Specifically, when significant lumps and/or pain is present at the secondary site, but there are no clear symptoms at the primary site, positron emission tomography-CT and single-photon emission CT may contribute to the evaluation of early metastasis in the bone tissues. Furthermore, histopathological evaluation should be considered for the final diagnosis of potential metastatic carcinomas.In the present study, two cases of HCC metastasis which had migrated to the mandible, with extension to the ramus and condyle, were identified. Although the articular disc and the cranial bones were found to be unaffected in either case, simultaneous dissection of the tumor and the articular disc was performed to avoid recurrence. In spite of an increased survival time and extended life-expectancy following treatment, the prognosis of extrahepatic metastasis remains unsatisfactory. Therefore, the management of metastatic carcinomas is mainly focused on pain relief and organ preservation. Based on the findings of the present two cases, radical dissection of the secondary tumors combined with arterial infusion chemotherapy was attempted for the primary tumors. However, the survival time of the two cases was less than one year. Accordingly, radiotherapy to control bleeding and/or pain relief should be considered as an essential option to prevent disease progression.


We reported two rare cases of congenital diaphragmatic hernia with abdominal wall closure defect, which were not associated with septum transversum diaphragmatic defects or Fryns syndrome.Case 1: a Japanese baby boy was delivered at 37 weeks’ gestation by urgent cesarean section because of the diagnosis of severe fetal distress. Congenital diaphragmatic hernia with omphalocele was prenatally diagnosed with fetal ultrasound. A ruptured omphalocele was confirmed at delivery. A silo was established on the day of his birth; direct closure of his diaphragmatic defect and abdominal wall closure was performed on the fifth day after his birth. Trisomy 13 was confirmed by genetic examination. His postoperative course was uneventful and he was discharged 5 months postnatally with home oxygen therapy. He was readmitted because of heart failure and died at 6 months.Case 2: a Japanese baby boy, who was prenatally diagnosed with gastroschisis, was delivered at 35 weeks’ gestation by urgent cesarean section because of the diagnosis of fetal distress. Silo construction using a wound retractor was performed on the day of his birth and direct abdominal closure was performed on the tenth day after his birth. Trisomy 21 was confirmed by genetic examination. Treatment for his respiratory distress was continued after surgery. A retrosternal hernia was revealed at 6 months and direct closure of retrosternal diaphragm with the resection of hernia sac was performed. His postoperative course was uneventful and he was discharged with home oxygen therapy.Attention should be paid to chromosomal abnormality in cases in which the coexistence of congenital diaphragmatic hernia and abdominal wall closure defect are observed. Malformations associated with congenital diaphragmatic hernia (CDH) can influence the morbidity and prognosis of the affected neonate . The comWe encountered two cases of CDH combined with an abdominal wall defect. This report describes their clinical features, which are unlike those reported previously, and our surgical management. Our two patients did not fit the syndromes previously reported, but both had chromosomal anomalies.A Japanese baby boy was delivered at 37 weeks’ gestation by emergent cesarean section because of the diagnosis of severe fetal distress. At 33 to 34 weeks’ gestation, fetal CDH and omphalocele had been detected by fetal ultrasonography and magnetic resonance imaging  support was performed because hypoxia, which might have been due to congenital cardiovascular disease and apnea due to trisomy 13, was observed. He was discharged 5 months postnatally with home oxygen therapy. However, he was readmitted because of heart failure and died at 6 months.A Japanese baby boy was delivered at 35 weeks’ gestation by emergent cesarean section because of the diagnosis of fetal distress. At 21 weeks’ gestation, fetal gastroschisis had been detected by fetal ultrasonography. Five minutes after delivery, he was intubated and mechanical ventilation was started because of hypoxia. His birth weight was 1892 g and his Apgar score was seven at 1 minute and eight at 5 minutes. His entire small intestine, part of his colon, and the greater curvature of his stomach were outside his abdomen, so a silo was constructed using a wound retractor . He had et al. reported a 9 to 11 % incidence of CDH associated with an abdominal wall defect  that was higher than the frequencies given in the reports just cited [et al. reported the association of CDH and omphalocele in a patient with trisomy 18 [Coexistence of CDH and an abdominal wall defect is considered to be rare and our case 2 is the first report of gastroschisis associated with a retrosternal hernia. Borys and Taxy performed a 10-year review of autopsy records and reported that the incidence of CDH associated with omphalocele was 0.077 % , and 3.1st cited , 11, 12.st cited , 13. Coest cited  or in thOther anomalies associated with CDH may have an important influence on survival and it was recently reported that pulmonary hypoplasia is critical , 3. AccuWe treated two cases of CDH associated with an abdominal wall defect. Coexistence of these anomalies is rare and may be associated with septum transversum diaphragmatic defects or Fryns syndrome. Our cases did not feature such anomalies, but both babies had chromosomal abnormalities. In neonates with abdominal wall defects and chromosomal abnormalities, we need to pay attention to the possible coexistence of CDH . Accumulation of more cases is required to determine whether this rare combination is due to multiple malformation syndromes and defects of midline development or occurs by chance.Written informed consent was obtained from the parents of the patients for publication of this case report and any accompanying images. A copy of the written consents is available for review by the Editor-in Chief of this journal.


