A case of malignant mesenchymoma of the bladder containing leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, and myxomatous components is described. The primary pedunculated tumor measuring 14 × 13 × 7 cm and weighing 343 g arose from the left trigone of the bladder and was treated by total cystectomy. The histogenesis of malignant mesenchymomas and their optimal management strategy and prognosis remain uncertain. Herein, we present the fifth case of malignant mesenchymoma of the urinary bladder to be reported in the literature, which presented five unrelated differentiated tissues more than did previously reported cases. Malignant mesenchymoma, which was described by Stout in 1948, is defined as a malignant soft tissue tumor that consists of two or more distinctly different mesenchymal components in addition to fibrosarcomatous elements. A review of the literature revealed only four cases of bladder sarcomas that fit the criteria.A 77-year-old Taiwanese man had a multiyear history of symptoms of micturition difficulty. Gross hematuria with severe pain had developed suddenly in the previous month. The patient is a retired military soldier had neither history of cigarette smoking nor habbit of alcohol comsumption. He had a history of hypertension and type II diabetes mellitus for 20 years controlled with regular medication. He didn't exposures to paint components or eat undercooked meat. There is no specific illness could be traced from the patient's family pedigree. On physical examination, the patient appeared to be recently poorly nourished and had normal vital signs and mild elevated blood pressure at 151/88 mmHg. His lungs were clear, heart sounds were normal, and the abdomen was soft, with no masses and no tenderness. There was no edema of the legs. In laboratory data, the results of a complete blood count and the levels of electrolytes were normal. However, serum total protein, albumin, creatinine (Cr), urea nitrogen (BUN), and glucose were abnormal and showing hypoproteinemia (5.5 g/dL), hypoalbuminemia (3.0 g/dL), abnormal renal function with moderate azotemia (BUN/Cr = 36/1.3), and hyperglycemia (179 g/dL).Real-time sonographic evaluation of the abdomen showed increased echogenicity of bilateral kidneys. The sizes of the kidneys were 9 cm on the right and 10.3 cm on the left. Mild dilatation of left renal pelvis is also noted. Furthermore, one lobulated mass with urinary bladder wall thickening was also identified.Magnetic resonance imaging (MRI) of the pelvis demonstrated a large mass in the urinary bladder. The mass shows focal contrast medium enhancement on the left side with adjacent vesical wall thickening and obliteration of the lower signal intensity on T2WI Figure . UrinaryTransurethral resection of the urinary bladder tumor was performed. Pathological examination led to a diagnosis of high-grade sarcoma composed of myofibrous differentiation, focal myxomatous pattern, and a few chondroid changes with marked nuclear atypia and increased mitotic figures (more than 10/10 high power field). Radical cystectomy was performed. The patient currently remains well and prepares to undergo adjuvant chemotherapy.Macroscopically, the urinary bladder contained a nonencapsulated, huge, and lobular mass, which was located in the left trigone and measured 14 × 13 × 7 cm. On its cut surface, this tumor was composed of a predominant white, myofibrous-like component (70%), with myxomatous (20%), hemorrhage, and tumor necrosis (7%) foci. Several hard osteochondroid-like nodules were also palpable, and they measured around 3% of the whole tumor lesion Figure , and 1D.Microscopically, the tumor cells exhibited pleomorphic spindle tumor cells composed of predominant myofibrous differentiation with focal rhabdoid, chondroid, osteoid, and focal myxomatous differentiation. Nuclear atypia, focal clear cytoplasm, marked increased mitotic figures, focal hemorrhage, and tumor necrosis are also noted Figure . The spiPrimary sarcomas of the urinary bladder are uncommon and mostly originate from muscle such as rhabdomyosarcoma, which is dominant in children, or leiomyosarcoma, which is dominant in adults. Other rare sarcomas documented in the literature include primary osteosarcoma 31 cases) [ cases 1, primaryMalignant mesenchymoma is the rarest sarcoma of the urinary bladder, and it was defined by Stout as a sarcoma comprising two or more unrelated differentiated tissue elements other than a fibrosarcoma component. Our case presents five unrelated differentiated tissues other than fibrosarcoma. To our knowledge, in the English-language medical literature, only four cases of malignant mesenchymoma of the urinary bladder have been previously reported -7. The mMalignant mesenchymoma can occur at all locations in the body, including the retroperitoneum, soft tissue of the lower limbs, heart , mediastSeveral neoplasms remain that could qualify as malignant mesenchymoma according to the definition, but these are frequently treated as distinct and separate entities, such as malignant triton tumor, ectomesenchymoma, dedifferentiated liposarcoma, and chondrosarcoma with a second differentiated component .Malignant mesenchymoma appears to arise from a primitive mesenchymal cell with the capacity for totipotent differentiation, but the histogenesis remains uncertain.One should be careful when diagnosing malignant mesenchymoma, because some sarcomas are easily combined with bone and chondroid metaplasia that could mimic this diagnosis. The histological distinction between neoplastic and metaplastic bone is based on the pattern of the deposited bone, the cytological features of the bone-forming cells, and the cellular composition of the intratrabecular tissue. Metaplastic bone often has a lamellar architecture, and it is usually organized around areas of hemorrhage or portions of tumor. In contrast, the neoplastic osteosarcoma is usually surrounded by deposited osteoid material Figure .The prognosis of malignant mesenchymomas remains controversial. Malignant mesenchymomas are commonly accepted as high-grade malignant neoplasms. Bradythe had reported two- and three-year survival rates of 75% and 37%, respectively, in eight femoral and retroperitoneal cases . HoweverBecause of its extreme rarity, there are insufficient data to suggest the best modality or combination of treatments for this condition ,22,23. CIn summary, malignant mesenchymoma is a rare tumor, and this is the fifth reported case of a malignant mesenchymoma of the urinary bladder. Because of the limited experience with this extremely rare tumor, there are insufficient data to suggest the optimal management strategy and prognosis for malignant mesenchymoma of the urinary bladder.The authors declare that they have no competing interests.YFL drafted the manuscript. CPY carried out the immunohistochemical stains evaluation. STW and MSD provide clinical information. HSL supervised this manuscript. All authors read and approved the final manuscript.


Brain tumors, especially high-grade gliomas, can present with focal or generalized signs due to mass effect, parenchymal infiltration and destruction. In general, at the time of diagnosis, tumors could cause common neurological symptoms and major clinical signs depending on their localization. In rare instances, brain tumors colud be manifested with unusual symptoms.We describe three cases presenting with unusual clinical symptoms: ulnar neuropathy, vertigo and syncope attacks. Microscopic total tumor excision was done and histopathological analysis revealed that these tumors were glioblastoma multiforme. Both external beam radiotherapy and chemotherapy were given as adjuvant treatments.Physicians should keep brain tumors in mind in the case of patients who present with atypical symptoms such as those reported here. Brain imaging should be performed over a prolonged period following presentation if the patient’s symptoms remain unresolved after adequate treatment. Astrocytomas, including glioblastoma multiforme (GBM), are the most common malignant central nervous system (CNS) tumors in neurosurgical practice. GBM is t189A 42-year-old man was admitted with progressive tingling, paresis and hypoesthesia of his left hand through an orthopedic clinic. Electromyography and nerve conduction studies revealed that the velocity of the ulnar nerve was slowed between the elbow and 2 cm site above of elbow , even thN- acetylaspartate peak and creatinine [Figures A 46-year-old man presented with multiple syncope attacks. The patient felt faint and then passed out. The physician did not think that these drop attacks were secondary to seizure activity. Six months earlier, he had begun to notice intermittent headaches. He applied to our emergency department with confusion, a severe headache and left side hemiparesis and hemihypoesthesia. He was referred to a neurology clinic, and a right parieto-occipital intracerebral hematoma was detected on cranial MRI. Our differential diagnosis included intracranial malignancies,[ Figures  and b. T Figures  and b. TA 70-year-old woman presented with progressive and continuous vertigo for six months. She had complained about right-sided otalgia and bilateral hearing loss without tinnitus and applied to the ear, nose, and throat (ENT) clinic. The patient had a normal ear examination but audiometry demonstrated 60-70 dB moderate sensorineuronal hearing loss bilaterally. Neurological examination was normal except left-sided nystagmus. Cranial axial MRI with contrast revealed a 1.5 × 2 cm nodular tumor in the right lateral recess of forth ventricle . The tumPre-operative evaluation of patients with possible brain lesions requires a careful history and physical examination followed by radiological examination. Patients with high-grade gliomas (HGG) often present with headache, new-onset seizures, altered mental functions ,1014 unus1072711Despite the attempts to classify symptoms as focal or generalized and to determine GBM locations, clinical situations are nonspecific and unreliable. Therefore, physicians should keep brain tumors in mind in the case of patients who present with atypical symptoms such as those reported here. Consequently, brain imaging should be performed over a prolonged period following presentation if the patient’s symptoms remain unresolved after adequate treatment. High suspicion of GBM is mandatory in patients over 40 years of age who present with changes in neurological status.


