Primary cardiac angiosarcoma is a rare and aggressive tumor with a high incidence of metastatic spread (up to 89%) at the time of diagnosis, which restricts the indication for surgical resection to a small number of patients. We report the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor (with curative intent) and reconstruction of the right atrium with a porcine pericardial patch. However, after a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence. Angiosarcomas, although rare, are the most common primary malignant neoplasms of the heart ,2. AlthoIn this report we describe the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor and reconstruction of the right atrium with a porcine pericardial patch. Surgical resection was performed with curative intent and resection margins were free of tumor cells. However, after a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence.A 50-year old female presented with shortness of breath, chest and shoulder pain, and pericardial effusion. Pericardiocentesis yielded 950 ml of pericardial fluid, which was sent for biochemical, microbiological, and cytological analysis. The diagnosis was inconclusive and she was treated for presumed idiopathic recurrent pericarditis with prednisone. Five months later she presented with clinical signs of subacute cardiac tamponade. After pericardiocentesis she quickly recovered, however, pericardial fluid analysis again remained inconclusive. Three months after this second episode transthoracic echocardiography (TTE) revealed a tumor in the right atrium. The patient was then referred to our institution for further evaluation.A chest X-ray Figure  showed eTransthoracic (TTE) and transesophageal echocardiography (TEE) confirmed pericardial effusion, and showed a giant mass (51 × 44 mm) that infiltrated the right atrial free wall and that protruded into the right atrium Figure . Left veCoronary angiography of the right coronary artery Figure  showed tFluorodeoxy-glucose-18 - positron emission tomography - computer tomography (FDG-PET-CT) scanning Figure  was perfCardiovascular magnetic resonance imaging (MRI) Figure  and CT  showed aCT, MRI, and PET did not reveal any positive lymph nodes or mediastinal or lung involvement. MRI of the brain did not show any cerebral metastases and a normal mammogram excluded primary breast cancer.Twenty-two days after the initial diagnosis the patient underwent surgical excision of the tumor Figure . HistoloThe resected tumor was 100 × 70 × 45 mm in size Figure . HistopaPostoperative recovery was uneventful and the patient was discharged on the eleventh postoperative day. A follow-up CT-scan after three months revealed no tumor recurrence. After a symptom-free survival of five months the patient unfortunately presented with bone and liver metastases without evidence of local tumor recurrence. Both chemotherapy and irradiation were started.Primary cardiac tumors are rare, with an incidence at autopsy from 0.0017% to 0.033% . MetastaPCAs may present abruptly with a fulminant clinical course. The clinical signs and symptoms are often nonspecific. Symptoms are often absent for a long time and are related to the cardiac location of the tumor, its size, the degree of myocardial involvement, and the presence of metastases ,7,8. BecPCAs are aggressive tumors, with metastasis found in 66 to 89% of patients at the time of diagnosis ,3. PCAs The differential diagnosis of a right atrial mass includes benign entities such as myxoma and thrombus and malignant causes such as metastatic involvement of the heart, primary cardiac angiosarcoma and other sarcomas, pericardial mesothelioma, and primary cardiac lymphoma .Echocardiography has become the primary diagnostic technique because of its high degree of accuracy, non-invasiveness, and cost effectiveness . BesidesCytologic examination on fluid obtained by pericardiocentesis rarely yields a conclusive diagnosis . In caseHistopathology defines angiosarcoma as a malignant tumor whose cells display endothelial differentiation . MicroscAppropriate evidence-based treatment guidelines have not been established because of the rarity of the tumor . SurgicaChemotherapy and irradiation were reported not to improve survival  and theiThe prognosis of cardiac angiosarcoma is universally poor: survival ranges from six to nine months, regardless of the treatment chosen . Death rThe high frequency of metastatic spread at the time of diagnosis (up to 89%) combined with the aggressive behaviour of PCAs usually results in disappointing treatment outcomes. However, early detection and aggressive treatment may lead to a more favorable outcome and may extend survival beyond one year. Therefore, when a patient presents with (recurrent) pericardial effusions or when a right-sided cardiac mass is detected, there should always be a high level of suspicion for PCA. Newer imaging modalities, including CT and MRI, can help define the exact location and extent of the tumor and aid in the planning of surgical resections. Due to the rarity of PCA treatment options are at this point limited and not evidence-based.This case of primary right atrial angiosarcoma in a 50-year old Caucasian woman highlights its nonspecific clinical presentation, the diagnostic delay, the broad spectrum of diagnostic imaging modalities, and the rapid and aggressive natural course of cardiac angiosarcomas. Although successful surgical resection of PCA (with curative intent) in the apparent absence of metastatic spread is possible, there may be micrometastases at the time of diagnosis and surgery. In that case, an apparently curative  surgical resection may provide substantial symptom-free survival, but it has little influence on the rapid and aggressive natural course of PCA.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.WB and CL collected the data and wrote the manuscript. TW, AS, IH, TE, and MM participated in the design of the manuscript and they revised and critically reviewed the manuscript. All authors read and approved the final manuscript.


Inflammatory pseudotumour (IPT) is a rare benign pseudoneoplastic proliferation of unknown etiology, often showing locally aggressive behaviour. Conflicting theories about exaggerated response to injury versus true neoplastic origin have been suggested.We report a case of laryngeal pseudotumour in a 73-year-old man presenting with hoarseness and slowly progressive dyspnea and a short review of the English language literature on the subject.Management consisted of midline vertical thyrotomy, excision of the tumour, and a temporary tracheotomy. No recurrence observed eight months postoperatively.Laryngeal IPT is extremely rare, and it may easily be misinterpreted as a malignant tumour. Conservative excision and anti-inflammatory therapy are advocated, since its general behaviour is benign. IPT is a controversial entity. Although it is histologically benign, it often shows locally aggressive behaviour that mimics a neoplastic process. Although lungs and orbits are the most common sites of involvement, it has been reported in virtually any tissue or organ of the body –3. The p in 1992 . Due to  in 1992 . The purA 73-year-old man was referred to the ENT Department of the University Hospital of Larissa complaining of persistent hoarseness and slowly progressive dyspnea during the past year. The patient had undergone microlaryngoscopy with biopsies for a left vocal cord tumour nine years ago and his histology examination revealed hyperplasia of the surface epithelium, focal keratosis, slight chronic inflammation, and subepithelial scarring. He was a smoker with medical history of chronic renal failure, coronary artery disease, and diabetes. Endoscopic examination revealed a large nodular lesion along the left true vocal cord with impaired laryngeal mobility ipsilaterally. Chest X-ray was normal. MRI scan showed soft tissue fullness in the area of the left vocal cord . No lympInflammatory pseudotumour (IPT) is an unusual benign solid mass composed of myofibroblastic spindle cells with acute and chronic inflammatory cells that mimics a neoplastic process , 3, 7. Tet al, no history of trauma was present except in one patient who had a traumatic intubation for a totally unrelated procedure [The origin of IPT is still disputed , 7. One rocedure . Furtherrocedure , 5, 6. Arocedure . The conrocedure .et al who presented a 34-year-old woman with IPT and supported the hypothesis of a true neoplastic process [The neoplastic theory has been supported by the occasional recurrence and even metastatic potential of IPT . Aneuploidy, clonality, chromosomal rearrangement at chromosome 2p23, expression of ALK1 , p80 5––5–7, 10, process .et al considered two different types of lesion within this generic term: inflammatory/reparative and myofibroblastic proliferations [et al in their initial paper, where they presented the first series of eight laryngeal IPT, mostly described them as being unencapsulated mass [Clinical characteristics suggestive of IPT include patients in their 30s–40s, hoarseness of varying severity and duration , 7, perserations . Accordierations , 4, 7. Serations –10, 13. ted mass , 10.et al, IPT is stained negative with h-cladesmon, S-100, and CD34 [et al first demonstrated the ALK1 expression immunohistochemically (36%) and ALK gene rearrangements by fluorescence in situ hybridization (47%) [The immunohistochemical panel is of great assistance in the diagnosis of laryngeal IPTs . The strand CD34 . Also, Cmically 3% and ALKon 47%) , 7.% , 7.et Laryngeal IPT should be differentiated from malignant neoplasms to avoid unnecessary overtreatment , 6. SurgThe recurrence rates range from 18 to 21% , 9. The et al, and by one of the cases of Wenig et al, a diagnosis of IPT should alert the clinician to strict follow-up affected patients, because there are no morphological features that can predict its biological behaviour [Due to the recurrence potential as shown by the case reported by Corsi ehaviour , 10, 13.ehaviour .In conclusion, laryngeal IPT is a rare benign pseudoneoplastic proliferation that should be differentiated from malignant laryngeal tumours, both due to its reduced aggressiveness and its respectability by conservative surgery, unlike neoplasms. For achieving optimal results, the surgeon should balance between preservation of function and completeness of excision. Strict follow-up is required due to its potential for recurrence.Inflammatory pseudotumour (IPT) is a stromal tumour with low aggressiveness.Laryngeal inflammatory pseudotumour is a rare benign pseudoneoplastic proliferation, which should be differentiated from malignant laryngeal tumours.Surgical resection with negative margins remains the gold standard treatment for laryngeal IPT.The surgeon should balance between preservation of function and completeness of excision.Strict follow-up is required due to its potential for recurrence.