A neonate presented with abdominal mass in left flank was investigated and operated upon. Histopathology confirmed the diagnosis of clear cell sarcoma of the kidney. Post-operatively, chemotherapy was given according to the NWTS-5 protocol. During follow-up, the patient has shown good recovery after 7 months of surgery. Wilm's tumors comprise 6% to 7% of childhood cancer; whereas the remaining non-Wilm's renal tumors comprise less than l% [1]. In neonates amongst non-Wilm’s tumors, most common are congenital mesoblastic nephroma (CMN), rhabdoid tumor of the kidney, and multilocular cystic nephroma, nephroblastomatosis complex and ossifying renal tumor of infancy. CCSK is most commonly observed in the age group of 3 to 5 years [2]. It is the most frequently misdiagnosed renal tumor in children, as it is unusual, has varied morphology, and there are no specific diagnostic markers [3]. Here we report a case of CCSK in a neonate.A 28-day-old male neonate was hospitalized with progressive increasing abdominal mass for last 2 weeks. On palpation, a hard lump was noted in left flank. Ultrasound abdominal showed the mass of renal origin; there was no vascular involvement or lymphadenopathy. Routine investigations were within normal limits. Computed tomographic (CT) scan revealed left renal mass of heterogeneous nature along with cystic areas. X-ray of the chest was normal. Left nephrectomy was done and the specimen was examined histopathologically. Cord cells and spindle shaped cells interspersed in fibrovascular matrix arranged in storiform pattern were seen. Diagnosis of clear cell sarcoma of kidney was made. Skeletal survey was done after confirmation of histology to rule out bone metastasis. Depending on histopathology report and work up for metastasis patient was found to have Stage 1 disease. Chemotherapy according to regimen I of NWTS-5 protocol was administered for 24 weeks. Patient was examined on his each hospitalization for chemotherapy schedule. There were no significant complications due to chemotherapy. Radiotherapy was not given. Contrast enhanced CT of the abdomen and pelvis was repeated. X-ray chest and skeletal survey were done after 3 and six months to rule out recurrence. There were no signs of any residual disease on follow-up. CCSK was first described in 1978 by three distinct groups, Beckwith and Palmer, Morgan and Kidd, and Marsden and co-workers who named it “bone metastasizing renal tumor of childhood” since it has predilection for skeletal metastasis [1]. Bone metastases occur in 40% to 60% of patients with CCSK, unlike less than 2% of patients with Wilms’ tumor. Other clinical features, which separate it from Wilm’s tumor, are a lack of association with sporadic aniridia and hemihypertrophy [1]. The peak incidence is between 3 and 5 years of age. It is very rare in infants younger than 6 months [4]. The present case is of a neonate. Ultrasonography is the initial investigation, which showed the mass was heterogeneous in echogenicity with cystic components and necrosis. On CT scan, non-homogeneous enhancement, with attenuation less than that of normal renal parenchyma is usually seen. Also, these tumors may contain areas of low attenuation corresponding to necrosis and cysts [5]. On cut section, these tumors appear grossly as tan-gray, soft, and mucoid. Cystic foci are almost always present and may represent the dominant feature, resembling that of multilocular renal cyst. On histopathological examination, CCSK has three components, namely, (1) cord cells, (2) septal cells, (3) an intercellular matrix of mucopolysaccharide. Cord cells are small round to oval shaped cells with mitotic figures. Septal cells are spindle shaped cells seen surrounding the fibrovascular septa. The matrix of mucopolysaccharide broadcasts the clear appearance of CCSK. There are four modalities of treatment surgical resection, surgical resection with chemotherapy, surgical resection with radiotherapy, surgical resection with chemotherapy and radiotherapy. For unexplained reasons, CCSK has been known to have the best outcome amongst all perinatal tumors [6].Radical nephrectomy is the initial treatment of choice if the lesion is resectable [5]. It has been found that in the NWTS-3, the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival in patients with clear cell sarcoma of the kidney [7]. Siebel et al found that in NWTS-4, patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival rate compared with patients treated for 6 months (87.5% vs 60.6% at 8 y). Also, the overall survival has improved for patients with clear cell sarcoma of the kidney from NWTS-3 to NWTS-4 (83% vs 66.9% at 8 y) [8]. In the NWTS-5 protocol, CCSK is treated with radical nephrectomy followed by chemotherapy with vincristine, Doxorubicin and Etoposide for 24 weeks and radio- therapy. Overall survival is 69% [9]. Four important prognostic factors are treatment with Doxorubicin, stage of the disease, age at the time of diagnosis and presence of tumor necrosis [7].Emphasis should be given to look for necrosis in the radiological investigations in such cases as presence of necrosis is one of the prognostic indicators. Survival in this case can be attributed to earlier detection and lower stage of the disease at time of diagnosis.Source of Support: NilConflict of Interest: NoneEditorial Comment: In the largest SIOP cohort described so far, the median age at diagnosis for CCSK was 2.6 years [1]. Stage IV disease and young age were significant adverse prognostic factors for event-free survival (EFS), whereas factors such as gender, tumour volume and type of initial treatment were not found to be prognostic for EFS and overall survival (OS) [1]. Survival of a neonate with CCSK therefore is worth lauding. As such in the world literature, less than a dozen cases of CCSK have been reported in the fetuses and the neonates. In 2008, the celebrated pediatric pathologist Issac H Jr. in a review of 210 renal reported tumors diagnosed between 1960 and 2007 in 47 fetuses and 163 infants less than 2 months olds had reported only 7 cases of CCSK [2]. On review of the literature in 2005, Hung had found only 7 cases of CCSK aged less than 6 months of age  of which two were extrarenal cases [3]. Although the authors have quoted that for unexplained reasons, CCSK has been known to have the best outcome amongst all perinatal tumors [2], it is hard to fathom. One issue that needs to be understood is that the neonates would tolerate chemotherapy poorly; the authors were lucky in the index case. It would be prudent to restrict adjunct therapy to single agent chemotherapy, e.g., vincristine [4], and to avoid radiotherapy for such young presenters of CCSK.References1. Furtwängler R, Gooskens SL, van Tinteren H, de Kraker J, Schleiermacher G, Bergeron C, et al. Clear Cell Sarcomas of the Kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: A report of the SIOP Renal Tumour Study Group. Eur Cancer, 2013: 49: 3497-3506.2. Isaacs H Jr. Fetal and neonatal renal tumors. J Pediatr Surg. 2008; 43:1587-95.3. Hung NA. Congenital "clear cell sarcoma of the kidney". Virchows Arch. 2005;446:566-8.4. Suzuki H, Honzumi M, Itoh Y, Umehara N, Moriyama S, Funada M. Clear-cell sarcoma of the kidney seen in a 3-day-old newborn. Z Kinderchir. 1983; 38:422-4.