Complete remission from advanced-stage synchronous double primary (SDP) esophageal and gastric adenocarcinoma by chemotherapy alone is rare. We report a case of advanced-stage SDP esophageal and gastric adenocarcinoma in which a complete response to treatment was obtained with S-1 and cis-diamminedichloroplatinum (CDDP).The patient was a 74-year-old man referred to our hospital complaining of dysphagia. Gastrointestinal endoscopy was performed and advanced-stage SDP esophageal and gastric adenocarcinoma diagnosed. Computed tomography revealed multiple regional lymph node metastases in the mediastinum. Neoadjuvant chemotherapy with S-1 and CDDP for advanced esophageal and gastric cancer was planned. An endoscopy following two courses of chemotherapy revealed that the esophageal cancer had been replaced with a normal mucosal lesion and the gastric tumor with a scar lesion; the results of biopsies of both were negative for cancer. Computed tomography revealed that the multiple lymph node metastases had disappeared. We diagnosed a complete response to S-1 and CDDP in advanced-stage SDP esophageal and gastric cancer. The patient is still alive with no signs of recurrence 22 months after the disappearance of the original tumor and metastatic lesions without surgical treatment.These results suggest that complete remission from advanced-stage esophageal and gastric cancer can be obtained with chemotherapy with S-1 plus CDDP. The standard treatment for advanced-stage esophageal cancer is esophagectomy. Despite advances in surgical technique, the prognosis of advanced-stage esophageal cancer is still poor . Additio2 per day for 21 consecutive days followed by a 14-day drug-free interval. Infusional CDDP was administered at a dose of 60 mg/m2 for 90 min on day 8. The patient developed only grade 1 gastrointestinal dysfunction in all the course of this chemotherapy. An endoscopy performed after two courses of chemotherapy revealed that the esophageal cancer had been replaced with normal mucosa and the gastric cancer with a scar lesion (Figures 2 per day for 28 consecutive days followed by a 14-day drug-free interval without surgical treatment. A periodically performed upper-gastrointestinal endoscopy performed in July 2011 revealed no new tumor lesions and CT has revealed no lymph node metastasis. As of September 2011, the patient is alive with no signs of recurrence at 22 months after the disappearance of the original tumors.The patient was a 74-year-old man who had previously been treated for diabetes, meningitis, hypertension and hyperglycemia by his family doctor. In October 2009, the patient was referred to the Department of Digestive Surgery, Nihon University School of Medicine Itabashi Hospital, complaining of dysphagia. An upper-gastrointestinal endoscopy revealed a mid-esophageal type I tumor measuring 2.5 cm × 2.5 cm Figure a and an  Figures b. We diaSeveral retrospective studies have reported that the complete response rate of advanced-stage esophageal cancer to chemotherapy is between 2.0% and 5.6% -12. In cChemotherapeutic regimens including S-1 have recently produced good clinical responses and survival benefits in patients with gastric cancer in Japan. These positive responses were also observed in patients with non-resectable advanced-stage gastric adenocarcinoma . The effGenerally, the initial treatment for esophageal cancer is either chemotherapy or surgical resection, with or without esophageal preservation. However, esophagectomy results in dysphagia more commonly than does chemotherapy . FurtherThis case confirms the potential for a complete response to S-1 and CDDP chemotherapy in patients with advanced-stage SDP esophageal and gastric adenocarcinoma. The accumulation of further such cases may enhance our understanding of this phenomenon and lead to the development of new treatment strategies for advanced-stage esophageal adenocarcinoma.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in- Chief of this journal.The authors declare that they have no competing interests.YM reviewed relevant literature and wrote the initial draft. MK wrote and reviewed the initial draft. MF provided clinical expertise and reviewed the manuscript. NK contributed the CT scans images. TK performed the chemotherapy and reviewed the manuscript. YM performed the chemotherapy and reviewed the manuscript. TF performed the chemotherapy and reviewed the manuscript. TM performed the chemotherapy and reviewed the manuscript. TT provided clinical expertise and reviewed the manuscript. All authors read and approved the final manuscript.


Lymphangioma is an extremely rare cause of scrotal swelling. We are reporting such a tumor in a one and half year old child presenting with a painless, progressive scrotal swelling. The mass was evaluated and excised completely. Histopathology confirmed it as Lymphangioma. Lymphangioma are congenital lymphatic hamartomas, 95% of which occur in neck and axilla [1]. The scrotum and retroperitoneum are unusual sites [2]. The scrotum is one of the rarest sites for lymphangioma. It can involve scrotal wall, tunics, testis, epididymis, spermatic cord or Colle’s fascia [3]. This is a report of scrotal lymphangioma with special emphasis on clinical, ultrasonographic, and histopathological findings. An 18-month-old male child presented with painless, progressive enlargement of left hemiscrotum for last one year. Examination revealed a non-tender and soft mass in left hemi scrotum which was compressible with positive transillumination test. The left testis could be felt separately from the mass . Patient underwent surgery after routine investigations. At operation a multilocular cystic mass measuring 5cmx5cm adherent to the tunics found. The left testis and spermatic cord were not involved . Complete excision of the mass along with overlying skin was done. Suction drain kept at surgery was removed on 2nd postoperative day. Child was discharged on 3rd postoperative in good health. On histopathological examination, it was reported as cystic lymphangioma. No recurrence was noted at one year follow up. Lymphangioma results from inadequate drainage of lymph from sequestrated lymphatic vessels and are considered to be lymphatic hamartomas. Most of these are congenital due to paucity or atresia of the efferent lymphatics or lack of communication between lymphatics and venous channels [1]. These lesions can also be acquired because of obstruction of lymphatics after inflammation, trauma or degeneration [3]. Histologically, three types – capillary, cavernous and cystic are described. The cystic form is the most common variety. Half of these are recognized at birth, 90% are evident by the age of 2 years and 95% occur in the neck or axilla. Unusual sites are scrotum, retroperitoneum, intraperitoneum, gluteal region, mediastinum, groin, pelvis, mesentery, omentum and spleen . Scrotum is a rare site for lymphangioma. Singh et al reported thirty two cases of cystic lymphangiomas in children, and only one was located in the scrotum [1]. Loberant et al estimated that less than fifty cases of scrotal cystic lymphangioma have been reported in literature till 2002 [6]. When scrotum is involved, it is usually misdiagnosed as hernia, hydrocele, varicocele, large epididymal cyst or acute scrotal conditions which may lead to inadequate treatment with a risk of recurrence . Hurwitz et al reported seven cases of scrotal cystic lymphangioma over a period of ten years, all of which were misdiagnosed preoperatively [7]. Ultrasound scan usually shows a multicystic extra testicular mass with internal septae; internal echoes in the cysts due to debris and/or hemorrhage may be noted as a result of complication . The treatment consists of surgical excision of the entire mass along with the overlying skin. Incomplete excision can result in recurrence [7]. Scrotal lymphangioma though rare must be kept in mind while exploring any scrotal swelling.Source of Support: NilConflict of Interest: None declared