Introduction. Coexistence of carcinoid tumor inside a mature cystic teratoma is an extremely rare phenomenon, especially in young women. We present the case of a 28-year-old woman diagnosed with a right ovarian carcinoid and treated uneventfully with conservative surgical approach. Case Report. A 28-year-old woman, gravid 0, parity 0, presented to our department for her annual gynecological examination and Pap smear test. During her examination, a mobile cystic mass was detected in the right lower abdomen. Ultrasound indicated a right ovarian mass 10.5 × 6.3 cm, confirmed by CT scan. Further investigation revealed AFP levels (1539 ng/mL). The ovarian mass was excised by laparoscopy, leaving intact the remaining right ovary. Frozen sections showed a mature cystic teratoma. However, paraffin sections revealed the presence of a small carcinoid within the teratoma's gastric-type mucosa. The patient was set to a close followup. Nine months postoperatively, ultrasound pelvis imaging and CT scan of the abdomen as well as serum tumor markers have shown no evidence of recurrence disease. Conclusion. Despite the weak evidence, fertility spare surgical approach for women wanting to preserve their genital tract might be a reasonable option. Mature cystic teratomas (MCT) represent 10–20% of all ovarian neoplasms –6. The occasion of a carcinoid tumor arising from MCT is very rare with a very small number of published cases , 8. FurtWe present a case of a 28-year-old nulliparous woman diagnosed with carcinoid tumor arising from a mature ovarian cystic teratoma. A 28-year-old woman, gravid 0, parity 0, presented to our department for her annual gynecological examination and Pap smear test. The woman had an uncomplicated medical and gynecological history with regular menstrual cycle. During her examination, a mobile cystic mass was detected in the right lower abdomen. Ultrasound examination confirmed the presence of a mass approximately 10 × 6 cm derived from the right ovary, characterized by echogenic heterogeneity. Further investigation of the patient was decided, including serum tumor markers  and computed tomography (CT) of the abdomen. All serum markers were normal with the exception of AFP (1539 ng/mL). CT scan confirmed the presence of a right ovarian mass (10.5 × 6.3 cm) containing calcification, while no other peritoneal pathology was demonstrated. The right ovarian mass was excised by laparoscopy, leaving intact the remaining right ovary. Frozen sections showed a mature cystic teratoma and so no other intervention was performed at the time of the operation. Increased AFP levels returned postoperatively to normal and the woman was discharged the next day without any remarkable complication being mentioned. However, paraffin sections revealed a mature teratoma that presented an incidental microscopic focus of a carcinoid tumor, 5 mm in diameter, not seen in the frozen sections. The carcinoid consisted of trabeculae and groups of cells with mild nuclear atypia , which wThe weak evidence regarding the optimal management of a carcinoid arising from an MCT, the small size of the lesion, which was confined to the gastric mucosa within the mass of the MCT, and the fact that our patient was young and nulliparous led us not to proceed in further radical operation. The patient was set to a close followup with gynaecological examination and ultrasound imaging every 3 months and performance of CT scan every 6 months. Conservative approach and close followup were also considered as the optimal approach by the attending surgeon. Nine months postoperatively, ultrasound pelvis imaging and CT scan of the abdomen as well as serum tumor markers have shown no evidence of recurrent disease. Teratomas represent neoplasms containing cells of multiple germ cell layers that may be observed in several anatomic regions, including ovary, testis, peritoneum, spinal cord, or kidney , 11. TheOptimal therapeutic strategy remains the main challenge regarding ovarian teratomas with TMT. The majority of ovarian mature teratomas with malignant transformations are observed in postmenopausal women, in which a more radical surgical treatment including hysterectomy and bilateral salpingo-oophorectomy may be considered as a reasonable option . HoweverClinical and histopathologic prognostic factors of the neoplasm represent the basic determinants of the therapeutic strategy in such rare cases without evidence-based guidelines. Cyst wall invasion, intraoperative rupture of the ovarian mass, tumor dissemination, and adhesions are mainly considered as unfavorable prognostic factors . FurtherIn conclusion, despite the weak evidence related to the coexistence of a carcinoid tumor within a mature teratoma in younger patients, it seems that fertility spare surgical approach for women wanting to preserve their genital tract may be a reasonable option. However, further evidence is needed in order to support definitively the option of conservative treatment in such cases without compromising patient's survival.


Omental torsion is caused by the rotation of the greater omentum on its axis which may lead to tissue infarction and necrosis. It is a rare cause of acute abdomen. Signs, symptoms and paraclinical data are not specific. The patients usually undergo laparotomy for acute abdomen of poorly defined origin. High index of suspicious is required for the diagnosis of this entity. The diagnosis is usually confirmed after an explorative laparotomy. We present clinical characteristics and imaging findings of omental torsion in a young man following repeated blunt abdominal trauma. Omental torsion is a type of volvulus created by the rotation of the omentum on its long axis, which can lead to tissue ischemia and necrosis. Torsion of the omentum is a rare pathology in which the omentum twists until its vascularity is compromised , 2. The A thirty year old man was admitted to the Emergency Department of Shahid Beheshti university hospital with the chief complaint of abdominal pain, which occurred after jumping his one year old daughter on his abdomen. The patient also noted another blunt abdominal trauma, due to a car turn-over, 20 days previously. The pain was sustained and felt in the peri-umbilical region. In the physical examination; blood pressure was 120/70 mmHg, pulse rate was 86, respiratory rate was 12 and the patient’s oral temperature was 36.8° C. The abdomen was diffusely tender in deep palpation without distention, rigidity or guarding. In the laboratory data; white blood cell (WBC) count of 9000, neutrophil count at 65%, Hemoglobin (HB) was 13.3 g/dL and Urine analysis was normal. Ultrasonography showed increased echogenicity of mesentery without free fluid in the abdomen cavity. An abdominal Computerized tomography (CT) scan revealed increased attenuation in the small bowel mesenteric fat. No vein or artery was detected in the mesenteric .3/μL, neutrophils = 83%, and HB = 12.5 g/dL. Therefore, we decided to perform an explorative laparotomy, and a midline laparotomy was subsequently performed. There was hemorrhagic fluid collecting in the peritoneal cavity. The greater omentum was twisted around its basal axis and it was black, showing necrosis  were adults and the remaining 15% were found in the pediatric population . Most paOmental torsion is not a common pathology, but it should be considered in the differential diagnosis of acute abdomen, particularly in patients with a history of repeated blunt abdominal trauma.