Primary systemic amyloidosis (PSA) is one of systemic amyloidosis, characterized by clonal plasma cell disorder. The disease is rare and with high fatality. Signs and symptoms of PSA are various and complex, which depend on the organs involved. Here we report a case in which the patient initially suffered from gastrointestinal symptoms. Gradually periorbital purpura, skin fragility, and subsequent petechiae, ecchymoses and sclerosis of the distal limbs, appeared. Biopsy of his palmar skin showed scleroderma-like changes. However, histopathology of the petechiae lesion on forehead with Crystal Violet Staining prompted deposition of amyloid; gastric mucosal biopsy with Congo Red staining was also positive, which made clear the diagnosis of PSA. Bone marrow biopsy and serum immunofixation electrophoresis (IFE) revealed plasmacytosis and M proteinemia. Other examinations were performed to assess the function of organs. PSA was challenging due to the initial atypical clinical presentation and absence of biopsy with special staining. The case demonstrates that PSA should be considered in patients with multisystemic symptoms and biopsy with Congo Red staining should be performed to exclusively diagnose amyloidosis. Primary systemic amyloidosis (PSA), also called Lubarsch-Pick disease, is a rare, complex and protean disease. Currently, it is a mortal disease without any effective therapy, and its median survival time is approximatedly 13 months . Here weA 47-year-old male presented with recurrent dizziness, generalized weakness, easy fatiguablility, accompanied with discontinuous abdominal pain, dyspepsia and constipation for six years. He had been examined with gastroendoscope and magnetic resonance imaging (MRI) of the head for several times, but no specific lesion could be identified. Gradually the skin of his fingers and toes became sclerotic. At the same time, multiple, non-itchy petechiae, purpura and ecchymoses lesions can been found around the eyelids, as well as on the tongue, cheek, neck and the higher chest wall bilaterally Fig. -f. BiopsPSA is a clonal plasma cell disorder, including the production, aggregation, polymerization, fibril formation and finally extracellular deposition in various organs of the precursor protein, ultimately leading to organ dysfunction and death . SymptomFirstly, in order to make correct diagnosis of the disease, biopsy with Congo Red staining of the affected organ is significant. Routine pathological examination without the special staining always leads to misdiagnosis –7. As inIn summary, clinical manifestations of PSA are diverse and nonspecific. It should be considered in patients with skin lesion such as petechiae, purpura and ecchymoses, especially accompanying with multisystemic symptoms. Biopsy of lesion with Congo Red staining is of diagnostic significance.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images.


Hairy polyps are common congenital benign lesions of oroandnasopharynx containing elements of both ectodermaland mesodermal origin. However, their occurrence in palateis quite rare. Here we present a case of hairy polyp associatedwith palatal cleft in an eight months old female infant. Wediscuss the clinicopathological features, etiology, proposedtheories related to its formation and its significance. Congenital tumors of oral cavity are rare and among thosethat occur, hairy polyp is the most common, affecting oroandnasopharynx. The total number of documented cases is135.An 8 months old female infant was admitted to craniofacialunit of our institution with a growth present on the hardpalate. The growth was present since birth and graduallyincreased in size. Examination of the oral cavity revealed asoft tissue growth measuring about 2 × 2 cm in size on thehard palate associated with cleft of the soft palate. Thegrowth was pedunculated, overlying mucosa was normalwith pigmentation seen around the base of the lesion. There was history of nasal regurgitation and difficultyin feeding. As per family history the marriage wasnon-consanguineous with normal childbirth. The generalcondition of the patient was normal. The lesion wasprovisionally diagnosed as lipoma/ dermoid cyst.CT-scan revealed the presence of a well-defined solitary,round to oval lesion attached to the hard palate with imagingcharacteristics of hypodensity compared to that of adjacentbone and muscle .The lesion was surgically removed under generalanesthesia along with the closure of the palatal defect usingthe Langenback’s technique.Gross examination of the excised specimen measured3 × 2 cm, grayish white in color with smooth surface. Thesurface of the lesion showed hair growth. The lesionexhibited rubbery consistency .Microscopic examination revealed epithelium ofparakeratinized stratified squamous type  withimmature hair follicles, sebaceous and sweat glands infibrous connective tissue stroma . Vacuolated cellsare seen in the follicle and lobules of adipose tissue arepresent.Based on the histopathological findings, a final diagnosisof hairy polyp was given. The postoperative course wasuneventful and the patient was discharged on 5thpostoperative day. On follow-up the patient is free of disease,with normal feeding and without evidence of recurrenceafter 6 months.Hairy polyps are unusual but well-recognized entities oforo- and nasopharynx.2Arnold first classified neoplasms arising from nasopharynxas dermoid, teratoid, teratomas, epignathi.Hairy polyps are typically pear or sausage shaped lesionsthat may be sessile but more often are pedunculated.Macroscopically, the size may vary from 0.5 cm to 6 cmand are usually gray or pink in color. Radiological studiesare crucial to help delineate the extent and origin of thelesion. Bony anomalies and dehiscence are best identifiedby CT scanning.The clinical, gross and histopathological findings in thepresent case were similar to the description mentionedabove. Also CT findings rule out the bony involvement ofthe lesion.Various theories have been proposed regarding the hairypolyp formation.Disturbed development during the fusion of epiblast ofthe stomatodeum with anterior foregutFailure of nasopharyngeal membrane to regress duringthe seventh week of gestationMisdirected pluripotential tissueMisdirected first pharyngeal apparatus in germ cellrests.As evident from the histopathological features of thiscase, the constituents of the lesion were derived exclusivelyfrom the ectoderm and mesoderm and hence the tumor canbe correctly designated as hairy polyp.Although hairy polyp occurring in nasopharynx has beenreported previously, such lesions occurring in the palate isquite rare. Our case is unique in that it is present inassociation with cleft palate. This is the first such case inmore than 1000 cleft palate cases reported in our institution.Also very few cases have been reported in the literature.The presence of cleft palate along with hairy polyp mayplay significant role in the etiopathogenesis of the cleftpalate.5The cleft palate seen in our case might have been theresult of failure of closure of palatal shelf caused by thepresence of the mass.6It is hence necessary to distinguish hairy polyp as aseparate entity because of its clinical significance andmicroscopic features and they should be considered asdevelopmental malformations.