Background. The risk of ovarian cancer is increased in the association of ovarian tumor, ascites, and hydrothorax with the significant elevated tumor marker CA-125. However, this association can be observed in a rare clinical and benign pathological entity, that is Demons-Meigs' syndrome. Objective. To describe a rare case of Demons-Meigs' syndrome observed in our department. Methods. A black African woman of 35 years old, seventh gravida and fourth parous, underwent a total abdominal hysterectomy with bilateral salpingoophorectomy for large bilateral ovarian masses associated with significant ascites, bilateral pleural effusion, and particular highly elevated tumor marker CA-125 (1835 UI/mL) in a pronounced general alteration condition. Results. The postoperative course was uneventful characterized by a complete remission of hydrothorax and ascites with normal level of CA-125 three months after tumor excision. Histology of both masses revealed a bilateral ovarian fibrothecoma, a benign tumor of the ovary, thus confirming the diagnosis of Demons-Meigs' syndrome. Conclusion. The Demons-Meigs syndrome, although it strongly mimics the clinical picture of malignant metastatic ovarian cancer, remains a disease with benign prognosis after surgical tumor resection. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices. The Demons-Meigs syndrome (DMS) in its initial typical form is a pathology that combines ovarian fibroma or fibrothecoma, ascites, and hydrothorax . CompletWe report the first documented case of a rare Demons-Meigs syndrome in our country associating bilateral fibrothecoma, ascites, and bilateral pleural effusion with significant elevation of the tumor marker CA-125.The patient, Z.A.S, a 35-year-old black African woman, housewife, seventh gravida and fourth para, with 5 living children, was received at the gastrointestinal surgery emergency of the University Hospital of Cocody  on 25/05/2012 for abdominopelvic pain and abdominal mass. The beginning dates back to about a year by the occurrence of progressive abdominal distension in a context of amenorrhea and asthenia. Abdominopelvic pain is of recent onset (approximately 10 days prior to the patient admission) associated with a dry cough and difficulty breathing.On admission, his general condition was altered. She was emaciated, dyspneic, and very asthenic. Conjunctivae were slightly colored, without jaundice. She had no edema or collateral venous circulation. Taking constant gave blood pressure = 10/06 Cmmhg, pulse = 120 bpm, breathe frequency = 22 cycles/min.upper abdomen. On vaginal examination, it was difficult to relate the mass to the uterus or annexes. Clinical examination also noted bilateral pleural effusion. Heart sounds were regular without breath.Physical examination showed an abundant ascites and a large abdominopelvic mass, diffuse, solid, mobile, and slightly painful in its entirety with increased pain in the pelvis. This mass extended from the pelvis to the The chest radiograph  performeGiven the importance of dyspnea, pleural paracentesis (500 mL) and ascites (4.5 liters) were performed in emergency. Microscopic and chemistry study of both effusions was in favor of a transudate without any suspected malignancy cells.The biological study of the liver and kidney was normal. A complete blood count was noted anemia to 7.9 g/dL. Tumor markers were measured: CEA (carcinoembryonic antigen) = 4.77 ng/mL ; serum CA-125 level = 1835 IU/mL, more than 60 times above the normal superior limit ; AFP  = 8.1 ng/mL ; ECG (electrocardiogram) was normal.Given the importance of the alteration of the general condition and particular high elevated serum CA-125 with normal levels of other markers, the probable preoperative diagnosis of ovarian cancer was retained despite the lack of evidence of ovarian carcinoma at CT scan. After multidisciplinary consultation, an exploratory laparotomy was decided and performed on 03/06/2012 one week after the patient's admission.Intraoperatively after a midline incision under and above the umbilicus, we find a 2.5 liter of ascites and two large ovarian masses Figures : a rightWe performed a total hysterectomy with bilateral salpingoophorectomy. A blood transfusion of 1 liter of packed cells was required associated with analgesics. The postoperative course was uneventful without ascites reconstitution and the patient was discharged to J 12. Histological analysis of the two surgical specimens whose result is achieved 28 days later after the intervention concluded a double ovarian fibrothecoma which is a begnin tumor Figures .The patient was revisited in consultation each month until the 3rd postoperative month. Clinical examination noted a lack of pleural effusion confirmed by chest radiography and absence of ascites by ultrasound postoperative control. The level of serum CA-125 was normal at last control. It then concluded at a Demons-Meigs syndrome.The Demons-Meigs syndrome typically though not exceptional is a rare clinical and pathological . AlthougOvarian fibroma is a benign tumor most frequently observed in this syndrome in the order of 80–85% and may be pure and nonsecreting or associated thecoma elements (fibrothecoma) sometimes responsible for estrogen secretion. Thecomas and fibrothecomas represent 10% of cases , 4. FibrThe prevalence of DMS begins to increase in the third decade and increases progressively to peak in the seventh decade. Although the syndrome may affect women of any age in both full genital activity (as our observation) and in prepubertal period . The DemDespite the many cases of the Demons-Meigs syndrome or pseudo-Meigs syndrome published in the literature, the mechanisms of formation of peritoneal and pleural effusions composing this syndrome remain unclear and speculative. Several theories attempt to explain the etiology of these two elements.In DMS, ascites is present in 10–15% of cases and hydrothorax is found in only 1% of cases , 7–9. AsThe hydrothorax is mostly unilateral and occurs most often on the right-sided (75%), rarely left-sided, but can be bilateral as in the observation that we present.Indeed ascites once formed would spread through the diaphragm or via the lymphatic vessels of the foramen of Bochdalek in the chest cavity, more developed in the right than the left explaining the frequency of right-sided pleural effusion. Blockage of these lymphatics prevented the accumulation of pleural fluid and caused an increase in ascetic fluid. However, the minimum volume of ascites that can induce hydrothorax is not known , 5 thinkAscitic and pleural fluids in Demons-Meigs' syndrome are similar in nature. It may be either transudative or exudative with a high frequency of exudate. The elevation of the tumor marker CA-125 is a confounding factor for that the CA-125 is increased in 80% of epithelial ovarian cancers but also in other malignancies and benign tumors . Let us The circumstances of discovery are variable and were the consequences of elements of the triad syndrome . It can be a tension with abdominal bloating and weight gain or weight loss, respiratory distress associated with a cough or abdominal pain secondary to adnexal torsion as in the case we describe. A more or less pronounced alteration of the general condition can be observed. It may be a fortutious discovery during a routine gynecological examination, especially when the patient is asymptomatic.Physical examination will typically find ascites, pleural effusion, and abdominal mass of variable importance. In the literature, most patients present an ovarian asymptomatic mass, solid, and unilateral, most often left-sided whose dimensions can be large , 12. In Chest radiography confirmed hydrothorax while abdominopelvic ultrasound confirms the presence of a mass and ascites. The abdominopelvic CT scan specify the ovarian origin of mass and no signs of distant metastases. In our case, the CT scan did not diagnosis with certainty the organs which depend on the masses. Laparoscopic exploratory, which is still a luxury in our context, is reserved for masses of small dimensions. CA-125 can be increased but is not a diagnostic element because it is not specific to ovarian origin in the context of ascites; cytology of ascites and hydrothorax does not show any cells suggestive of malignancy .In our case, such elevated rate of CA-125  with pronounced impairment of general condition and a tumor filling the whole abdominal cavity associated with serous effusions imitated advanced malignant ovarian tumor. In fact, any ovarian tumor with ascites and serous effusions should be considered malignant until proven otherwise histologically. Thus, the preoperative diagnosis of Demons-Meigs' syndrome or pseudosyndrome is difficult because of the clinic .Medical treatment is purely symptomatic and includes thoracocentesis and draining of ascites. Blood transfusion was necessary in our patient because of her preoperative anemia. Curative treatment is surgical by laparotomy in almost all cases –14. IndeThe Demons-Meigs syndrome, although it strongly mimics the clinical picture of advanced ovarian cancer, remains a disease with benign prognosis after being properly managed. It should be considered systematically to all women presenting with a pelvic mass whatever the size or number associated with ascites and unilateral or bilateral hydrothorax with or without elevated CA-125 and in whom there is no concept of heart failure, nephrotic syndrome, or liver failure. The importance of general signs should not exclude the surgery. The histology of the tumor should confirm the syndrome but complete and definitive remission after surgery is also an important element of diagnosis. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices.


A 4-year-old boy presented with vomiting and recurrent cough. He was investigated and found to have thoracic oesophageal duplication cyst. He was taken up for thoracoscopic removal of the cyst. The cyst was attached to the oesophagus and shared a common wall. The boy tolerated the procedure well and follow-up showed no recurrence of the cyst with total resolution of the symptoms. We share our experience with the management of this boy. Developmental disorders of ventral budding of the lung primordium from the embryonic foregut may present as cystic duplications or diverticuli of the bronchus or oesophagus.Resection of mediastinal cysts is often indicated for definitive diagnosis, to alleviate symptoms and to prevent the development of associated complications.Open resection by thoracotomy has been the only definitive treatment for these lesions in the past, but in view of their benign histology, a less-invasive approach is desirable. Growing experience and improved instrumentation for small patients have broadened the repertoire of procedures that can be accomplished by paediatric minimal-access surgery (MAS).There have been previous reports on video-assisted thoracoscopic resections of benign mediastinal cysts. However, there has only been few reports on the resection of an oesophageal cyst using this approach.2 We haveA 4-year-old boy was previously well presented to us with 3-month history of vomiting and intermittent cough. Chest X-ray films showed a mild haziness in the right cardiac border. A CT scan of the thorax revealed a well-circumscribed cystic mass closely attached to the oesophagus, just above the diaphragm . The masWith the use of general anaesthesia and endotracheal intubation, the patient was placed in the full left lateral decubitus position. A nasogastric tube was put into place. The patient was prepared and draped as for a thoracotomy. A 10-mm 30º telescope was inserted through the fifth intercostal space at the posterior axillary line. Two instrument ports were created, at the fourth intercostal space, below the tip of scapula and at the seventh interspace just in front of the posterior axillary fold . The medVentral budding of the lung primordia from the foregut occurs at 3-4 weeks of gestation. Aberrations in this process during this or subsequent stages of development may result in duplications of the oesophagus or bronchi. Simple oesophageal or bronchial duplication cysts are most commonly found in the mediastinum.3 The commThe diagnosis of bronchogenic cyst cannot be made solely based on a lung radiograph. In 69% of cases, it requires a CT scan and in 100%, it depends on an MRI that enables distinction between cystic and tissue mass. Precise These cysts are histologically benign and some controversy exists regarding the need to excise asymptomatic cysts in adult patients. Complete excision is the preferred treatment in children because of the high risk of obstructive respiratory problems, and also because these cysts do not regress spontaneously and occupy space destined for growing respiratory tissue. Malignant transformation, although rare, has been described in children and adults. Our patiet al, in their report of eight cases in children ranging from 3.5 months to 8 years had an average operating time of 106 min with six cases having complete cure and all cases discharged by 3 days with minimal pain. One patient had a recurrence due to incomplete resection which was managed by open thoracotomy and one patient developed pneumothorax as chest drain was not inserted postoperatively. The patient recovered with the insertion of chest drain. They conclude from this experience that many simple foregut duplications may be excised safely by MAS in children. Two technical points are noted: (a) central mediastinal lesions require chest-tube drainage postoperatively; and (b) complete excision is required to avoid recurrence.[et al, in a series of six cases of foregut duplication cyst managed thoracoscopically report short operating time, improved cosmesis and less pain obviating the need for narcotics. In their series most patients had a short hospital stay with tube thoracostomy required for less than 12 hours. They suggest thoracoscopic resection should be considered the standard of care for resection of these benign cysts.[Treatment of thoracic duplication cyst consists primarily of excision, and this may be accomplished thoracoscopically, although this may not be always possible. For thoracoscopic approaches, at least three and occasionally four ports are required. Reports 10currence. Hirose egn cysts.Technically they are best approached through the side of the thorax into which they protrude. Oesophageal duplications are often surrounded by oesophageal muscle which should be incised longitudinally to expose the cyst. DecompreCyst in the wall of the oesophagus can be removed by opening the oesophageal muscle and performing an extramucosal excision of the cyst, leaving the oesophageal mucosa intact. After thoracoscopic removal the surgeon must be sure that the oesophageal lumen was not entered by inserting fluid or air in the lumen. An unrecognized oesophageal leak will lead to mediastinitis and severe morbidity. AspiratiFor thoracoscopic procedures, single lung ventilation will often facilitate the resection. Double lumen endotracheal tubes are available down to 26 French calibre, which may be used on children of over 25 kg. In smaller children or infants, mainstem intubation of the contralateral bronchus or placement of a balloon catheter  will allow deflation of lung to facilitate exposure. Selective ventilation is most useful when the lesion is located entirely in the parenchyma and less useful when it is mediastinum.In cases where the resection is incomplete electrocoagulation or laser destruction of the residual wall can be used to destroy the patent pathological cells, thereby significantly reducing the recurrence risk.69 Carefu6Thoracoscopic resection of an oesophageal cyst in a young child is technically feasible, associated with minimal chest wall trauma and has a favourable outcome.