Inflammatory pseudotumors (IPT) of the liver are fairly uncommon lesions. IPTs are difficult to diagnose due to the absence of specific symptoms. The correct diagnosis is easily missed, particularly in livers with hepatitis B virus (HBV)-related cirrhosis. The current study presents the case of a 58-year-old male with a ten-year history of HBV infection, who was diagnosed with a primary liver tumor by computed tomography (CT) and magnetic resonance imaging (MRI). The α-fetoprotein levels ranged within normal limits. A local resection was performed and the histopathological analysis identified IPT of the liver. The patient recovered well following surgery. Inflammatory pseudotumors (IPTs) arise most commonly from the lungs . HistoloThe current study presents the case of liver IPT with HBV-related cirrhosis, which was misdiagnosed as a primary hepatic malignant tumor. The final diagnosis of IPT was made by a post-operative pathological examination. Written informd consent was obtained from the patient.A 58-year-old male was referred to the First Affiliated Hospital  following detection of a lesion in the right lobe of the liver by ultrasonography of the abdomen. Upon admission, the patient was free from symptoms and in good general health, without jaundice. The liver and spleen were not palpated, since the patient had no hepatomegaly and only had mild splenomegaly, and there was no sign of any abdominal mass. In addition, the patient had a 10-year history of HBV infection.Laboratory investigations revealed normal liver function test results. The hepatitis serology for HBsAg was positive and no hepatitis C infection was identified. In addition, no leukocytosis was observed and normal AFP, CA19-9 and CEA levels were detected. The upper GI endoscopy and colonoscopy results were normal. The abdominal CT  and MRI Macroscopically, the cut surface of the resected specimen was that of a yellowish-white tumor, which was 4.0 cm in diameter. A microscopic examination revealed a process with benign characteristics, which included numerous infiltrating lymphocytes, mainly plasma cells . These hLiver IPT was first described in 1953 by Pack and Baker . To dateFor cases of suspected IPT, the importance of percutaneous needle biopsy has been emphasized, and due to the risk of spontaneous regression, unnecessary surgery must be avoided . In the In general, IPTs are considered to represent benign lesions, however, the correct treatment protocol for these pseudotumors remains controversial. Certain studies have reported that lesions are likely to be completely resolved following treatment with antibiotics. However, specific lesions have recurred following this treatment protocol. By contrast, IPTs have never been reported to recur following surgical resection. We recommend that short-term observation should be performed in patients diagnosed with IPT. In addition, for cases where the lesion is difficult to differentiate from the malignancy or is associated with high risk factors, including HBV-related cirrhosis, surgical resection must be considered.


Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. These are usually slow growing tumours and are often detected incidentally. Pre-operative diagnosis is extremely difficult as there are no definitive signs on imaging. Aspiration biopsy is often inconclusive or misleading. Surgical excision is both diagnostic and therapeutic. As these tumours are often large in size, open excision is most commonly performed. We describe a case of a large, cystic schwannoma of the pelvis causing bladder outlet obstruction and bilateral hydroureteronephrosis. Complete surgical excision was performed laparoscopically. Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. They are difficult to diagnose as there are no specific clinical or radiological signs and they can mimic a number of pelvic lesions.A 54-year old man presented to us with history of obstructive urinary symptoms for 2 years. He also complained of gradually progressive distension of the lower abdomen as well as numbness of the left hemiscrotum and medial aspect of the thigh. On examination, there was a 10cm × 8cm mass extending from the umbilicus to the pubic symphysis. The lower margin of the mass could not be palpated per-abdominally. The mass was dull on percussion. On rectal examination, the mass was anterior to the rectum. The prostate could be felt separately. There was no neurological deficit apart from loss of sensation of touch over the femoral triangle and lateral part of the left hemiscrotum. The haematological examination, serum biochemistry and urine analysis were normal.Ultrasonography of the abdomen revealed a cystic mass with thin septations located between the bladder and rectum . ContrasLaparoscopic excision of the mass was planned. Cystoscopy and stenting of the ureters was performed. Four trocars were inserted below the umbilicus in an inverted U fashion and laparoscopy was performed with a 30° scope . The perThe mass was removed following aspiration of the contents. The cut section of the tumour showed a cystic mass with areas of calcification . The hisThe patient made an uneventful recovery and was doing well at the six-month follow-up appointment. The numbness of the left hemiscrotum and medial part of the thigh failed to improve, prompting us to presume that the schwannoma originated from the left genitofemoral nerve. The non-visualisation of the left genitofemoral nerve during surgery corroborated this presumption.de novo or may be associated with von Recklinghausen’s disease. They are most commonly benign but may undergo malignant changes if they are a part of von Recklinghausen’s disease. They can occur in any part of the body but are extremely rare in the pelvis with fewer than 20 cases reported to date in the English literature.Schwannomas are tumours of neurogenic origin. They may arise Pre-operative diagnosis of pelvic schwannomas is difficult as there are no imaging findings that are specific for schwannomas. The differential diagnoses include psoas abscess, adnexal mass, fibrosarcoma, liposarcoma, ganglioneuroma, hydatid cyst and haematoma. CT and magnetic resonance imaging may reveal a well circumscribed mass with areas of calcification, cystic degeneration, haemorrhage and necrosis.As imaging findings are not specific, percutaneous biopsies are often used but they have been reported to be inaccurate and occasionally misleading. Surgical excision and histopathological examination is therefore necessary to reach a confirmatory diagnosis. As these tumours usually attain a large size, open surgical excision is generally performed. There are only anecdotal reports of successful laparoscopic excision of these tumours.Pelvic schwannomas are difficult to diagnose pre-operatively. A high index of clinical suspicion is required. Pre-operative imaging and biopsy are often inconclusive, and surgical excision is the mainstay of treatment. Laparoscopic excision is feasible and should be offered to the patients.