Spinal vascular malformations can be classified in arteriovenous malformations, cavernomas, and capillary telangiectasias. Arteriovenous malformations are the most common spinal vascular anomaly and may be located intra- and/or perimedullary. According to their nidus type and hemodynamic flow patterns, they can be differentiated into fistulous, glomerular and juvenile categories. In our case, a hyperintense extradural mass was misinterpreted as a neurinoma. The histological analysis revealed typical signs of an arteriovenous malformation.A 57-year-old Caucasian woman presented with back pain and hypesthesia in digiti two to four of her left foot. Magnetic resonance imaging showed a long-segment intraspinal extradural soft-tissue mass in the left L4 - S1 paravertebral region with homogeneous enhancement of contrast medium. Due to another similar lesion in the lower ankle and additional cutaneous manifestations, the suspected diagnosis was a systemic disease with neurinomas (e.g. Recklinghausen’s disease). To clear up the origin and type of this lesion exploratory surgery with a hemilaminectomy of L5 was performed. This showed abnormally arterialized, dilated, and tortuous vessels. After complete resection, the intra-operative impression of an arteriovenous malformation was confirmed by a neuropathologist.Completely extradural intraspinal arteriovenous malformations in the lumbar spine are extremely rare. In magnetic resonance imaging they are often misinterpreted as a tumor. Arteriovenous malformations can cause compression and venous congestion, or mask symptoms like a spinal disk herniation.In cases presenting with these symptoms and magnetic resonance imaging findings, an extradural intraspinal arteriovenous malformation should be considered as a possible diagnosis. Pre-operative angiography or magnetic resonance imaging angiography can be used to verify the diagnosis. Arteriovenous malformations (AVMs) are the most common spinal vascular anomaly and can be located intra- and/or perimedullary. According to their nidus type and hemodynamic flow patterns they can be differentiated into fistulous, glomerular and juvenile categories .In our case, an extradural mass with contrast enhancement in the lumbar spine was misinterpreted as a neurinoma. However, histological examination revealed the typical characteristics of an AVM.A 57-year-old Caucasian woman presented to our hospital with a tumor in her lower ankle. She reported hypesthesia in digiti two to four of her left foot. She also presented with temporary back pain.A magnetic resonance imaging (MRI) scan of her lumbar spine revealed a long-segment intraspinal extradural soft-tissue mass in the left L4 - S1 paravertebral region with homogeneous contrast enhancement. The mass was compressing the nerve roots of L4 and L5 and causing bone destruction of the fifth lumbar vertebra Figure . OverallNo neurological deficit occurred after the operation. She had an uneventful recovery and was discharged seven days later. During the clinical follow-up three months post-surgery, the pain in her lower back had subsided, but the hypesthesia in digiti two to four of her left foot had remained. An MRI scan showed a regular recovery with no signs of a residuum.Spinal AVMs are often located intra- and/or perimedullarily. Roughly 25% of all spinal vascular shunts are performed on AVMs. The different types usually become symptomatic in early adulthood. Medullary damage can be caused by venous congestion and hemorrhage. The space-occupying effect is rarely the reason for the symptoms .et al.[et al.[Only a few cases of lumbar intraspinal extradural AVMs have been published ,3. The pet al. only proThe typical appearance of spinal cord AVMs in an MRI scan is as a conglomerate of dilated vessels that are demonstrated on T2-weighted sequences as flow voids, in T1-weighted sequences (depending on their flow velocity and direction) they present mixed hyper/hypointense tubular structures. Often these lesions have dense contrast enhancement. They can cause damage by acute intramedullary or subarachnoidal hemorrhage . If intrIn our case this contrast-enhancing lesion has been misinterpreted as a neurinoma, as its signal characteristics and affinity to contrast medium appeared similar to neurinomas . AdditioAfter analysis of all the sequences, other differential diagnoses (for example a solitary lymphoma or a metastasis) were possible because of a similar appearance in the MRI scan, but an AVM was not considered at all.Despite the unexpected intra-operative finding, this lesion could be completely removed by the neurosurgeon without foreseeable complications. In cases such as these, the German Society of Neurosurgery recommends in its guidelines a pre-operative angiography. This is necessary to define the anatomy of the involved vessels and to evaluate the hemodynamics. This in turn, makes it possible to classify the AVM exactly, to decrease the operative risk, and to improve the post-operative outcome. According to these guidelines, a non-invasive magnetic resonance angiography can also be used to detect a spinal AVM.A completely extradural intraspinal AVM in the lumbar spine is extremely rare. In MRI scans they are often misinterpreted as a tumor. They can cause compression and venous congestion, and cause the same symptoms as a spinal disk herniation.In cases presenting with these symptoms and MRI findings, an extradural intraspinal AVM should be considered as a possible diagnosis. A pre-operative angiography or magnetic resonance angiography can be used to verify the diagnosis.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AVM: arteriovenous malformation; MRI: magnetic resonance imaging; SPIR: Spectral Presaturation with Inversion Recovery; TSE: Turbo Spin Echo.The authors declare that they have no competing interests.SS was a major contributor in writing the manuscript. SS, AR and MF analyzed the images. US performed the neurosurgical procedure. OG was part of the neurosurgical team and was also a major contributor in writing the manuscript. JN performed the pathological examination. All authors read and approved the final manuscript.


The association of bowel tumor and inguinal hernia is rare. We report according to our research the first case of the migration of a small bowel schwannoma into an inguinal hernia.We report the case of a 51-year-old Moroccan malen admitted for a non-reducible right inguinal hernia in which surgical exploration showed the presence of a small bowel tumor that had migrated into his hernia sac. A histopathological examination of the tumor was in favor of a small bowel schwannoma.Small bowel schwannoma is an exceptional clinical entity for which the diagnosis is difficult; its confirmation needs histological and immunohistochemical studies. The migration of an intestinal tumor into inguinal hernia is a very rare entity. However the migration of a schwannoma of the small bowel into inguinal hernia is exceptional.We report according to our research the first case of migration of small bowel schwannoma into an inguinal hernia.We report the case of a 51-year-old Moroccan man with no past medical history who presented with a right inguinal mass looking like an inguinal hernia.The clinical history started 1 year ago with the appearance of a right inguinal mass, impulsive during cough and reducible.He observed an increase in the mass volume, and the appearance of a second hard and non-reducible mass 3 months later.A clinical examination showed the presence of two inguinal masses the former soft and reducible and the latter hard and non-reducible. There was no associated nausea or vomiting. He had a history of hypertension for the previous 9 years. He had no significant changes in standard biochemical findings. The first diagnosis was the migration of his bowel in the hernia sac.During surgical exploration we found an indirect inguinal hernia; however, at the opening of the sac we discovered a 10cm mass originating from the ileal portion of his bowel tract  117 and anti-desmin, hence proving its neurological nature schwannoma Figure .The content of 10% of inguinal hernias is the bowel system ,3; howevet al.