Idiopathic orbital pseudotumor is a benign, noninfectious, and nonneoplastic disease with unknown cause. It is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorder. Idiopathic orbital pseudotumor is extremely rare in pediatric age group and may cause real diagnostic problems. This paper describes a 4-year-old girl who presented with sudden ptosis in the right eye and swollen eyelid. She recovered completely with high-dose steroid therapy. We report clinical and magnetic resonance imaging findings of orbital myositis, which is a rare subtype of idiopathic orbital pseudotumor in children and needs to be differentiated from other orbital disease especially malignancy. Idiopathic orbital pseudotumor (IOP) is a nongranulomatous inflammatory process in the orbit with no known local or systemic causes. After Graves' disease and lymphoproliferative disorders, orbital pseudotumor is the third most common ophthalmologic disease of the orbit and accounts for approximately 8–11% of all orbital tumors. The most frequent subtype of IOP is a local mass within the orbit (50%), followed by dacryoadenitis 29%), myositis (8%), perineuritis 4%), eyelid pseudotumor (4%), and diffuse orbital inflammation (4%) 9%, myosi. Magneti%, eyelidA previously healthy and normally developing 4-year-old girl presented to our pediatric clinic with a 2-day history of painless, gradually progressing ptosis and swollen right eyelid. Visual acuity was 20/20 in her both eyes. Ophthalmological examination of the patient was normal other than upper eyelid ptosis, proptosis, and ophthalmoplegia in the right eye. She was not able to use her left leg and left arm at admission. For this reason, cranial computed tomography (CT) was performed to check for intracranial hemorrhage or mass and turned out to be normal. Complete blood count, electrolyte levels, and sedimentation rate were in normal ranges. There was no atypical cell in peripheral blood smear. In orbital MRI examination, postgadolinium fat-saturated T1-weighted images revealed marked contrast enhancement of medial  and infeFirst described by Birch-Hirschfield in 1905, idiopathic orbital inflammatory syndrome, also known as IOP, is a nonspecific, nongranulomatous inflammatory process of the orbit with spontaneous resolution . OrbitalRadiologic findings in IOP are characterized by inflammatory changes in various intraorbital structures, such as the globe, lachrymal glands, extraocular muscles, orbital fat, and optic nerve . MRI is Some of the differential diagnosis of pediatric IOP may include orbital cellulites, rhabdomyosarcoma, leukemia, orbital trauma with retained foreign body, ruptured dermoid cyst, lymphangioma, neuroblastoma, metastatic retinoblastoma, and thyroid related orbitopathy . EnlargeIOP can mimic malignant tumors both clinically and radiologically. Both IOP and lymphoid tumors appeared isointense to muscles on T1-weighted images. Patients with IOP show isointense and lymphoid tumors hyperintense to fat on T2-weighted images. Also IOP patients reveal enhancement with contrast more prominent than observed in lymphoma cases , 13.Systemic corticosteroid therapy is the cornerstone of managing IOP. Similar to our case, over 75% of patients show dramatic improvement within 24 to 48 hours of treatment . ImproveIn conclusion, IOP is not common in pediatric age group, and extraocular muscle involvement by itself is rare. Awareness of MR imaging features of IOP provides a better differential diagnosis and prevents unnecessary biopsies. Follow-up MRI examination, performed shortly after the initiation of steroid treatment, is necessary in order to reveal the improvement of the findings and confirms the suggested diagnosis.


We report a case of idiopathic tumoral calcinosis localized to the thumb without prior trauma or surgery. Initial physical examination and imaging studies were suggestive of more common etiologies of thumb pain. After treatment failure, a biopsy specimen revealed calcium phosphate salt deposition in the soft tissue around the metacarpophalangeal joint, which was treated by excision of the tumoral calcinosis masses. Tumoral calcinosis can occur idiopathically in the hand and digits and should be considered when other more common pathologies of thumb pain have been ruled out. The list of the most common diagnoses for thumb pain is generally short and straightforward. However, when the nontraumatic thumb pain patient's diagnosis is not osteoarthritis, deQuervain's tenosynovitis, gout, or trigger thumb, the frequency of occurrence of other conditions on the differential diagnosis drops precipitously. While infrequent, less prevalent disease processes are important to consider when the clinical scenario is not consistent with an expected etiology. We present a case of an uncommon thumb pain diagnosis: idiopathic tumoral calcinosis.First described by Giard4With physical findings that can present similarly to cellulitis or synovial chondromatosis, diagnostic interpretation of tumoral calcinosis can be easily misconstrued. A missed diagnosis can start the patient on a clinical treatment cycle that delays proper intervention. We present a case of idiopathic tumoral calcinosis of the thumb with explanation of the diagnostic process and management. This case adds to a very limited supply of previous reports of tumoral calcinosis localized exclusively to the thumb without prior trauma or surgery.5A 31-year-old right hand dominant, African American female presented to the University of Chicago Emergency Department with swelling and progressive pain of her right thumb (first digit) that worsened with activity. She first encountered pain in her previously healthy thumb 2 weeks prior to presentation. She denied trauma and surgery to the affected digit. On physical examination, the digit was erythematous and moderately tender to palpation. Motor function was grossly intact but range of motion (ROM) at the metacarpophalangeal (MP) and interphalangeal joints was reduced because of pain. No neurovascular deficits were noted. There were no palpable masses. The patient was afebrile and a complete blood count and basic metabolic panel were within normal limits. X-rays of the digit revealed radio-opaque masses around the volar aspect of MP joint consistent with synovial chondromatosis without evidence of acute fracture (Fig After 24 hours, physical examination revealed moderately decreased erythema and swelling and improvement of thumb ROM. Severe thumb pain persisted. A hand surgery consult was requested, and CT scan of the hand was obtained. Consistent with the initial x-rays, foci of mineralization adjacent to the thumb MP joint were noted (Fig During surgery, access to the masses was achieved by making an L-shaped incision transversely along the digital-palmar crease of the thumb and extending it distally up the radial mid-axial line. The radial and ulnar neurovascular structures were identified during subcutaneous dissection and retracted to the periphery of the incision. Approach to the MP joint was performed at the radial border and dissection was carried down between the abductor pollicis brevis and the flexor pollicis brevis. During exposure of the intermuscular plane, a thick, yellow-tinted fluid extruded into the surgical field. The fluid was aspirated and microbiological examination and gram stain revealed unidentified debris without evidence of bacteria or leukocytes. Deeper dissection revealed multiple firm, irregularly shaped masses adjacent to but not penetrating the MP joint capsule Fig . These mGALNT3, FGF-23, and Klotho. As illustrated by the current case, the condition can also occur in patients without a positive family history.Tumoral calcinosis most often affects young African American adults and can follow both autosomal dominant and recessive genetic patterns. Familial forms of tumoral calcinosis are linked to mutations of various genes including Tumoral calcinosis is characterized by calcified nodules that progressively enlarge and encase normal adjacent structures as occurred here. Its pathogenesis is thought to stem from dysregulation of phosphate metabolism.In general, nodules are well-demarcated and usually occur at joint extensor surfaces. Consistent with the presentation of our patient, pain, swelling, and decreased ROM at the affected joint are commonly reported. Though generally insidious in nature, tumoral calcinosis can mimic acute infection.Radiographically, tumoral calcinosis lesions appear as rounded, dense periarticular opacities. Masses may be small and solid or large and cystic on CT. Despite a large amount of calcification, masses usually display high signal intensity on T2-weighted MRI images.2Histologically, the two conditions demonstrate distinct differences. While calcification of periarticular masses is an obligatory finding of tumoral calcinosis, it may be absent in patients with synovial chondromatosis. Conversely, chondrocyte aggregation is requisite for the diagnosis of synovial chondromatosis but is not found in cases of tumoral calcinosis. An additional clue suggestive of synovial chondromatosis is the radiographic presence of calcific lesions in a ring-and-arc morphology, which is indicative of the presence of cartilage.The recommended management of masses caused by tumoral calcinosis is surgical excision.Few conditions result in most cases of thumb pain; however, it is important to consider less common disease processes when initial treatment fails. Though tumoral calcinosis may be suspected based on presentation alone, histological evidence of calcium phosphate deposition is required for diagnosis. In cases where synovial chondromatosis is also suspected, we believe that biopsy is essential to prevent inappropriate definitive treatment. Preservation of the joint capsule during lesion excision and early intervention of hand therapy are key to ensuring full functional return of digits.