Objectives. To investigate hemangiomas in the differential diagnosis of the nasal cavity neoplasms, even though it is an extremely rare mesenchymal tumor of the nasal cavity, and the world literature was reviewed. Case Report. A 57-year-old woman applied to our department with a 5-year history of left-sided nasal obstruction without history of epistaxis, nasal packaging, or facial trauma. Anterior rhinoscopic examination revealed a mass originating from inferior turbinate that completely obstructs the left nasal cavity. Paranasal computed tomography (CT) showed that the bony mass originated from the anterior part of the left inferior turbinate. Surrounding tissues were normal, and there was not any erosion or destruction. Mass was excised by the endoscopic approach. Histological diagnosis was reported as osseous cavernous hemangioma. Conclusion. Hemangiomas are a rare cause of intranasal masses. Its unusual site and masked presentation makes the differential diagnosis difficult. When a bony hard, well-shaped mass was seen in the nasal cavity, the possibility of intraosseous hemangioma must be remembered. A wide range of tumors can be seen in the nasal cavity. Nonepithelial tumors are uncommon and most of them are benign . HemangiA 57-years-old woman applied to our department with a 5-year history of left-sided nasal obstruction. She had no history of epistaxis, nasal packaging, or facial trauma. Anterior rhinoscopic examination revealed a mass originating from inferior turbinate that completely obstructs the left nasal cavity. The mass was bony hard and covered with intact, normal colored, and nonhypervascularized mucosa . No otheHemangiomas are benign vascular tumors, and it can occur in any tissue that includes vascular component like skin, mucosa, muscles, glands, and bones. Head and neck region is a common site for hemangiomas but nasal cavity is uncommon localization. The most common sites for nasal hemangiomas are the nasal septum (65%), lateral wall 18%), and vestibule (16%). Hemangioma arising in the turbinate is reported previously; however, most of them arise from the mucosa. Intraosseous hemangiomas constitute less than 1% of all primary bone tumors, and the most common site is vertebral column , 3. Hema8%, and vThe etiology of intraosseous cavernous hemangioma is unclear. Local trauma and menopause are proposed causes although not proven , 4, 5. IAt radiographic study osseous hemangioma have characteristic soap-bubble-shaped appearance . AngiogrTreatment options of intraosseous hemangioma include surgery, radiotherapy, sclerotherapy, and embolization. Complete surgical excision is mainstay of treatment and also plays a role in definite diagnosis , 6. AlthIn summary, we report a rare case of intraosseous hemangioma of the inferior turbinate. Its unusual site and masked presentation makes the differential diagnosis difficult. When a bony hard, well-shaped mass was seen in the nasal cavity, the possibility of intraosseous hemangioma must be remembered.


Gallstones are relatively rare in children. At-risk populations include patients suffering from hemolysis syndromes. Regardless of etiology, these patients usually will present with postprandial abdominal pain, and ultrasonography is the mainstay of diagnosis. However, some gallstones are radiopaque and can be visualized on plain abdominal radiography.We present the uncommon but classic plain x-ray finding of a calcified gallstone in a 3 year-old Hispanic boy. He was treated with elective laparoscopic cholecystectomy.Cholelithiasis is rare in children, and calcified stones that will appear on plain abdominal x-rays are even rarer. If symptomatic, cholecystectomy by a pediatric surgeon is the treatment of choice. We discuss some of the recent developments in treatment of this condition in this patient population. Although not common in children, gallstones are seen with increased incidence in pediatric patients with hematologic disorders . In adulAn otherwise healthy 3 years and 10 months-old, 99 cm, 14 kg Hispanic boy presented to our Emergency Department after multiple visits to other facilities during the same week with a chief complaint of abdominal pain. The pain was described as postprandial and crampy, and the child’s mother endorsed his aversion to fatty foods and carbonated beverages. She denied emesis, jaundice, icterus, pruritus, or symptoms of altered mental status. Physical examination was unremarkable. Laboratory studies showed only borderline elevated total bilirubin. A plain abdominal x-ray was obtained, which revealed a radiodense mass in the right upper quadrant (Figure Fifteen percent of gallstones are sufficiently calcified to be radiodense enough to be visualized on plain radiographs, and of these, two thirds are pigment stones . In geneDespite its relative rarity in comparison to adults, cholelithiasis must always be on the differential diagnosis with a childhood complaint of postprandial abdominal pain. Cholecystectomy is the appropriate treatment for symptomatic cholelithiasis, especially so in children with sickle cell disease or other hemolytic disorders. It can be performed safely in the outpatient setting, and the emerging technique of single-incision laparoscopy will likely play an increasingly important role in its management in carefully selected patients.Written informed consent was obtained from this minor patient’s parents for publication of this case report and the accompanying images. A de-identified 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.ERB, JRP and CS performed the literature search and wrote the manuscript. CS and OZ performed the surgeries, and SR and SRH provided radiologic images. All authors have read, critically reviewed, and approved the final manuscript.


Small bowel phytobezoars are rare and almost always obstructive. There have been previously reported cases of phytobezoars in the literature, however there are few reports on radiological findings for small bowel bezoars. Barium studies characteristically show an intraluminal filling defect of variable size that is not fixed to the bowel wall with barium filling the interstices giving a mottled appearance. On CT scan, the presence of a round or ovoid intraluminal mass with a ‘mottled gas’ pattern is believed to be pathognomonic. Since features on CT scans are characteristics and physical findings are of little assistance in the diagnosis of bezoar, the diagnostic value of CT needs to be emphasised. Phytobezoars are concretion of poorly digested fruit and vegetable fibres that are found in the alimentary tract, particularly orange pith or pulp in patients with history of surgery and persimmon in patients without previous surgery . PersimmPrevious gastric resection or ulcer surgery such as partial gastrectomy or truncal vagotomy with pyloroplasty predisposes to bezoar. Other predisposing factors are ingestion of high fibre foods, abnormal mastication, diminished gastric secretion and motility, autonomic neuropathy in diabetic patients and myotonic dystrophy . BezoarsNormally found in the stomach, they may pass into the small bowel. Primary small bowel bezoar is very rare and is normally formed in patients with underlying small bowel disease such as diverticulum, stricture or tumour. Phytobezoar can also develop secondarily if there are areas of sufficient stagnation within a dilated bowel segment as may occur in patients with strictures caused by Crohn's disease, TB or previous surgery, or in patients with small bowel diverticula. In such cases, the bile constituents or calcium salts contribute to bezoar development .We present an unusual case of small intestinal obstruction caused by phytobezoar and discuss the radiological investigations with special emphasis on CT appearance that leads to the diagnosis of this condition.An 81-year-old Chinese woman was referred to our centre from a private medical centre for further management of intestinal obstruction and pneumonia. She presented with one week history of epigastric discomfort associated with vomiting and abdominal distension. Her bowel habit was mildly altered but there was no history of passing blood per rectally. She denied any loss of weight or appetite. Interestingly she gave a history of eating a large amount of Chinese mushroom a few days prior to her presentation. Medically she was being treated for hypertension and congestive cardiac failure. Her past surgical history consists of a laparotomy performed 26 years ago and a right nephrectomy for renal stone performed 20 years ago. She was however unable to ascertain the reason for the laparotomy.On admission, her vital signs were stable with BP of 200/90 and HR of 100/m. Chest auscultation revealed reduced air entry and crepitation at the right lower lobe. Her abdomen was soft but slightly distended. A mobile vague mass was palpable at the left iliac fossa. Bowel sound was sluggish and per-rectal examination revealed an empty rectum with no palpable mass. Her hernia orifice were normal.Chest radiograph was done and showed right lower lobe consolidation with evidence of chronic obstructive airway disease and cor pulmonale. Echocardiogram showed evidence of cor pulmonale with no acute ischaemic changes. Her blood investigation results were unremarkable. Blood culture was positive for coagulase negative Staphylococcus. Urine culture and sputum AFB were negative. Her amylase level was normal.Prior to admission to our hospital, the patient had a barium meal and follow-through examination and CT scan of the abdomen done at the referring hospital. The barium studies showed proximal small bowel dilatation with an oval intraluminal filling defect seen at the end of the dilated segment which was about 1 foot from the duodeno-jejunal junction. Also present were ‘coil spring’ and ‘claw’ appearance which led to the impression of an intussusception . It was Pathological report of the operative specimen was degenerate vegetable matter with no malignancy and a left paraovarian simple cyst. Post operative period was uneventful, where she was started on nourishing fluid and soft diet. She was discharged 10 days later with Tab Lisinopril 15mg bd, Tab Unasyn 375mg bd and Tab Bisolvent 8mg od. She was found to be