[In a series of 22,000 inguinal hernioplasties described by Nicholson et al., they foet al.-10, one et al.,11. In oet al.; the preet al.,14. Otheet al.-17. The et al.,14,18. Iet al.. The ageet al.; a slighet al.-16 size et al..In general, at endoscopic examination, the tumor appears submucosal with a large protrusion into the lumen of the digestive tube associated to small erosions of the mucosa. A biopsy is usually negative  and the We should differentiate between schwannomas and digestive stromal tumors. The latter are rare; they frequently originate from the connective system of the digestive tube .Four types are identified :– Smooth muscle cells tumor.– Nervous system tumor.– Nervous and smooth muscle cells tumors.– Undifferentiated tumor.Stomach and small bowel are the most frequent localization.All submucosal tumors of the digestive layer are considered stromal tumors; neither endoscopy nor echoendoscopy may confirm the diagnosis, only histological examination and immunohistochemical study may give us the precise diagnosis. Digestive stromal tumors show structural and immunophenotypic heterogeneity according to the segment in the digestive tube, and in the same tumor, the expression of CD117 is frequent but less constant [The association of inguinal hernia and bowel schwannoma is exceptional; the prognosis is favorable.The main difficulty is in precise diagnosis and for this histological and immunohistochemical studies are necessary.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.ANTI PS 100: Antibodies peroxidase immunostaining 100; CD: Cluster of differentiation.The authors declare that they have no competing interests.AZ reviewed our patient’s case, data and figures, and was a major contributor in writing the manuscript. HC and YT reviewed our patient’s case and data, completed subsequent drafts of the manuscript and were major contributors in writing the manuscript. HE and JE were involved during the initial presentation of our patient’s case and were major contributors in writing the manuscript. KS was involved during the initial presentation of our patient’s case. MT reviewed our patient’s case, data, coordinated the authors and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


A 26 year old man, with no specific past or family history, presented with slow growing subcutaneous mass in the right parietal region of about 4 cm. Skin examination revealed a firm subcutaneous nodule that was movable over the underlying bone. Physical examination was not remarkable other than above-described skin lesion. X-rays of the skull (A) and CT scan (B) showed a subcutaneous mass next to the right parietal eminence measuring 17×39 mm and containing calcifications. Surgery resection of the tumor was complete and easy (C). The nodule was not attached to underlying skull. The postoperative course was unremarkable. Histological examination confirmed the diagnosis of soft-tissue chondroma (D). Extraskeletal chondroma is a rare, benign cartilaginous tumor of the soft tissue. It presents as a solitary subcutaneous mass measuring less than 3 cm in diameter that is usually painless and slowly growing. It is most frequently found in the hands and feet of adults in the fourth and fifth decades. Its location in the scalp is exceptional and atypical. Complete excision is recommended for the treatment of extra skeletal chondroma.


Costal exostosis is a benign condition that sometimes requires emergent surgery because of associated hemothorax; in addition, there have been cases with malignant transformation to chondrosarcoma. Here, we describe an asymptomatic patient who underwent thoracoscopic resection for primary costal exostosis.A 16-year-old male was found to have a bow-shaped shadow on a chest X-ray. Chest computed tomography revealed a rod-like mass with a soft tissue shadow adjacent to the left fifth rib. A thoracoscopic partial resection of the left fifth rib was performed. Intraoperative findings included thickening of the pericardium near the tip of the growth and erosion of the visceral pleura of the left lung. The resected specimen was diagnosed as a primary costal exostosis based on histopathological findings.We review the published literature on costal exostosis and discuss the surgical indications of asymptomatic cases. A costal exostosis is a benign growth capped by cartilage, which protrudes from a rib. It may sometimes require emergent surgery because of an associated complication such as damage to an intrathoracic organ , 2, and A 16-year-old male was found to have a bow-shaped shadow to the left of the middle lung field on chest X-ray. Chest computed tomography (CT) revealed a rod-like mass with a soft tissue shadow adjacent to the left fifth rib (Fig. The patient underwent thoracoscopic partial resection of the left fifth rib. An approximately 50-mm long structure protruding from the fifth rib into the chest cavity was observed through the thoracoscope. Thickening in the pericardium near the tip of the structure and erosion of the visceral pleura of the left lung were also seen Fig. . The carHistopathological examination of the resected specimen found that the growth was composed of mature bone tissue; there were no atypical cells or bone marrow within the growth. No cartilage cap was found, but thickened fibrotic tissue was observed and thought to represent inflammatory changes caused by degeneration and omission or mechanical irritation of the cartilage cap. There were no signs of malignant changes, and the growth was diagnosed as a primary costal exostosis.Exostosis has accounted for 50 % of primary benign bone tumors and has been observed to occur at the metaphysis of long bones or originate from the surface of flat bones . It geneEXT genes and is manifested by skeletal deformities caused by abnormal bone growth [Primary exostosis occurs as solitary or multifocal lesions, and multiple exostosis is a hereditary disease that occurs in infants , 6. Heree growth .Solitary exostosis occurs in both infants and adults , 4, and Past reports of organ damage due to costal exostosis have described longitudinal lacerations of the diaphragm and pericardium adjacent to the tip of the exostosis that were caused by movements during respiration. Even in cases such as ours, with asymptomatic anterior left fifth costal exostosis, histopathological changes were seen in the pericardium and lung. In a patient with anterior left fourth rib exostosis, thickened pericardium and visceral pleura possibly caused by scratch have been reported . AlthougMalignant transformation of an exostosis to chondrosarcoma  is relatBecause of the risks of hemothorax, other life-threatening complications, and malignant transformation, prophylactic surgical resection should be considered for patients with costal exostosis. Care should be taken to perform resections with adequate margins in order not to leave the residual cartilage cap, especially for cases where the extent of the cartilage cap cannot be determined, and for cases of exostosis occurring at sites other than the ribs. There have been reports of cases of exostosis involving other bones that have developed postoperative recurrence because the initial resection was inadequate.Journal of Cardiothoracic Surgery.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of the We reviewed the published literature on costal exostosis and discussed the surgical indications of asymptomatic cases. Prophylactic surgical resection should be considered for patients with costal exostosis.