Peripheral cemento-ossifying fibroma is a reactive gingival overgrowth occurring frequently in anterior maxilla. It is a slow-growing benign tumor which may lead to pathologic migration and other periodontal problems, so it should be excised as soon as possible. The recurrence rate of peripheral cemento-ossifying fibroma is reported to be 8% to 20%, so a close postoperative follow-up is required. Herein, we are reporting a similar case of peripheral cemento-ossifying fibroma in the maxillary anterior region. Ossifying fibroma is a benign neoplasm arising in craniofacial bones, composed of proliferating fibroblasts with osseous products that include bone and ovoid calcifications; these lesions are well demarcated from the adjacent bone.There are two types of ossifying fibromas: the central type and the peripheral type. The central type arises from the endosteum or the periodontal ligament adjacent to the root apex and causes expansion of medullary cavity. The peripheral type occurs solely on the soft tissues covering the tooth-bearing areas of the jaws.In 1872, Menzel first described ossifying fibroma; but only in 1927, Montgomery assigned a terminology to it.It occurs exclusively on the gingiva and accounts for 3.1% of all oOssifying fibroid epulis; peripheral fibroma with calcification; peripheral cemento-ossifying fibroma; and calcifying fibroma are the terms which refer to peripheral ossifying fibroma.Peripheral cemento-ossifying fibroma appears as a nodular mass, either pedunculated or sessile. It most commonly appears to originate from interdental papilla. The color ranges from red to pink, and the surface is frequently but not always ulcerated. It is more commonly seen in the first and second decades of life and has a female preponderance. There is a slight predilection for the maxillary arch 60%) and the incisor cuspid region (50%). A potent0% and thA 45-year-old woman with the chief complaint of painless swelling in upper right front region reported to the Department of Periodontics, Institute of Dental Sciences, Bareilly. She reported that the lesion was present for the last 7 months. It was of pea size when it started, and gradually it increased to attain the present size.On extraoral examination, swelling was present on the right side, extending from philtrum to angle of mouth .Intraoral examination revealed a gingival overgrowth in relation to interdental papilla between 11 and 12 of approximately 1×1.5 cm in size, pedunculated, with color same as that of adjacent gingiva. Surface was smooth with few areas of ulceration.Teeth associated were grade II mobile and pathologically migrated .Radiographic examination revealed moderate amount of bone loss in relation to 11 and 12 in both intraoral periapical and occlusal radiographs Figures  and 4. RThe patient had no systemic problem, and surgery was planned on the basis of the clinical and radiographic examinations 7 days after thorough oral prophylaxis. After extraoral and intraoral antisepsis, local anesthesia was given. Excision of the lesion was done, followed by curettage of the area and scaling of the involved teeth. Periodontal dressing was placed Figures –7. The pThe tissue excised was sent to the Department of Oral Pathology for histopathological examination.Histopathological report revealed parakeratinized epithelium, dense fibrous connective tissue stroma which comprised of plump-to-stellate fibroblasts, along with spindle-shaped fibroblasts. Connective tissue also showed large hematoxyphilic areas of calcification/osteoid with few areas showing entrapped osteocytes and peripheral lining of osteoblast-like cells. Focal areas of inflammation were seen in connective tissue, mainly comprising of lymphocytes and few plasma cells .The patient was recalled every third month for maintenance therapy and to check for possible recurrence .Peripheral cemento-ossifying fibroma is a focal, reactive, non-neoplastic tumor-like growth of soft tissue, often arising from the interdental papilla. A lot ofet al. to distinguish cementifying fibromas from ossifying fibromas and fibrous dysplasias by using immunohistochemical analysis for keratan sulfate and chondroitin-4-sulfate, in which the cementifying fibromas showed significant immuno-reactivity for keratan sulfate, and ossifying fibromas and fibrous dysplasias showed intensive immunostaining for chondroitin-4-sulfate.[Cementifying fibromas may be clinically and radiographically impossible to separate from ossifying fibromas. An attem-sulfate.Though the etiopathogenesis of peripheral ossifying fibroma is uncertain, an origin from cells of the periodontal ligament has been suggested. The reasons for considering periodontal ligament origin for peripheral ossifying fibroma include exclusive occurrence of peripheral ossifying fibroma in the gingiva , the proximity of gingiva to the periodontal ligament, and the presence of oxytalan fibers within the mineralized matrix of some lesions. ExcessivLesions involving the gingival soft tissues are rare compared to the lesions appearing within bone. MesquitaThe reported gingival overgrowth has been clearly diagnosed as peripheral cemento-ossifying fibroma after histopathologic examination. Clinical picture of less vascular growth rules out the possibility of pyogenic granuloma.Histopathology showed no presence of giant cells in connective tissue stroma, thus ruling out the possibility of peripheral giant cell granuloma.Furthermore, peripheral cemento-ossifying fibroma tends to occur in the second and third decades of life, with peak prevalence between the ages of 10 and 19 years. Almost two thirds of all cases occur in females, with a predilection for the anterior maxilla, i.e., incisor canine region. In the present case, the findings correlate with the general characteristics except for age. The size of the peripheral ossifying fibroma ranges from 0.4 to 4.0 cm. In the present case, the dimensions of the lesion were well within the above-mentioned range. Further, the diagnosis was confirmed by histopathologic evaluation.Lesion was successfully treated, and follow-up was done at 8 months to check for any recurrence.Peripheral cemento-ossifying fibroma is a slowly progressing lesion, with limited growth. Close postoperative follow-up is required because of the growth potential of incompletely removed lesions, as well as 8% to 20% recurrence rate.[


Synovial sarcoma (SS) is a rare sarcoma with distinct morphologic and genetic features, which, despite its name, does not arise from synovium. While most SSs (>80%) arise in the deep soft tissue of the extremities, up to 5% of these tumors are encountered in the body axis including the spine, mediastinum, retroperitoneum, and head/neck regions. Reports of SS located within the spinal axis have been rare to date.We searched the medical records at our institution and found three patients who were diagnosed and treated for SSs involving the spine. We also performed an exhaustive literature search using PubMed to identify all reported cases in the literature.In this study, we report on three SS cases involving the spine. All three cases involved the paraspinal muscles and spinal nerve roots, with one case having a significant leptomeningeal involvement. In two cases, “smaller operations” were performed first because the lesions were thought to be benign, however, when the final pathology identified them as SSs, more radical procedures were performed. Additionally, we identified 14 cases of SSs involving the spine published in the literature and all cases are reviewed here.Due to limited numbers of cases, spine SS long-term outcomes are hard to quantify. The currently accepted standard of treatment for SSs starts with wide surgical excision with negative margins followed by chemotherapy and radiation. We summarize the available literature on spinal SSs and review the current treatment options available for these tumors. Synovial sarcoma (SS) is a rare soft tissue tumor comprising 5–10% of soft tissue sarcomas and less than 1% of all malignancies.71630 SS 716769142530614Surgical excision with wide, negative margins is the currently recommended treatment with adjuvant radiotherapy and/or doxorubicin-based chemotherapy. While th66Reports of SS arising from, near, or metastatic to the spine are rare and difficult to find in the journals. In this article, we present the experience at our institution with these cases and review the available literature.synovial sarcoma, spine, paraspinal, cervical, thoracic, or lumbar and identified three patients diagnosed and treated for spinal SS since 2004. We also searched PubMed at http://www.ncbi.nlm.nih.gov/pubmed/ using the same keywords and found 14 cases reported in the available literature.We searched the patient and surgical pathology databases at our institution using the keywords A 59-year-old woman presented with a two-year history of constant, progressively worsening, left-sided upper thoracic pain. In the month prior to presentation, she also experienced ascending paresthesias from her toes to the T5 dermatome along with gait weakness and instability. Imaging studies elsewhere revealed a dumbbell-shaped upper thoracic mass emanating from the T5 foramen with extensive encasement and compression of the thoracic cord from approximately T4 through T6. Additionally, the tumor appeared to extend into the T5 vertebral body. A CT-guided biopsy of the mass was performed and was diagnosed as a “spindle cell tumor consistent with schwannoma.” Since her symptoms continued to progress despite treatment with steroids and radiation, she was referred to our institution for further treatment including surgical resection.Following a lengthy discussion, the patient chose to undergo partial resection of her lesion. The tumor was exposed through a T3-T6 laminectomy and an extensive epidural mass was noted dorsally between T4 and T5 and extending out the T4-5 foramen, infiltrating the T4-5 facets and encasing the T4 nerve root. The tumor was grossly debulked, and the final pathology report revealed a high-grade SS . The patThe adjuvant therapy had stabilized her disease and 7 months following her initial evaluation she underwent a second-staged operation. A one-piece gross total resection of T4, 5, 6, and 7 vertebra with a posterior instrumented spinal fusion from T1-L1 was performed. The tumor was removed with negative margins. The patient recovered well from the operation. She was seen on a 6-month basis by oncology with no recurrence of her tumor noted and improving symptoms. A year and a half later, she returned, complaining of new-onset back pain in her right scapular region and left mid back. This pain was found to be attributed to a fractured rod, which was surgically repaired. She continues to be followed and she has no evidence of tumor recurrence or metastases 67 months after the final resection.A 54-year-old woman presented with several months of pain in her right buttock and hip, paresthesias of her right leg, and generalized right leg weakness. Her symptoms progressed, involving her left leg in a similar fashion and a sensory level at the costal margin was found bilaterally. Over the last month, she noted saddle numbness and loss of bladder and bowel control. Spine MR showed a large, right-sided, paraspinal mass centered around T10 with adjacent leptomeningeal enhancement—no spinal cord compression was noted . A CT-guThere was some question if the leptomeningeal enhancement was associated with the paraspinal mass or was an unrelated disease process. The location of maximum leptomeningeal enhancement was at the T12-L1 junction. A T12-L1 laminectomy was performed and the underlying thickened arachnoid was biopsied. The leptomeninges demonstrated the presence of a spindle cell tumor similar in morphology to the one seen in the epidural CT-guided biopsy. The tumor cell morphology suggested the diagnosis of monophasic SS, a diagnosis confirmed by immunohistochemical and RT-PCR studies . The tumThis 32-year-old woman presented with 8 years of progressive right-sided subscapular thoracic pain incited by lifting her arm above her head. This pain became more frequent and began to occur spontaneously, disrupting her sleep and causing her significant difficulties. Two MRI studies were performed during this time, the first in 2004 and the second in 2009, and she was told that the findings were unremarkable. When she presented to our institution, she had severe, mechanically reproducible pain located several inches to the right of her mid-thoracic spine. The pain also radiated both up to the axilla as well as down the right leg. A review of outside MRI studies revealed a lobulated, T1 isointense, mildly T2 hyperintense, 3 cm enhancing mass involving the right T5 nerve root through the foramen with extension into the paraspinal muscles . These iAs it was felt that this symptomatic lesion was likely benign and easily accessible, resection was advised. Intraoperatively, the mass was identified in the erector spinae muscles of the T4-7 region on the right side. The tumor was mobilized and gross total resection achieved, including the foraminal component. However, intraoperative pathology revealed a spindle cell sarcoma rather than a benign process, necessitating extension of the surgical resection. A T5-6 laminectomy was then performed, the dura incised, and the entire nerve root in the foramen of presumed origin was resected until negative dural margins were achieved. After the dural closure, all grossly visible tumor appeared to have been removed from the region and surrounding tissues. Final pathology showed a high-grade, fibrous-type, monophasic SS. The patient tolerated the surgery well and was neurologically intact.Concern for residual tumor remained as postoperative imaging studies demonstrated abnormal T5-6 transverse processes. To achieve a complete resection with negative margins a second surgery was undertaken where a one-piece gross total resection was performed of the entire tumor bed. Both transverse processes were resected and gross residual tumor was found in the T6 transverse process. Wide margins were excised, and the pathologist entered the OR to correlate the negative margins with the visualization of the previous tumor bed. Based on these findings, it appeared that the resection had removed all residual viable tumor present. The patient tolerated the second operation well.At 6-month follow-up, no local tumor recurrence was found, but several small lung nodules were noted on chest CT. These nodules were consistent with metastases and the largest was measured at 7 mm. She was seen in oncology and systemic chemotherapy to shrink the tumors and resection of these nodules has been planned. At 1-year follow-up, her spine continues to be disease free, while her lung nodules have slightly increased in size. Therapy is ongoing at this time to address the lung nodules.SS is a rare, aggressive neoplasm of uncertain origin predominantly affecting adolescents and young adults.30 It is 69142530Definitive characteristics making SS completely distinguishable on radiologic examination have not been seen, making diagnosis difficult. Plain raIt is widely accepted that the current most effective treatment for SS is a wide surgical excision with negative margins.1319 Use 135Most of the available information on SS has come from tumors localized to the extremities. However, up to 5% of these tumors are encountered in the body axis, including the spine, mediastinum, retroperitoneum, and head and neck region. Reports of SS located within the spinal axis have been rare and are limited to 13 case reports on 14 patients published in the literature . Of thesClinical differential diagnosis for SS involving the spine includes primarily nerve sheath tumors, and most SS are assumed to be benign nerve sheath tumors preoperatively. Indeed, in two of our cases a simpler/smaller operation was performed first because the lesions were thought to be benign, however, when the final pathology identified SS, a larger more radical procedure was performed. With this limited data, the long-term outcomes are hard to quantitate for SS involving the spine. Based on the previous case reports, most patients died within 3 years following diagnosis, with the exception of a 14-year-old girl who remained disease-free at 6 years.[Over half of the reported spinal SS had dumbbell-shaped intraforaminal extension with a larger extraspinal and smaller intraspinal component. In most cases, the patients’ symptoms were caused by the intraspinal component and resultant compression of neural elements. It was striking however, how large the extraspinal portion was in many of the cases. The most common tumor presenting in this fashion is a benign schwannoma, whereas, malignant nerve sheath tumors are very rare. In regards to SS, it is the rapid growth of the extraspinal portion that is most suggestive of a malignant process. However, in one of our cases, the patient had serial MRI scans 5 years apart and there was minimal growth during this time, which is most unusual for a sarcoma.Some of these unique properties of SS are just now being understood at a molecular level. It has been recently reported that microRNAs (miRNAs) may play an expanded role in the tumorigenesis of some cancers, and when deregulated, depending on their mRNA targets, they can act to down regulate tumor suppressor genes and give a growth advantage to tumor cell lines. In SS, aExperts agree that the cornerstone of treatment for SS is wide surgical excisions with negative margins. Based on our experience, if a spinal dumbbell tumor has any characteristic that would be unusual for a benign nerve sheath tumor, a needle-guided biopsy should be performed first to obtain a diagnosis. Ideally, this is followed by a definitive operation to try and remove the tumor en bloc with negative margins. Traditionally, a 5 cm margin defines a negative margin in sarcoma surgery, however, in many of these cases this would encompass critical structures such as the spinal cord. Thus, in many cases, the best that can be hoped for is a gross total resection with “marginal margins” in which there is no pathologic specimen, but critical structures are not injured.SS of the spine can be challenging to diagnose and even harder to treat. Best available evidence suggests that a multimodal treatment strategy starting with aggressive surgical resection followed by radiation and chemotherapy offers the patients the best chance for a cure. The underlying molecular genetics for SS tumorigenesis is just now being elucidated and hopefully will lead to a better understanding in regards to tumor pathology and lead to novel therapeutics in the future.