well during follow up in surgical clinic one week later.Small bowel obstruction accounts for 20% of hospital admission. Common causes are adhesions, strangulated hernia, malignancy, volvulus and inflammatory bowel disease. Phytobezoars are rare, accounting for only 0.4 to 4% of all intestinal obstruction. No particular age or sex prevalence have been observed [There are 4 types of bezoars. Phytobezoars are the most common, and are composed of vegetable matter such as celery, pumpkin, grape skin, prune and persimmons and it contains large amount of non-digestible fibres such as cellulose, hemicellulose, lignin and fruit tannins. Trichobezoars are gastric concretion of hair fibres which usually presents in patients with history of psychiatric predisposition and in children with mental retardation. Pharmacobezoars consist of medication bezoars, which in bulk will adhere, such as cholestyramine, kayexalate resin, cavafate and antacids. Lactobezoars are milk curd secondary to infant formula, described in low birth weight neonate fed on highly concentrated formula within the first week of life .Primary small bowel bezoars almost always present as intestinal obstruction. They usually become impacted in the narrowest portion of the small bowel, the commonest site being the terminal ileum followed by the jejunum , as was Plain radiograph typically shows a classic obstructive pattern. Occasionally we may be able to see the outline of bezoar, which is difficult to differentiate from abscess or faeces within the colon. Ultrasound has been used to detect bezoar. In a retrospective study done by Ripolles et al. , ultrasoBarium studies characteristically show an intraluminal filling defect of variable size that is not fixed to the bowel wall. Barium filling the interstices gives a mottled appearance similar to that of a villous tumour . In our CT scan is fast becoming the first line examination for the evaluation of small bowel obstruction because it can exclude other causes of acute abdomen, differentiate between simple obstruction and strangulation, detect signs of concomitant intestinal ischemia and can accurately define the cause, degree and level of obstruction. The presence of round, non-homogenous mottled mass on CT enabled us to accurately diagnose bezoar as the cause of intestinal obstruction in our patient. Kim et al. found that in 11% of the cases, phytobezoar can appear as a soft tissue mass without gas making diagnosis difficult as it can resemble an intraluminal tumour or intussusception. They also described the presence of target sign found in 76% of their patients caused by mural edema or haemorrhage within the intestinal wall. The presence of this sign on CT indicates that the phytobezoar obstructing the bowel may have difficulty passing through the small bowel lumen. An encapsulating wall caused by a gel-like membrane covering the bezoar may also be seen on CT .According to Andrus et al., endoscopy can definitively diagnose phytobezoar where it appears as a dark brown, green, black mass of amorphous material in gastric fundus, antrum or remnant stomach. They found that barium swallow identified only 25% of bezoars found endoscopically [Small bowel bezoars are treated surgically. It is mandatory to explore the whole gastrointestinal tract in order to avoid synchronous bezoar and recurrence of intestinal obstruction due to retained bezoar. Other treatment options include enzymatic breakdown and endoscopic fragmentation for gastric bezoar ,5.Recurrence is common unless the underlying predisposing condition is corrected. Prevention includes avoidance of high fibre foods, introduction of prophylactic medication to improve gastric emptying and psychological or psychiatric follow up in patients with psychiatric disease . In diffIn conclusion we present an uncommon case of small bowel obstruction caused by phytobezoar. CT findings can be diagnostic when there is presence of an intraluminal mass with mottled gas pattern at the site of obstruction associated with distal luminal collapse.


Primary involvement of skeletal muscle is a very rare event in ALK-1 positive anaplastic large cell lymphoma (ALCL). We describe a case of a 10-year old boy presenting with a three week history of pain and a palpable firm swelling at the dorsal aspect of the left thigh. Histological examination of the lesion revealed a tumoral and diffuse polymorphic infiltration of the muscle by large lymphoid cells. Tumor cells displayed eccentric, lobulated "horse shoe" or "kidney-shape" nuclei. The cells showed immunohistochemical positivity for CD30, ALK-1, CD2, CD3, CD7, CD8, and Perforin. Fluorescence in situ hybridization analysis revealed a characteristic rearrangement of the ALK-1 gene in 2p23 leading to the diagnosis of ALK-1 positive ALCL. Chemotherapy according to the ALCL-99-NHL-BFM protocol was initiated and resulted in a complete remission after two cycles. This case illustrates the unusual presentation of a pediatric ALCL in soft tissue with a good response to chemotherapy. Anaplastic large cell lymphoma (ALCL) was discovered by Stein et al. and characterized by strong expression of the Ki-1 CD30) antigen ,3. The k0 antigenHere, we report a case of a 10-year old boy diagnosed with an ALK-1 positive anaplastic large cell lymphoma of the skeletal muscle. Primary involvement of skeletal muscle by ALCL is extremely rare and so far only four pediatric cases have been reported in the literature -11.A 10-year old boy presented with a painful swelling in the dorsal aspect of the left distal thigh without history of trauma. Pain started 3 weeks before, increased with time and resulted in difficulties in walking at time of presentation. No fever, night sweats or weight loss was reported. Physical examination revealed a 9 × 4 cm palpable firm mass in the biceps femoris muscle. Magnetic resonance imaging (MRI) demonstrated a 4 × 3 × 9 cm ill-defining tumor within the long head of the biceps femoris muscle with vivid uptake of contrast medium , CD3, βF1 (T-cell receptor beta chains) , CD5, CD8, CD10, CD56, Perforin , and CD34 . Antibody binding was visualized using the Envision System as described by the manufacturer.In situ hybridization analyses for the t translocation and for Epstein-Barr virus (EBV) encoded small nuclear RNAs (EBERs) was performed on four micron sections of formalin fixed, paraffin-embedded tissue paraffin sections using the ALK Dual Color, Break Apart Rearrangement Probe  and ZytoFast™ EBV Probe ; procedures were performed according to the manufacturers' indications.Histologically, the muscle of the biceps femoris displayed a diffuse polymorphic lymphoid infiltration of high mitotic activity, including atypical mitoses. While no haemangio invasion was demonstrable, lymphovascular invasion was detectable morphologically and by D2-40 immunohistochemistry. The majority of tumor cells was small to middle sized with pleomorphic nuclei and variable amount of pale cytoplasm. Intermingled and less common were larger cells with eccentric lobulated "horse shoe" or "kidney-shape" nuclei and abundant cytoplasm with an intense eosinophilic region representing the Golgi apparatus Figure . These "ALCL can be classified into ALK-1 negative and ALK-1 positive ALCL. ALK-1 negative ALCL occurs mainly in older patients (peak incidence ~ 60 y) and in advanced-stage disease. ALK-1 positive ALCL is a clinically aggressive lymphoma that mostly occurs in the first three decades of life . HoweverThe presented case illustrates a CD30+, ALK-1+ ALCL of T-cell origin  in a pediatric patient. Due to the abundance of small neoplastic cells with pale cytoplasm mixed with some medium-sized and large lymphoid cells this case is best counted among the "small cell variant" of ALCL (10%). Other ALCL subcategories are the common (70-80%) and lymphohistiocytic type (10%).Primary malignant lymphoma of soft tissue is an infrequent, and often diagnostically challenging neoplasm ,17. AmonInterestingly, the current ALCL is the first among the reported intra-muscular ALCL primaries to show a CD8 phenotype, which is extremely rare in ALCL. However, trauma, which theoretically could have acted as a stimulatory trigger providing signals for T cell homing to skeletal muscle  was deniIt should also be mentioned in this context that the slightly enlarged lymph nodes were only detected after MRI examination and were not clinically indicative. Unfortunately a lymph node biopsy was not performed. Therefore, a final statement about a possible secondary involvement of the muscle was not possible. However, we consider this possibility less likely because the majority of ALCL patients (70%) presents with advanced stage III to IV disease , which wALCL has to be distinguished from classical Hodgkin lymphoma (cHL), CD30+ non-Hodgkin B cell lymphomas and very rare ALK-1 positive  large B cell lymphomas. Differential diagnosis between ALCL and cHL can be made by expression of cytotoxic molecules such as Granzyme B, Perforin and T-cell-restricted intracellular antigen-1 (TIA1), EMA and CD45/LCA which are typical of ALCL, while positivity for CD15 (in 70% of cases), PAX5 (90%) and LMP1 (in 50%) are typical of cHL. To distinguish ALCL from ALK-1-positive large B cell lymphomas the lack of CD30 expression is critical and the t translocation cannot be demonstrated in the B cell lymphoma .We conclude that this case represents a very rare manifestation of an ALCL in soft tissue. Despite the anaplastic appearance a favorable outcome was possible after promptly applying aggressive chemotherapy. The expression of the NMP-ALK-1 protein and the young age of the patient might have influenced the positive outcome.Written informed consent was obtained from the patient, respectively the guardians for publication of this case report and accompanying images. A copy of the written consent is available for review to the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TG and AM drafted the manuscript and analysed the data. EG and AR served as reference pathologists. TS, HPS and MD were leadingly involved into the clinical examination and the treatment of the patient. DD carried out MRI and subsequent imaging analyses. All authors read and approved the manuscript.