The pathologic spectrum of diseases that infiltrate the pituitary infundibulum includes a broad variety of clinical entities. There are significant differences in the prevalence of these etiologies depending on the age of presentation. Lymphocytic infundibuloneurohypophysitis (LINH) predominates over other causes of infundibular disease in adults over age 21. Differentiating LINH from other causes of infundibular disease can be difficult because the various etiologies often have similar clinical presentations and radiologic imaging characteristics. We report the first case in an adult of a mixed germ cell tumor comprised of germinoma and embryonal cell carcinoma infiltrating the pituitary infundibulum. In our case, a 23-year-old female was initially misdiagnosed as having LINH. She presented with panhypopituitarism and diabetes insipidus, which is the most common initial presentation in both entities. The two diagnoses are difficult to distinguish based on MRI imaging, CSF findings, and histopathological examination. Our case demonstrates the need for close follow-up of patients with isolated lesions of the pituitary infundibulum and reinforces the need for biopsy of an infundibular lesion when progression of disease is demonstrated. In our case, biopsy with comprehensive immunohistochemical staining was the sole means of making a definitive diagnosis. The pathologic spectrum of diseases that infiltrate the pituitary infundibulum includes a broad variety of clinical entities. Congenital, developmental, inflammatory, infectious, granulomatous, neoplastic, and traumatic etiologies have all been described in the literature . HoweverDifferentiating LINH from other causes of infundibular disease can be difficult because the various etiologies often have similar clinical presentations and radiologic imaging characteristics. In many cases, biopsy is the only way to make the definitive diagnosis, but carries the risk of causing panhypopituitarism (PHP), and is therefore usually deferred in a patient with intact pituitary hormone function. A thorough search for the presence of other systemic involvement with disease and examination of cerebrospinal fluid may provide clues for the diagnosis. In light of the high prevalence of LINH, adult patients with isolated lesions of the pituitary infundibulum are often followed conservatively, since LINH generally has a self-limited course and treatment is unlikely to reverse pituitary hormone deficiencies. Close follow-up of these patients is important in order to assess any progression of disease via imaging or visual field determination. Progressive growth of the lesion on serial MR imaging, worsening of visual field deficits, and/or progressive loss of pituitary hormonal function should raise concern for a neoplasm. In that case, and if the diagnosis is still not apparent, biopsy of the infundibular lesion should be considered in order to guide appropriate management –9.Herein, we report the first case in an adult of a MGCT comprised of germinoma and embryonal cell carcinoma infiltrating the pituitary infundibulum. In our case, a 23-year-old female was initially misdiagnosed as having LINH. She presented with PHP and diabetes insipidus (DI), which is the most common initial presentation in both entities. In addition, the two diagnoses are difficult to distinguish based on MRI imaging, CSF findings, and histopathological examination. Our case demonstrates the need for close follow-up of patients with isolated lesions of the pituitary infundibulum and reinforces the need for biopsy of an infundibular lesion when progression of disease is demonstrated. In our case, biopsy with comprehensive immunohistochemical staining was the sole means of making a definitive diagnosis.A 23-year-old female was referred to our pituitary center for evaluation of a pituitary infundibular lesion. She initially presented a few months earlier, with recurrent, intractable left frontotemporal headaches. Pituitary MRI showed an infiltrative hypothalamic and infundibulum lesion, with atrophy of the pituitary gland. When a detailed history was obtained, she reported amenorrhea for 3 years, progressive fatigue, cold intolerance, dry skin, and weight gain, as well as polyuria and polydipsia of 2-year duration. The initial physical examination revealed short stature (4′′11′′) but was otherwise normal, with well-developed breast and normal pubic hair development for her age. The initial ophthalmological examination was consistent with a right inferotemporal visual field deficit . The basA variety of different etiologies for her hypothalamic/infundibular lesion were considered, but initially, the diagnosis of LINH was favored, based on her age at presentation. The patient was managed conservatively with close clinical follow-up and physiologic hormone replacement with hydrocortisone, levothyroxine, and desmopressin. Three months after her initial assessment, her visual symptoms worsened, with progression of the visual field deficit in the right eye . Repeat Surgical pathological slides and immunohistochemical staining results are shown in Although the overall true incidence of pituitary infundibular lesions remains unknown, these lesions often are discovered incidentally, due to an increase in use of more sensitive and advanced brain imaging studies carried out to investigate nonspecific symptoms or to evaluate hypopituitarism . A retroLINH was suspected in our case initially, because of the higher prevalence of LINH in her age group and the absence of systemic involvement with sarcoidosis or other neoplastic diseases. LINH is of autoimmune origin and is characterized by lymphocytic infiltration in the infundibulum and the neurohypophysis . It was Primary CNS GCTs are a rare and heterogeneous group of tumors comprised of germinomas and nongerminomatous germ cell tumors. Germinomas are the more common among the primary intracranial GCT, comprising 65% of GCTs, and the majority of germinomas (57%) arise in the suprasellar cistern. Nongerminomatous GCTs include embryonal carcinomas, teratomas, choriocarcinomas, and endodermal sinus tumors. These tumors preferentially involve the pineal gland (68%) and those arising in the suprasellar region are reported in only 4.6% of all intracranial GCTs. The overall prevalence of intracranial GCT is much higher in males; however, suprasellar GCTs are more frequent in females. Germinomas are usually diagnosed between age of 10 and 21 years, whereas nongerminomas are more frequently diagnosed between birth and 9 years . AlthougThe clinical triad of DI, pituitary insufficiency, and visual abnormalities is common at presentation with a pituitary GCT, but obstructive hydrocephalus can be seen in the presence of pineal tumors . RadioloSeveral case reports have emphasized the similarity in the clinical presentation of LINH and germinomas –5, 7, 8.There are several reports emphasizing the difficulties encountered in differentiating between LINH and GCT upon histopathologic examination, as lymphocytic infiltration can be seen in both entities. It is not unusual for intrasellar germinomas to be missed, especially when a few scattered neoplastic cells are embedded in diffuse predominant sheets of lymphocytes. Because the lymphocytic infiltration can be so predominant, the identification of the neoplastic GCT may be difficult –28. PropDespite their rare occurrence in adults, clinicians should keep in mind the possibility of a GCT when evaluating patients with an infiltrating disease of the pituitary and infundibulum, even when serum or CSF markers are negative. As our case demonstrates, GCT and LINH can present with highly similar clinical signs and symptoms as well as similar pathological findings on biopsy. In our patient, the invasion of the lesion into the optic chiasm and progression of the pituitary stalk involvement on MRI, along with worsening of the visual field deficits, raised our suspicion for a neoplasm. This finding highlights the need for close follow-up with serial MRI imaging and visual field testing when evaluating these patients. Tissue biopsy with adequate sampling was essential for the correct diagnosis in our case and fortunately was of low risk to the patient, as she presented with PHP, and thus biopsy of the infundibulum would not contribute to further hormonal dysfunction. Comprehensive immunostaining was also important, both to ensure differentiation of GCT from LINH and to determine the subtype of GCT, which guided appropriate treatment.