To report the rare association of peripheral retinal ischemia in a patient with Waldenström's macroglobulinemia.A 39-year old man with a recent diagnosis of asymptomatic Waldenström's macroglobulinemia (WM) was referred from his physician for ocular evaluation. The fundus examination in his right eye (RE) revealed very mild central vein dilation, while retinal hemorrhages associated with microaneurismal alterations of the vascular plexus were detected at the temporal periphery. Fluoroscein angiography of his RE revealed an extended area of capillary dropout distal to the microaneurismal lesions. In our patient with WM an extensive area of capillary non-perfusion, in the absence of severe involvement of the posterior pole was documented; this association to the best of our knowledge has never been reported before.Although the incidence of the disease is rare, meticulous examination of the retinal periphery should be performed in all patients with WM and vice versa the differential diagnosis of peripheral retinal ischemia of unknown origin should include an investigation to rule out asymptomatic Waldenström's macroglobulinemia. Waldenström's macroglobulinemia (WM) is a non-Hodgkin's B-cell lymphoplasmocytic lymphoma characterized by bone marrow infiltration and production of monoclonal IgM. PatientThe increased concentration of proteins may lead to the development of hyperviscosity syndrome (HVS) which is the main reason of central retinal disorders like vascular occlusion and hemorrhages in patients with WM-4. We reA 39-year old male with a recent diagnosis of asymptomatic Waldenström's macroglobulinemia confirmed with bone marrow biopsy  was referred from his internist, to our clinic for ocular evaluation. The patient had a history of vitrectomy for retinal detachment in his left eye in 2003. Both eyes of the patient, were highly myopic (13D OU). His visual acuity was 20/20 RE and 20/30 LE. Anterior segment examination was unremarkable. The intraocular pressure was 14 mmHg in the RE and 12 mmHg in the LE. Fundus examination of his RE revealed very mild central vein dilation associated with peripheral  retinal hemorrhages and microaneurismal alterations of the vascular bed. Examination of his LE revealed peripapillary atrophy, mild retinal pigment epithelium (RPE) alterations of the fovea, and an area of peripheral chorioretinal atrophy due to cryopexia. Fluorescein angiography of his RE demonstrated an extended area of capillary non-perfusion distal to the microaneurismal lesions (Figure 2 have recently reported that these changes represent the more/most severe findings, but not necessarily the main findings. Venous dilation seem to be the first signs of HVS followed by peripheral retinal hemorrhages, which can only be observed by indirect ophthalmoscopy. Only in advanced HVS one can find central venous dilation, increased tortuosity, central hemorrhages, disc edema, and venous sausaging[The most frequent reported retinal findings in hyperviscosity syndrome are central retinal hemorrhages and vein dilation,3. Menke1Fluoroscein angiography, in our case, revealed an extensive area of peripheral capillary dropout associated with microaneurysms and retinal hemorrhages Figure , 2. Non-In our case, peripheral capillary ischemia and hemorrhages were associated with only a very mild central vein dilation showing that microcirculation may, at least in some cases, be more susceptible and involved earlier than macrocirculation in patients with HVS. Especially in patients with low levels of IgM, evaluation of the peripheral vascular plexus may reveal early signs of HVS in otherwise asymptomatic patients. The fact that our patient was highly myopic should be also taken into consideration; it is not unlikely, that capillary meshwork is more susceptible to damage or to occlusion in patients with high myopia and hyperviscosity syndromes. Additionally, capillary non perfusion would be expected to be bilateral, however, this could not be evaluated in the fellow eye of our patient, where extensive cryotherapy had been applied during vitrectomy for the treatment of the retinal detachment.In our patient, with asymptomatic WM, the peripheral retinal hemorrhages were associated with an extensive area of capillary non-perfusion, in the absence of severe involvement of the posterior pole; this association to the best of our knowledge has never been reported before. Although the incidence is rare, meticulous examination of the retinal periphery should be performed in all patients with WM and vice versa the differential diagnosis of peripheral retinal ischemia of unknown origin should include an investigation to rule out asymptomatic Waldenström's macroglobulinemia.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.The authors declare that they have no competing interests.CK, IG and AK were in charge of the medical care of the patient and performed the different surgeries. DP, IL were responsible for the photographs and the literature review. CK, IG and AK wrote the manuscript. All authors reviewed it, drafted it critically and provided helpful comments. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/10/30/prepub


Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. They are almost always congenital lesions and often cause the surrounding soft tissue and bone to grow aberrantly. We treated a 12-year-old boy who presented with asymmetric pectus excavaum and an anterior chest wall plexiform neurofibroma. The pectus excavaum was corrected by modified Nuss procedure, followed by simultaneous resection of the giant mass. The patient is doing well at the 4 years follow-up visit. Plexiform neurofibromas (PNFs) are benign nerve tumor resulting from aberrant growth of the cells of nerve sheath. They are usually congenital, but they may instead present during the first year as a subtle soft-tissue enlargement or a large patch of cutaneous hyperpigmentation. PNFs are generally painless, slowly growing neoplasmas. Although most neoplasms are asymptomatic, they can be particularly debilitating due to their potential to grow to very large sizes. Presenting symptoms depend on the location of the tumors. Tumors of head, neck, and face are most common, followed by facial disfigurement and lesions of the spine, extremities, and abdomen . Early cA 12-years-old boy with known neurofibromas type 1 (NF1) came to the hospital, stating that a lifelong mass on the anterior chest wall had grown steadily to its present size. He also presented with an 8 years history of increasing depression of the anterior chest wall. Systematic questioning of the patient and his parents elicited no description of significant symptoms. However, the mass was increasingly prominent and the chest wall depression became progressively worse with age, causing the patient considerable emotional distress. Physical examination revealed a soft, fixed, painless mass originating from the anterior chest wall. The mass was associated with thinning of the skin, hyperpigmentation, and it surrounded by numerous café-au-lait spots. Below the mass, a substantial chest wall depression was found Figure . The finGiven the size and location of the mass and the risk of malignant transformation, the patient was referred to plastic surgery for excision. The patient was placed in the supine position after general anesthesia. Frozen section was performed routinely at the beginning of the procedure to exclude malignancy. Surgical technique for repair of the PE was based on the modified Nuss procedure. One small vertical skin incision was made in the midaxillary line each side. A bilateral submuscular tunnel was created using a blunt dissection through bilateral thoracic skin incisions. The use of thoracoscopic visualization was not used. An appropriate introducer  was placed into the tunnel from left side. The tip of the introducer was kept in contacting with the anterior thoracic wall while the introducer was slowly advanced across the retrosternal space. Subsequently, the introducer was slowly advanced across the opposite intercostal space and brought out through the incision on the right side. The bar was attached to the tip of the introducer and the introducer was slowly withdrawn from the tunnel followed by the bar with the bar's convexity facing posteriorly until it emerges on the contralateral side. The bar was placed directly on the ribs in the submuscular position. Both the bar and the stabilizer were fixed together onto the underlying rib with multiple interrupted non-absorbable sutures. An excision of the entire soft tissue mass was carried out right after the Nuss procedure. The visible tumor was of grayish color and of a gelatine-like consistency, stating immediately below a layer of subepidermal fat and did not extend deep to the pectoralis major. A 21.0 × 17.5 × 5.0 cm mass was successfully resected. Signs of erosion of the rib and sternum were no found. After excision of the bulky skin, two Redon drain was inserted and the wound was closed without tension. The patient received an epidural catheter for perioperative patient-controlled analgesia (PCA) for 4 days, and had a good postoperative recovery without complications. He and his parents expressed satisfaction with the cosmetic relief which achieved by the resection and reconstruction. 4 years later  the patient is well and free from tumor and PE recurrence , kyphoscoliosis, and segmental bone hypertrophy of the leg are the skeletal abnormalities previous reported with PNFs ,4,5. WheHistorically, PE was repaired with chondrosternal resection, or some combination of cartilage incision and osteotomy with or without external or internal fixation ,11. MiniIn the literature, surgical management of cardiac anomalies or other intrathoracic diseases and associated chest deformity has been well documented ,17. PrioWritten informed consent was obtained from the patient for publication this case report and accompanying images. A copy of the written consent is available for review by the Editor-in Chief of this journal.The authors declare that they have no competing interests.YJ was involved in the preparation of draft and finalization of the manuscript. BX advised regarding preparation of the manuscript. WYL was the chief surgeon and responsible for finalisation of the manuscript. XJW and SYC helped to draft the manuscript. All authors read and approved the final manuscript. The authors are indebted to all reviewers for their kindly reviewing of the manuscript.