It usually occurs in vertebrae and secondarily in long bones. Its presence in mandible is extremely rare event. A 48-year-old man consulted to our clinic with a chief complaint of pain in his mandible. Radiography revealed a destructive lesion in body and ramus. The initial pathologic evaluation revealed a high grade pleomorphic neoplasm. The diagnosis was confirmed by immunohistochemical markers. Overall, plasmacytoma with anaplastic features can be confused with high grade sarcoma clinically and histologically Here we report a case of SPB of the mandible with destructive radiographic and anaplastic cytological findings.2,3Solitary plasmacytoma of the bone (SPB) is characterized by abnormal proliferation of malignant plasma cells, which usually occurs in axial skeleton, vertebrae and skull. Diagnostic criteria for SPB include a single lesion, negative skeletal survey, and tumor-free bone marrow. Patients are usually male and are in their sixth or seventh decade of life.A 48-year-old man was referred to our laryngology clinic, complaining of pain in the left mandible and numb chin syndrome. The cone beam computed tomography (CBCT) revealed a large destructive lesion of the left mandible involving the body of the mandible and condylar processes . The skuThe differential diagnosis was plasma cell tumor and rhabdomyosarcoma. The malignant cells were immunopositive for kappa light chain and CD38 and negative for MyoD1, desmin, SMA, LCA and cytokeratin . The final diagnosis was solitary plasmacytoma of mandible. Boone marrow aspiration and biopsy revealed normocellular marrow with no evidence of involvement. The patient was referred to an oncologist and underwent local radiotherapy. No significant improvement in the condition of the patient was observed. Therefore, the patient underwent a left hemimandibulectomy. No evidence of recurrence was noted during a 9-month follow-up period. Written informed consent was obtained from patient and the study was approved by Research Ethics Committee of the Shiraz University.1 SPB accounts for 2% to 3% of all plasma tumors. Bataille and Sany critria,1,2 Solitary plasmacytomas of other bones progress to MM in 35% to 75% of the cases in an average period of 38 months. It is not possible to predict which patients may develop systemic disease because no certain well known risk factor is identified. Therefore regular clinical follow up with monitoring of immunoglobulins and monoclonal proteins in serum and Bence-Jones proteins in urine is advised.1,4,5Plasma cell neoplasms are classified into three groups: multiple myeloma (MM), solitary plasmacytoma of bone (SPB), and extramedullary plasmacytoma.2,6The most frequent clinical symptoms of SPB are pain referred to the jaws and teeth, and less commonly pathologic fractures. Radiographically, the tumor appears as a unilocular or multilocular radiolucent destructive lesion and in our patient CBCT revealed a typical lytic radiolucent area.,8Plasma cells produce osteoclast-activating factors that promote the growth of osteoclasts and stimulate the bone resorption. Therefore, SPB usually appears on radiographic images as radiolucent areas.76,9,10 Biopsy specimen analysis as well as bone marrow, serum protein electrophoresis with skeletal radiologic investigation confirms the diagnosis. Both surgery and radiation have been successfully used in the treatment of SPB.SBP is frequently misdiagnosed as benign lesions , inflammatory disease such as osteomyelitis and granuloma and less frequently as malignant tumors (both primary and metastatic).


Amyloidosis comprises a heterogeneous group of disorders characterized by the deposition of amyloid protein in various organs of the body. The larynx is one of the rarer sites where amyloidosis occurs. 2 laser and on follow-up his voice got much better, but the hoarseness was not fully resolved.A 36-year-old man presented with a two-year history of hoarseness of voice. He had a positive history of smoking, but no history of long-term consumption of alcohol. Physical examination revealed a pinkish mass about 1.5× 1.5 cm in size on his left False Vocal Cord (FVC) extending to the left arytenoid, which resulted in asymmetry of the posterior larynx. He also had a chronic perforation of the right tympanic membrane with a conductive hearing loss consisting of a 50 dB gap in pure tune audiometry. The FVC mass was excised with a COAmyloidosis of the larynx is a rare, usually benign process but the area is the most common site for isolated amyloid deposits to occur in the head and neck.  Amyloidosis comprises a heterogeneous group of disorders characterized by the deposition of amyloid protein in various target organs of the body. This ultimately leads to organ malfunction and failure . AmyloidWhile amyloid is not benign in its systemic form, it usually behaves in a harmless fashion when localized to one site in the head and neck region. Laryngeal involvement has been described at all levels of the larynx. Some authors have stated that the most common location is at the true vocal cord. Other sites include the eye, the orbits, and the major and minor salivary glands, while submucosal deposits have been observed in the nose, paranasal cavities, nasopharynx, oral cavity, stomatopharynx, bronchotracheal tree, and lungs. Oral and paranasal amyloidosis is usually a manifestation of systematic amyloidosis, mainly plasma cell dyscrasia.Patients with amyloidosis of the larynx typically present with hoarseness, although they may present with cough, globus, hemoptysis, stridor, or dyspnea. The deposition of amyloid may occur either diffusely or in a single tumor nodule. Microscopic examination typically shows a diffuse submucosal globular deposition of a largely acellular eosinophilic material that exhibits apple-green birefringence under polarized light when stained with Congo red. There may also be an associated sparse, mixed chronic inflammatory infiltrate consisting of mature plasma cells and lymphocytes . Here, wA 36-year-old man presented with a two-year history of hoarseness. He had a positive history of smoking but not of long-term consumption of alcohol. Physical examina- tion revealed a pinkish mass about 1.5 × 1.5 cm in size on his left False Vocal Cord (FVC) extending to the left arytenoid, which resulted in asymmetry of the posterior larynx .He also had a chronic perforation of the right tympanic membrane with a conductive hearing loss consisting of a 50 dB gap in pure tune audiometry. Other systemic examina- tions of the patient were normal, and there was no generalized lymphadenopathy or hepatosplenomegaly. An MRI and CT-scan of the neck were taken in which a mass-like lesion (about 10 mm) without significant contrast enhancement was visible in the left vocal cord and bulging into the airway .The patient underwent direct laryngoscopy and biopsy. The left arytenoid seemed to be dislocated, but attempts to reduce it were unsuccessful. On histopathologic examination homogeneous eosinophilic deposits in the stroma under the epithelium were seen. Apple-green birefringence with Congo-red stain confirmed the diagnosis of amyloidosis. No signs of malignancy or dysplasia were seen ,4.A thorough medical workup including tests such as cell blood count, C-reactive protein, erythrocyte sedimentation rate and purified protein derivatives CBC, CRP, ESR, PPD, liver function tests, rheumatologic tests, urine volume, creatinine and protein, serum BUN and creatinine, bone marrow biopsy and aspiration, and an abdominal wall fatty tissue biopsy was performed. 2 laser and on follow-up the patient’s voice got much better, but the hoarseness was not fully resolved.The result of serum protein electrophoresis was normal and in electrocardiography and echocardiography, cardiac involvement was ruled out. Sonography of the abdomen and pelvis was also normal and the workup revealed no evidence of amyloid outside the larynx. Finally, the FVC mass was excised with a COTwo theories have been proposed to explain localized amyloidosis of the larynx. The first suggests the occurrence of a plasma cell reaction to inflammatory antigens and is supported by pathologic studies showing the presence of mixed polyclonal plasma cells interspersed with the amyloid tissue. Another, more likely scenario, points to the inability of the body to clear light chains produced by plasma cells located in the mucosal-associated lymphoid tissue . MRI is the technique of choice to detect the most specific features of amyloidosis, since amyloid deposits present an intermediate T1-weighted signal intensity and low T2-weighted signal intensity .When a diagnosis of laryngeal amyloidosis is made, workup should include studies to rule out systemic disease, as well as an accurate assessment of the laryngeal involvement. Multiple myeloma, rheumatic diseases, and tuberculosis are some of the systemic causes that must be considered. Amyloidosis associated with familial syndromes and endocrinopathies, such as medullary thyroid cancer, also needs to be investigated. The workup should include a pulmonary evaluation; tuberculin skin test; complete blood cell count; blood urea nitrogen and creatinine levels; liver enzyme studies; sedimentation rate; determination of Rh factor; urinalysis; antinuclear antibody values; and serum and urine immune- electrophoresis. For most cases of suspected localized amyloidosis it is probably not necessary to investigate for systemic disease with biopsies of the lip, rectum, or abdominal fat, because of the low yield. However, fine-needle aspiration of abdominal fat has been shown to be a simple and effective procedure to rule out systemic amyloidosis . 