Obturator hernia is a rare form of abdominal hernia and a diagnostic challenge. It is commonly seen in elderly thin females. Its diagnosis is often delayed with resultant increased morbidity and mortality due to bowel ischemia/gangrene. It is mistakenly diagnosed as femoral or inguinal hernia on USG. Computed tomography is diagnostic and is a valuable tool for preoperative diagnosis. This report presents a case of 70-year-old thin female presenting with intestinal obstruction due to left sided obstructed obturator hernia. USG showed small bowel obstruction and an obstructed left sided femoral hernia. CT scan of abdomen and pelvis with inguinal and upper thigh region disclosed left sided obturator hernia. It also detected clinically occult right sided obturator hernia. Early diagnosis and surgical treatment contribute greatly in reducing the morbidity and mortality rate. An obturator hernia is a rare cause of all abdominal wall hernias commonly seen in females. Its clinical diagnosis is often difficult due to uncommon incidence, its deep location, and infrequent symptoms and signs. Delay in its diagnosis causes poor prognosis with increased morbidity. Early CT imaging establishes diagnosis and detects asymptomatic contralateral obturator hernia. The following case report highlights these diagnostic difficulties and reviews the current literature on diagnosis and management of such cases.70-year-old known hypertensive female patient presented with intermittent abdominal pain and vomiting for 2 days. She gave past history of pulmonary Koch's 10 years back for which she completed AKT. On examination the patient was thin-built, conscious, and well oriented. Blood pressure was 150/90 mmHg. Respiratory rate was 22/min. Per abdomen examination showed mild abdominal distension. She was referred for USG Abdomen and Pelvis. USG Abdomen and Pelvis showed mild dilatation of small bowel loops in entire abdomen  with intermittent to-and-fro peristalsis. Mild free fluid was noted in pelvis and in between small bowel loops. Left inguinal and left upper thigh region showed a herniated small bowel loop extending in medial aspect of upper thigh which was irreducible. A diagnosis of obstructed and irreducible left femoral hernia was made. X-ray standing abdomen  revealedExploratory laparotomy was performed which confirmed obstructed left obturator hernia and the entrapped segment of bowel was released. Small bowel 100 cm proximal to ileocaecal junction was found to be gangrenous. Hence, resection anastomosis was done. Approximately 60 cm of small bowel was resected. Obturator hernia was also confirmed on right side containing mesentery. The hernial defect was covered on either side with prosthetic mesh. Care was taken not to damage the obturator nerve on either side while covering it with prosthetic mesh.Obturator hernias account for 0.07–1% of all hernias and 0.2–1.6% of all cases of mechanical obstruction of small bowel . They haIt is commonly seen in elderly females and postpregnancy patients due to greater width of the pelvis, larger obturator canal, and increased laxity of the pelvic tissues. It is nicknamed “little old lady's hernia” as it affects this group due to atrophy and loss of preperitoneal fat around obturator vessels in obturator canal predisposing to obturator hernia. Multiparity, COPD, constipation, ascites, and causes of raised intra-abdominal pressure are its other predisposing factors .Arnaud de Ronsil was the first to describe the obturator hernia in 1724 and Obre was the first to perform the successful operation in 1851 [Obturator hernia occurs through obturator canal which is 1 cm wide and 2-3 cm long. The obturator foramen is formed by continuity of pubic and ischial bones and is covered by obturator membrane except in its anterosuperior aspect where it is perforated by obturator nerve, artery, and vein. These travel along 2-3 cm in oblique tunnel  formed by obturator externus and internus muscles. Peritoneal hernia develops through this defect by first increasing separation of muscular band of obturator internus muscle and later separating obturator externus muscle. The hernia sac finally lies on top of obturator externus muscle beneath pectineus muscle.Clinical diagnosis of obturator hernia is difficult due to uncommon incidence, deep location, and infrequent symptoms and signs. Early diagnosis is needed as delay in its recognition causes poor prognosis in patients. Vast majority of patients fail to complain of obturator neuralgia. Physical examination is not contributory with resultant delay in diagnosis. Hernial sac irritates and compresses obturator nerve within the canal causing medial thigh pain (Howship-Romberg sign). Howship-Romberg sign is pain radiating down the medial aspect of the thigh to the knee and less often to the hip due to compression of the anterior division of the obturator nerve. It is considered pathognomonic of obturator hernia and is seen in up to 15–50% of patients. Palpable mass is found in 20% of cases in the proximal medial aspect of the thigh at the origin of the adductor muscles . High inEarly CT imaging causes early diagnosis with reduced morbidity and mortality associated with obturator hernia . X-ray sVarious surgical approaches are described in the literature in cases of obstructed obturator hernia. Abdominal, inguinal, retropubic, obturator, and laparoscopic approaches have been described. The published data favors the abdominal approach, utilizing a low midline incision. This method allows the surgeon to establish the diagnosis, avoid obturator vessels, give better exposure of the obturator ring, and facilitate bowel resection if necessary. Herniorrhaphy is performed by simple closure of the hernial defect with interrupted sutures, placement of a synthetic mesh. These have the lowest complication rates. Laparoscopic repair of obturator hernia can also be done. It produces less postoperative pain with shorter hospital stay and fewer pulmonary complications , 7.High index of suspicion for obturator hernia should be made in a patient presenting with small bowel obstruction with medial thigh pain. All hernia orifices  should be assessed and screening for Howship-Romberg sign should be done. Early diagnosis can be done by early CT scanning after ruling out inguinal and femoral hernias. This will increase the speed of diagnosis and avoid complications like bowel ischemia.Clinical FeaturesEmaciated thin elderly females.Intermittent attacks of small bowel obstruction.Howship-Romberg sign.Referred pain relieved by flexion of thigh and aggravated by extension, abduction, and medial rotation of thigh.Tender swelling in region of obturator foramen on vaginal and rectal examination.X-RaySmall bowel obstruction.Gas shadow in obturator foramen area.USGHernia deep to pectineus muscle on axial scan of inguinal region.Hernia occurring in plane similar to femoral canal but deep to pubic ramus.Small bowel obstruction.CTIdentification of hernia between pectineus and obturator externus muscles.Small bowel obstruction.