Epithelioid hemangioma is a rare benign vascular lesion that presents as a nodular lesion in the skin of head and neck region. It is a superficial vascular entity which can either be due to tumor or reactive lesion, but the exact etiology is still unknown. We hereby present a rare case which has been reported with the history of small nodular-like growth on mandibular buccal and lingual area. The excisional biopsy was performed and tissue was submitted for histopathological diagnosis. The immunohistochemistry was performed to check the expression of CD31 marker which proved that origin of epithelioid cells was vascular. Oral hemangiomas represent 14% of all human hemangioma. There are various subtypes of hemangioma like capillary hemangioma, lobular hemangioma, cellular hemangioma, and epithelioid hemangioma.Hemangiomas are the benign vasoformative tumors, characterized by an increase in number of blood vessels especially veins and capillaries in a focal area of submucosal connective tissue and are self-involuting in nature. The “hemangioma” in Greek means Epithelioid vascular tumors are a diverse group of lesions which are characterized by endothelial cells which are epithelioid in appearance. These include three variants: benign vascular tumors or epithelioid hemangioma, borderline tumors, that is, epithelioid hemangioendothelioma, and malignant tumors—epithelioid angiosarcoma .Epithelioid hemangioma (EH) is an uncommon lesion, which occurs with greater frequency in the head and neck region . Wells aEpithelioid hemangioma typically arises on the head and neck, but the intraoral sites which are more frequently affected are lips, buccal mucosa, and tongue. The extracutaneous sites which are more commonly affected are bone, salivary gland, and muscular area or extremities. There are only limited intraoral cases of EH and only 16 cases have been reported in the English literature .The etiology and pathogenesis of this vascular entity is still uncertain. This entity typically presents as a small angioma-like nodule, red to brown in color and may be located intradermally or subcutaneously in young adults. EH can mimic lymphoproliferative disorders, especially when the lesion arises in atypical location such as the extremities .We hereby report a case of epithelioid hemangioma involving the lingual mucosa in a 30-year-old female patient with local recurrence.A 30-year-old female reported to the outpatient department of oral medicine and radiology for the complaint of a painful swelling in the lower front region since 20–25 days duration. History revealed that the growth increased rapidly in the first few days and there is no alteration in size since then. Patient had difficulty in speaking and eating, due to the growth. The patient was six-month pregnant and the patient did not give any history of trauma to the involved area.Extraoral examination of the head and neck did not show any abnormalities. On intraoral examination soft painless mobile and nodular growth measuring 1 cm × 2 cm was present in the lingual alveolar mucosa of 32, 33, and 34 region. A soft tissue mass measuring 4-5 mm was also present on the labial surface in between 32 and 33 Figures .There was slight bleeding on brushing and difficulty in speaking. The lesion was erythematous and the tooth adjacent to the mass had no caries and no periodontal pocket. The patient was nondiabetic and nonhypertensive. There was no associated regional lymphadenopathy. Radiographic findings showed no overt destruction of alveolar bone in the area of soft tissue enlargement. Blood investigation revealed raised level of ESR. Blood count did not show eosinophilia . The proThe excision of the lesion was done with proper margins under the lingual nerve block. The two soft tissues of size 0.8 cm × 1.8 cm  and 4 mmAutomated tissue processor (Yorco).Semiautomatic microtome (Leica).Hematoxylin and eosin (H&E) staining kit (Nice).E-Z antigen retrieval microwave (Biogenics).CD31 antibody kit (Dako).Routine two-day tissue processing of both the tissues was done using the automated tissue processor; after that the tissues were embedded in paraffin wax; 4-micrometer thick sections of the tissue were cut from the wax block and were placed on two poly-L-lysine coated slides. The routine H&E staining of the slide was done using the H&E staining kit.2O2 in methanol. The sections were washed in distilled water and antigen retrieval was done in E-Z antigen retrieval microwave as per supplier's instructions . The slides were then incubated at 20°C for 45 min with monoclonal CD31 antibody. The slides were then incubated with anti-mouse biotinylated bridging antibodies (1/200 dilution) for 30 min. Sections were then washed and incubated with standard avidin-biotin complex (Dako) for 30 min. Antibody binding was seen using H2O2 as a substrate and diaminobenzidine as chromogen. The slides were then counterstained using hematoxylin.The immunohistochemistry of the second slide was done. The sections were deparaffinized in xylol and then they were rehydrated in graded alcohol series. Endogenous block was done using 3% HMicroscopic examination of the biopsy specimen revealed focally ulcerated stratified squamous epithelium overlying the connective tissue stroma. At a few places there were sheets of epithelioid cells. Atypical rounded cells with central nucleus were seen proliferating in the connective tissue mass . ConnectThe immunohistochemistry revealed the positivity of these tombstone appearing cells for CD31 proving that the epithelioid cells seen are vascular in origin . A finalThe patient was kept on regular followup. After a month, a similar lesion reoccurred at the same site. Excision of the swelling was again done with wider margins this time. The tissue was subjected to histopathological examination and it revealed the same features.Epithelioid hemangioma in the oral mucosa is a rare disease and often confused with other epithelioid vascular tumours and nonvascular soft tissue tumours. In the literature various terms have been used by different authors to describe this lesion, including angioblastic hyperplasia with eosinophilia (ALHE), nodular angioblastic lymphoid hyperplasia with eosinophilia, lymphofolliculosis, histiocytoid hemangioma pseudopyogenic granuloma, and atypical pyogenic granuloma. In 1982, a broad term, epithelioid hemangioma, is given to describe angiolymphoid hyperplasia with eosinophilia and some other vascular diseases . But theOral cases of epithelioid hemangioma are more common in men, mostly at young age . The lips are the most frequent site (16 out of 36), followed by the tongue (10 out of 36), buccal mucosa (6 out of 36), and palate (3 out of 36) . One casThe extraoral sites of EH are periauricular region, forehead, and scalp. Few cases with multiple lesions have been reported. In 20% of the cases, the lesion is associated with lymphadenopathy and blood eosinophilia .The etiology and pathogenesis of epithelioid hemangioma are unknown. There is a controversy among various authors whether epithelioid hemangioma is a true neoplastic growth or a reactive lesion. According to Martin-Granizo, microtrauma can be a cause of epithelioid hemangioma . It is pPeters et al. reviewed all the cases of the literature and found that, histologically, the vessels were lined by epithelioid or histiocytoid endothelial cells which extend into the lumen and impart them a “tombstone” appearance. These epithelioid cells had rounded nuclei and abundant eosinophilic cytoplasm and in some cases there were large vacuoles. The stroma of the tumour was filled with fibromyxoid tissue with a mixed chronic inflammatory cell infiltrate, including eosinophils, lymphocytes, macrophages, and mast cells and occasional germinal centers were seen .Epithelioid hemangioma is differentially diagnosed from other epithelioid vascular tumours and nonvascular soft tissue tumours showing epithelioid characteristics. Macroscopically, EH should be differentiated from Kimuras, disease, salivary gland tumour, squamous cell carcinoma, lymphoma, and pyogenic granuloma and histologically these are differentiated from bacillary angiomatosis, epithelioid angiosarcoma, epithelioid neurofibroma, epithelioid schwannoma, epithelioid fibrosarcoma, Kimura's disease, and epithelioid hemangioendothelioma . There hBartonella henselae or Bartonella quintana. Histologically it presents with abundant neutrophils with nuclear dust and clumps of bacteria [Bacillary angiomatosis is an infectious disease which is caused bacteria . Epithelbacteria . Epithelbacteria .Immunohistochemistry is a technique for identifying cellular or tissue constituents (antigens) by means of antigen-antibody interaction, the site of antibody binding being identified either by direct labeling of the antibody or by use of a secondary labeling method . The immAs the patient was pregnant it was thought that it can be pyogenic granuloma; moreover, its recurrence after excision also supported it but the histological picture and immunohistochemistry results contradicted the provisional diagnosis.The treatment should be started once the definitive diagnosis has been made. The treatment for epithelioid hemangioma is complete surgical excision of the lesion with conservative margins and regular followup. Other treatment options which have been successfully used for cases of epithelioid hemangioma are laser cauterization, diathermy, cryotherapy, pentoxifylline, and intralesional corticosteroids and in some cases success has been seen with oestrogen therapy .Recurrence has also been observed in about 20% of the reported cases of epithelioid hemangioma of oral cavity .By studying this case we conclude that epithelioid hemangioma although a rare entity with only a few cases of recurrence was presented in our patient supported by clinical and histopathological findings. Moreover, epithelioid hemangioma should be taken into consideration while differentiating any peripheral intraoral lesion in pregnant patients.