2 laser and on follow-up his voice got much better, but the hoarseness was not fully resolved.Our patient had a pinkish mass about 1.5 × 1.5 cm in size on the left FVC extending to the left arytenoid, which resulted in asymmetry of posterior larynx . An MRI 2 laser excision of the mass should be the first choice of therapy. The course of the condition under discussion is slow but sudden relapse is possible (Treatment of laryngeal amyloidosis varies from simple observation of the lesion to partial laryngectomy according to the extension of the disease into the larynx. Endoscopic COpossible .2 laser (2 laser is very effective for removal of diseased tissue. Other treatments options that have been described include corticosteroids, radiotherapy, and agents like colchicine and melphalan. However, these modalities have yielded variable results (Localized lesions can be completely removed endoscopically either via cold knife or CO2 laser . Surgica results ,10.In a study on 16 patients with amyloidosis, Pribitkin and colleagues reported the amyloidosis of the upper aerodigestive tract generally behaves as a benign, localized condition treatable by surgical resection. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence, and multiple surgical procedures may be required to control symptoms . HoweverAmyloidosis of the larynx is a rare, usually benign process but the area is the most common site for isolated amyloid deposits to occur in the head and neck. When a diagnosis of laryngeal amyloidosis is made, workup should include extensive studies to rule out systemic disease, as well as an accurate assessment of laryngeal involvement.


Regional odontodysplasia is a rare dental anomaly affecting both primary and adult dentitions in the maxilla or mandible or both jaws, whilst involvement of the maxilla is more common -2. In moThis report describes a case of regional odontodysplasia in a 3.5 year old Iranian girl whose chief complaint was the abscess formation in the left maxillary primary molar region. This case study aims to report the clinical and radiological findings of the current case. The condition was first described by Hitchin in 1934 , althougRegional odontodysplasia is a rare developmental dental anomaly and the prevalence of this condition is still not definitely clear since the studies have mainly been based on the case reports -5. Its pThe etiology of regional odontodysplasia is unknown, but several factors such as local trauma, infection, ischemia or vascular defects, neural damage, hyperpyrexia, nutritional deficiencies, teratogenic drugs, Rh incompatibility, activation of latent viruses residing in odontogenic epithelium, somatic mutations and disorders of neural crest cell migration have been advocated -11. It rThe regional odontodysplasia has no racial predilection and affects female more than male individuals (ratio F/M = 1.4/1) . AlthougThe regional odontodysplasia can occur in maxilla or mandible or both, although involvement of maxilla is twice and the involvement is usually unilateral. One of the distinguishing characteristics of regional odontodysplasia is that the condition rarely crosses the midline . The affected teeth are clinically small, grooved, hypoplastic and hypocalcified . The invThese teeth often display a brown or yellowish discoloration. The most frequent clinical symptoms accompanied by this anomaly are failure of eruption, swelling or abscess of gingiva and periapical infection. Moreover, nine cases of hemangioma and six cases with facial hemi-atrophy were reported to be present with regional odontodysplasia . Radiologically, the affected teeth illustrate abnormal morphology and hypoplastic crown and the lack of contrast between enamel and dentin is usually apparent. The enamel and the dentin are very thin, displaying a” ghost-like appearance” . The enlIn microscopic examination, reduction in the thickness of the dentin layer, expanded areas of inter-globular dentin and extended pre-dentin is observed. In severe cases, the dentinal tubules are reduced in number, whereas in the milder cases the mantle dentin appears normal. The pulp often contains denticles and amorphous calcified materials . The purpose of this study is to report a case of regional odontodysplasia in a 3.5-year-old Iranian girl and thereby describing its clinical and radiographic features.A 3.5-year-old girl was referred to the Department of Pediatric Dentistry, Dental School, Islamic Azad University; Tehran, Iran. Her chief complaint was the abscess formation in the left maxillary region. The prenatal, natal conditions of the patient and the attained medical history revealed no explicit findings. Both parents did not report any previous history of tooth abnormalities or genetic anomalies on either maternal or paternal family part of the case. There was negative history concerning the delayed eruption of the maxillary left teeth compared to their counterparts. Based on her mother’s report, the abscess existed from six month ago and its size was reduced after administration of antibiotics.The extra-oral examination revealed no swelling, no facial asymmetry and the lymph nodes were not palpable. 2 in size.The intra-oral examination showed the occlusion type was mesial step on both sides and the oral hygiene was excellent. Oral mucosa was normal, except for an abscess on the gingiva above the maxillary left primary molar; which was fluctuant and about 0.5 x o.5 cmAll the primary teeth on the left quadrant of the maxilla had abnormal crown, with a yellowish discoloration and hypoplastic enamel. The right maxillary central and lateral incisors as well as the canine had minor caries. The first primary molar was considerably hypoplastic with an obvious carious lesion. The abscess was considered to be related to the maxillary left first primary molar. The right maxillary and mandibular teeth were normal and caries-free .The periapical  and the The laboratory examinations showed that the serum sodium, potassium, calcium, phosphorous and alkaline phosphatase levels were in the normal range. On the basis of our clinical and radiological findings, a diagnosis of regional odontodysplasia was proposed. The patient was instructed for extraction of the left maxillary primary molar, restoration of the maxillary second molar with stainless steel crown (SS-crown) and a space maintainer  to maintain the space after extraction. Moreover, a glass ionomer build-up restoration for the left maxillary incisors and canine was deliberated. A programmed follow-up visits was planned for maxillary left permanent molar to be built-up by glass ionomer restorations as soon as completion of its eruption.The regional odontodysplasia is a relatively rare developmental anomaly -6; thereThe etiology of regional odontodysplasia has not been clearly understood. However, many theories are proposed concerning its etiology  such as The management of a child with regional odontodysplasia entails a multidisciplinary approach. The goal of the treatment is to improve the esthetics and to provide a good oral and dental function for the involved child . There iIn the current case, the maxillary left primary molar should be extracted. The remaining carious teeth in the left quadrant can be restored. The best modality is to restore the left maxillary second molar with stainless steel crown and likewise, the resin-modified glass ionomer build-up restoration for left maxillary incisors and canine would be the best choice. A space maintainer would also be desirable following the extraction of the maxillary left primary molar.Programmed follow-up examinations should be considered to observe and control the eruption of the maxillary left first permanent molar to perform fissure-sealant treatment as soon as it erupts.