This report presents the case of a 40-year-old female with an 18-month history of a tender mass in the left distal femur. Radiological findings demonstrated periosteal buttressing. Magnetic resonance imaging (MRI) revealed a chondrogenic tumor of 3 cm in size developing from the external bone surface. It was difficult to differentiate periosteal chondrosarcoma from periosteal chondroma on the basis of size and the radiological and MRI findings. PET/computed tomography (CT) revealed abnormal linear uptake with an SUVmax of 2.7, indicating a malignant tumor. A diagnosis of periosteal chondrosarcoma was made, and wide resection was performed. Tumor histology was consistent with grade II chondrosarcoma. PET/CT is thus useful in differentiating periosteal chondrosarcoma from periosteal chondroma.Periosteal chondrosarcoma is an extremely rare low-grade malignant cartilaginous tumor arising from the external bone surface. Diagnosis of periosteal chondrosarcomas may be challenging, since this condition closely resembles periosteal chondromas. It has been reported that positron emission tomography (PET) is useful in distinguishing benign from malignant cartilaginous tumors using a maximum standardized uptake value (SUV Periosteal chondrosarcoma is an extremely rare and low-grade malignant cartilaginous tumor arising from the external surface of bones , and it 18F-FDG) positron emission tomography (PET) is a diagnostic imaging technique that detects glucose uptake by cells with high metabolic activity, including the heart, brain and tumor cells. The usefulness of PET has been reported in diagnosing malignant cartilaginous tumors, particularly those with borderline histological, imaging and clinical characteristics  cut-off of 2.0 could be used to distinguish benign from malignant cartilaginous tumors. However, there have been no reports on the use of PET for diagnosing periosteal chondrosarcoma due to the rarity of this condition.Fluorine-18 fluorodeoxyglucose . Written consent was obtained from the patient and the patient’s family for the publication of this study.A 40-year-old female presented with an 18-month history of a tender mass in the left distal femur. Physical examination revealed a bony hard mass in the medial aspect of the metaphysis in the distal femur.18F-FDG uptake in the distal aspect of the femur. Furthermore, PET/CT images clearly demonstrated periosteal buttressing with an abnormal 18F-FDG SUVmax of 2.7, indicating a malignant tumor of the bone surface in the distal femur. In addition, no evidence of distant metastases was identified , and the negative predictive value was 0.96 .Previous studies have reported that PET is a useful imaging method for differentiating between benign and malignant cartilaginous tumors –9. An SUaccuracy . Lee et max of 2.7, PET/CT was able to indicate malignancy. Consequently, a periosteal chondrosarcoma of grade II or III was diagnosed and wide resection was therefore carried out. In conclusion, PET/CT can distinguish periosteal chondrosarcoma from periosteal chondroma, even where differentiation of the conditions is challenging on the basis of size and radiographical findings.In the present case, the typical findings of periosteal chondrogenic tumor were identified by radiography, CT and MRI. However, none of these findings could clearly differentiate periosteal chondrosarcoma from periosteal chondroma. However, given the SUV


Myofibroma is a rare benign neoplasm occurring in the head and neck, arising primarily in infants and children. Frequently, myofibromas grow rapidly leading to suspicion of malignancy and the potential for overaggressive surgical excision. We aim to report a rare case of myofibroma with ulceration and bone destruction. A nine-year-old female presented with an ulcerated left hard palate mass. Open biopsy was performed with pathology suggestive of myofibroma. A left partial maxillectomy and reconstruction with a buccal advancement flap were performed. Final pathology confirmed the diagnosis of a benign myofibroma. Myofibroma is a rare benign tumor of the head and neck which must be considered in the differential diagnosis by the clinician and the pathologist in order to prevent inappropriate and/or overaggressive treatment. In the pediatric patient, the differential diagnosis of a mass on the hard palate includes inflammatory, congenital, and neoplastic conditions, in descending frequency . SpecifiWe present a case of a rapidly enlarging myofibroma of the hard palate which initially raised concern for malignancy due to bone destruction. Myofibroma is a rare benign neoplasm occurring in the head and neck, arising primarily in infants and children . We emphA nine-year-old female was referred to our department for a mass arising in the left hard palate  that hadA first biopsy was performed at an outside center and reviewed at out institution. The biopsy consisted of a small fragment of ulcerated squamous mucosa with a submucosal bland spindle cell proliferation. Due to the limited biopsy and lack of material to perform further immunohistochemical analysis, it was not possible to establish the nature of the tumor. Consequently, an open biopsy was performed and showed that the spindle cells were characterized by elongated cigar-shaped nuclei, eosinophilic cytoplasm with indistinct borders, and a storiform to fascicular growth pattern. Significant nuclear pleomorphism, increased mitotic activity, and necrosis were absent. The spindle cells showed variable immunoreactivity for muscle specific and smooth muscle actin and had a low Ki67 proliferation index (<5%); they were negative for desmin, S100 protein, cytokeratin (AE1/AE3), and nuclear beta-catenin. The overall findings supported the diagnosis of a myofibroma.The patient was brought back to the operating room for resection which involved a left partial maxillectomy and reconstruction with a buccal advancement flap Figures . The tumMyofibroma is a disease of the young, developing mainly in the first 2 years of life, but can occur in patients up to 40 years of age [When compared to the previously reported patients , our patDiagnosis is difficult on the basis of clinical, radiographic, and pathological features. Ulceration is a tumor characteristic that may be suggestive of a more aggressive biologic potential and can increase the clinician's suspicion for malignancy. In one review of the literature, of the 11 ulcerated tumors, 6 (54%) grew rapidly compared to just 4 (5%) of the 78 nonulcerated oral myofibromas . RadioloMicroscopic evaluation of the lesion with a proper use of immunohistochemical stains leads to the correct diagnosis. Myofibroma should be distinguished from other benign spindle cell tumors as leiomyoma, fibrous histiocytoma, fibromatosis, and nodular fasciitis. In addition, some myofibromas can be multifocal (myofibromatosis) and present worrisome features as local destruction, mitotic activity, and subendothelial growth, mimicking vascular invasion. Awareness of these microscopic features can avoid the confusion with smooth muscle, fibroblastic, or myofibroblastic sarcomas .The definitive treatment for a solitary myofibroma is conservative surgical excision, as myofibromas may regress spontaneously . If tumoIn conclusion, myofibroma is a rare benign tumor of the head and neck which must be considered to prevent inappropriate and/or overaggressive treatment. The vast majority of tumors will not recur with complete, conservative resection, even with positive margins, although close clinical follow-up is essential to diagnose recurrence.