The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans.Cavernous malformation (CM) is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed  Intracranial cavernous malformations (CMs) are not uncommon in the clinic and account for 10 to 20% of intracranial vascular diseases ,4. CMs iA 28-year-old male complained of blurred vision in his right eye, which started 2 months before presentation and had worsened about 10 days before. He was otherwise healthy and had no negative past medical history or history of ocular trauma or surgery. Two months before presentation, the patient visited the Department of Ophthalmology, First Hospital of Jilin University, due to a sudden onset of blurred vision. Eye examination showed no obvious abnormalities, and no exact diagnosis was made. The patient did not return to the clinic until the sudden worsening of blurred vision with forehead pain 10 days before presentation. His best-corrected visual acuity was 0.5 in the right eye and 0.4 in the left eye. Anterior segment slit-lamp examination showed no obvious abnormalities. Fundus examination revealed binocular mild primary optic atrophy. Visual field examination showed left homonymous hemianopsia. No other neurological abnormalities were found. No abnormalities were found in the routine analysis of blood, blood coagulation or pituitary hormones.Magnetic resonance imaging (MRI) demonstrated a 1.8 mm × 1.7 mm mass located in the right suprasellar cistern and the right side of the optic chiasm. The mass lesion had clear boundaries and no edema around the lesion. T1- and T2-weighted images showed mixed signal intensity surrounded by a peripheral rim of low signal on the T2-weighted images, which indicated that the right side of the optic chiasm was pressed. Enhanced scans showed no significant enhancement , infratentorial (15%), at the spinal cord (5%) and, rarely, at the optic chiasm (<1%) -9. HowevMRI is currently considered to be the preferred imaging technique for the detection and characterization of CMs, especially atypical CM cases. As early as 1999, Arrué  reportedThe differential diagnosis of chiasmal CMs mainly includes some tumors in the optic chiasm, such as glioma, granuloma, metastatic tumors and germ cell tumors -19. Theset al.[Once a definite diagnosis of chiasmal CM is made, surgical resection should be performed to relieve the visual impairment induced by optic chiasm syndrome. In general, surgical approaches include frontotemporal, orbital zygomatic approach, pterional and interhemispheric approaches. The choice of surgical approach depends on the lesion size, disease development and familiarity of the surgeon with specific surgical approaches ,10,14,15et al. reportedet al.[Not all chiasmal CMs can be thoroughly removed by surgical treatment. For example, the total resection rate of 65 cases reported by Liu et al. was 60%,et al.,21. The et al.,22. As dIn summary, chiasmal CMs usually present with typical optic chiasm syndrome in most clinical cases, but visual field changes may occur when the chiasmal CM is located at the side of the optic chiasm. In either case, definite preoperative diagnosis by means of MRI technology and complete surgical removal by selecting the appropriate surgical approach contribute to satisfactory outcomes.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.CM: Cavernous malformation; MRI: Magnetic resonance imaging.The authors declare that they have no competing interests.XBN wrote the initial draft. KX and QL analyzed and interpreted the patient data. LMQ performed the histological examination. JLY was the surgeon and a major contributor in designing the manuscript. XBN and KX contributed equally to this work. All authors read and approved the final manuscript.Xianbin Ning has a master’s degree; Kan Xu has a doctor’s degree; Luo Qi has a doctor’s degree; Limei Qu has a bachelor’s degree; Jinlu Yu is a neurosurgeon and has a doctor’s degree.


Internal herniation of small intestine is a very rare entity, and it poses a real diagnostic challenge clinically. Recurrent entrapment of the bowel may lead to partial to complete intestinal obstruction and eventually strangulation of the small bowel. Of this rare clinical entity, left paraduodenal hernia is more common. High index of suspicion with prompt management may prevent bowel strangulation and gangrene. We present a case of acute intestinal obstruction due to left paraduodenal hernia with malrotation of midgut in a 55-year-old male patient. Internal herniation, either congenital or acquired, is a rare cause of dynamic intestinal obstruction. Paraduodenal hernias, which are a type of internal hernia, occur due to malrotation of midgut in peritoneal spaces and folds near the ligament of Treitz. Most of the internal hernias are incidentally detected during laparotomy or autopsy. Sometimes, they may present with the symptoms of intestinal obstruction with its dreaded complications like strangulation and gangrene. A high index of suspicion and prompt surgical intervention is required to minimize the morbidity and mortality. A 55-year-old male patient presented to our emergency with abdominal pain, distension, and absolute constipation for two days. The patient had three similar episodes in the past, which responded to conservative management. There was no history of abdominal surgery or trauma. The abdominal examination revealed distension with rebound tenderness and hurried intestinal peristaltic sounds. On digital rectal examination, rectum was empty with ballooning. Hematological investigations demonstrated neutrophilic leucocytosis. Abdominal X-ray on erect posture revealed multiple air fluid levels with presence of valvulae conniventes. Ultrasonography demonstrated dilated bowel loops without any ascites.The patient underwent exploratory laparotomy for acute intestinal obstruction. On exploration, almost entire small gut was found to be in the left paraduodenal area enclosed in a hernial sac . The infInternal hernia is a rare phenomenon having an incidence of 1-2% . As the The inferior mesenteric vein lies on the anterior free margin of the hernial sac . It is bPreoperative diagnosis of paraduodenal hernia possesses a real clinical challenge due its nonspecific symptoms. Sometimes, the patient may give a history of chronic postprandial pain for many years. But, it may present as acute intestinal obstruction, bowel ischemia, gangrene, even perforation of bowel . The finThe left paraduodenal hernia is treated with excision of peritoneal sac, normal repositioning of the small gut, and suturing of the neck of the peritoneal sac with retroperitoneum. Care has to be taken not to injure the inferior mesenteric vein, while dissecting the peritoneal sac. Our patient is now free of any abdominal symptoms for the last one year following his treatment. Internal herniation, if not diagnosed properly and in time bears the risk of ischemia, gangrene, even perforation leading to morbidity and even mortality. Therefore thorough knowledge, high index of clinical suspicion and immediate surgical intervention is essential to reduce the morbidity and mortality in such patients.


We present a rare case of fistulation of a dermoid cyst with the transverse colon. We illustrate how an infected dermoid cyst can be diagnosed as an appendix abscess although the management of these is quite different. The general surgeon should be aware of this as a differential diagnosis for an appendix abscess. Also known as dermoid cysts, mature cystic ovarian teratomas are benign tumours composed of two or more of the three germ layers . They are the most common germ cell tumour and often asymptomatic although they may present with abdominal pain or an abdominal mass. The most common complication is torsion.Malignant and benign gynaecological tumours reported previously have been due to fistulation with the rectum, sigmoid colon or ileum. This is only the third case report of fistulation between a mature cystic ovarian teratoma and the transverse colon, and we give an illustration of the presentation and differential diagnosis, which is relevant to the general surgeon.A 26-year-old woman presented to a rural South African hospital with a 1-day history of abdominal pain associated with nausea. On examination she appeared unwell, had cardiovascular compromise with a tachycardia of 120 beats per minute and blood pressure of 80/52mmHg, and remained afebrile. She had signs of localised peritonitis in the right iliac fossa. Digital rectal examination was unremarkable. Vaginal examination revealed a palpably abnormal cervix and bright red blood although she was not currently menstruating. A urine dipstick was negative for infection and β-human chorionic gonadotrophin. She had presented with similar symptoms one year previously with a suspected appendix abscess. This was treated with a percutaneous drain and antibiotics, and her symptoms resolved. She had a past medical history of abdominal tuberculosis and was HIV positive, currently on antiretroviral treatment with a CD4 count of 254/µl.In view of the patient’s history and concurrent medical problems, computed tomography (CT) was performed. This revealed a multiloculated abscess in the pelvis with a calculus noted adjacent to the collections . The difThe previous intra-abdominal abscess and abdominal tuberculosis may mean that an abdominal operation would be challenging technically. The patient would also be more susceptible to post-operative infection and poor healing due to her immunodeficiency although a CD4 count of over 250/µl is considered adequate for an elective operation at this hospital, which may have been why percutaneous drainage was performed previously. With clinical evidence of sepsis, local peritonism on abdominal examination and radiological evidence of a probable ruptured viscus, we proceeded to laparotomy.Intra-operative findings revealed a mass lying superior to the uterus fistulating with the transverse colon. There was some free turbid fluid in the abdomen but minimal adhesions from the patient’s previous abdominal tuberculosis. The mass was excised and, on opening it, contents consistent with a teratoma were found (hair and sebaceous fluid) as well as a flat worm, most likely an incidental finding. The fistulous tract entering the transverse colon was excised and the defect closed primarily.The patient completed a 7-day course of antibiotics, made an uneventful recovery and was discharged home on post-operative day 14. The histology confirmed a mature cystic ovarian teratoma. She was followed-up at six weeks and was doing well.Mature cystic ovarian teratomas are the most common germ cell neoplasm and have been identified intra-operatively as the most common ovarian tumour to be excised in some series.Torsion of a teratoma is the most significant cause of morbidity. Torsion may be associated with rupture of the cyst and also fistulation. It is thought that if torsion is complete, this will interrupt the arterial blood supply, causing necrosis and therefore rupture. However, if the torsion does not occlude the arterial blood supply completely, the cyst wall may undergo an inflammatory response, leading to adhesions with nearby structures. The adhered area may continue gradually with an inflammatory response and necrosis, eventually leading to fistulation with the adjacent structureInterestingly, a case of squamous cell carcinoma arising from a teratoma of the uterine cervix in a patient with HIV has been reported.The differential diagnoses for an infected teratoma include an appendix abscess and, despite imaging, the diagnosis may be made intra-operatively. The management of both is quite different. In our case, at the first presentation the patient was managed as having an appendix abscess, which may have led to the fistulation due to the percutaneous drainage of the abscess at her first presentation. Due to her immunocompromised status, a conservative approach would have been the preferred option. Teratomas can fistulate with the transverse colon as well as other sections of the large or small bowel and, although rare, may be due to malignancy, which must therefore be identified and managed appropriately.