Neuroendocrine tumor (NET) in adenoma of the gastrointestinal tract is a rare mixed glandular-endocrine neoplasm and has uncommonly been described mostly in the colon. Histologically, this tumor is composed of a predominant proportion of benign adenomatous component and a small portion of well-differentiated NE component. Only three cases of NET in gastric adenoma have been reported in the literature. We present 4 cases of NET in gastric adenoma mimicking invasive adenocarcinoma. The NETs were 0.62 mm to 4.1 mm in size and located at the basal lamina propria, muscularis mucosa and submucosa. Histologically, NETs consisted of nests, cords, tubules, and clusters of cells that predominantly interposed between the foveolar base without disturbing the overall polyp architecture. The lesions were completely removed by endoscopic submucosal dissection in three cases and in one case, subtotal gastrectomy was performed because endoscopic biopsy was invasive adenocarcinoma. The patients’ clinical course was uneventful without an evidence of recurrence or metastasis. The recognition of NET in gastric adenoma will help avoid potential diagnostic pitfalls masquerading as invasvie adenocarcinomas posed by their infiltrative pattern into submucosa.http://www.diagnosticpathology.diagnomx.eu/vs/1688552293761001The virtual slide(s) for this article can be found here:  Although localized endocrine cell differentiation in benign or malignant glandular neoplasms of the gastrointestinal tract is relatively common, truely mixed glandular-endocrine neoplasms are rare. These tumors are composed of both glandular component like adenomas and adenocarcinomas and recognizable neuroendocrine tumor compoments. Most mixed glandular-endocrine neoplasms of the stomach are malignant tumors arising in the background of atrophic gastritis -5. The hHerein, we describe the clinicopathologic features of four NETs in association with gastric adenomas, to further delineate their histologic features and discuss the diagnostic pitfalls. Recognition of this rare tumor is important not only it can be missed easily due to the minute size of the lesion but also it mimicks an invasive adenocarcinoma arising from adenoma, which may cause unnecessary surgery.Case 1. A 64 year-old man was referred to our hospital with a 3.8 cm sized polyp in the high body after a screening upper endoscopy at outside hospital. An elevated polyp was removed in one-piece by endoscopic submucosal dissection. The patient received a repeat endoscopy at one and two year and no remnant lesion was noted.Case 2. A 63 year-old man presented with indigestion and abdominal discomfort. Upper gastrointestinal endoscopy revealed a 0.5 cm sized elevated nodule in the lesser curvature of the antrum. The lesion was biopsied initially and endoscopioc submucosal dissection was done. The patient repeated upper gastrointestinal endoscopy at one and two year and no residual lesion was seen.Case 3. A 52 year-old man was found out to have a 1.5 cm sized polyp in the lower body of the stomach during staging work up for rectal cancer. The patient underwent endoscopic submucosal dissection and the lesion was completely removed. The resected segment of rectum revealed a moderately differentiated adenocarcinoma and a incidentally found, separately located 0.6 cm sized NET. He received a repeat upper and lower gastrointestinal endoscopy at two year without any evidence of recurrence.Case 4. A 65 year-old man with no family hstory of multiple endocrine neoplasia presented with chronic dyspepsia. Upper gastrointestinal endoscopy and computed tomography scan revealed a 5 cm sized gastric mass in the lesser curvature aspect of the body. Initial endoscopic biopsy diagnosis at the local clinic was well differentiated adenocarcinoma. He underwent a subtotal gastrectomy and was found to have a 5.5 cm tubulovillous adenoma. Unexpectedly, small nests of tumor cells forming a mass infiltrated into the submucosa with an invasion depth of 800 μm. All sixty regional lymph nodes procured from the resected specimen were free of tumor. The patient has no evidence of recurrence or metastasis during 12 years of follow-up.The histology of all four cases was similar. The clinicopathologic details of four cases are listed in Table The concept of a mixed adenoma-NET tumor of the gastrointestinal tract was first introduced by Moyana and Murphy in 1988 . RecentlWe report four rare cases of gastric adenomas containing NETs with an infiltrative growth pattern mimicking adenocarcinoma. The most important reason to recognize NET in a gastric adenoma is to avoid misdiagnosis of this rare lesion as an adenocarcinoma arising from adenoma, which is more common. In our cases, some areas at the base of a polyp showed an intermixture and mergence of adenomatous glands and neuroendocrine cells, which can be misled as tumor cell pleomophism found in adenocarcinoma. These lesions appeared to be arisen from the basal epithelium of adenomas showing “budding-off” angulated glands and infiltrated into the muscularis mucosa or submucosa.After confirmation of NET in our cases by immunohistochemistry, there was an issue to classify the proliferation of the neuroendocrine cells within adenomas depending on its size and distribution. Until now, no definite consensus on the histopathologic classification of proliferative endocrine cell lesion has been established. Solcia et al. has proposed the subclassification based on size, growth pattern, and numbers of endocrine cells within glands or crypt as follows: simple hyperplasia; linear hyperplasia; micronodular hyperplasia; dysplasia (< 0.5 mm in diameter); NET (> 0.5 mm) . NeuroenThe prognosis of benign mixed adenoma-NET of the stomach can not be completley determined due to the rarity of cases. Complete removal of an adenoma would be considered curative whereas the combined NET component would be a main predictive factor to determine the patients’ prognosis. In our four cases, three cases showed NETs limited in the lamina propria and focally extended into the muscularis mucosa without any evidence of local recurrence or metastasis. A large series by Soga et al. demonstrated that small submucosal NETs of the stomach had a relatively high metastatic rates . This stIn summary, NET may be found in adenomas of the stomach incidentally, has characteristic morphologic features and immunoexpressions as neuroendocrine cells, and can be under or overdiagnosed due to the small size of the the lesion and overlapping histology with adenocarcinoma. This lesion can be completely treated by polypectomy or endoscopic resection technique without recurrence or metastasis. However, if the NET involves the submucosal layer, close follow-up is recommended for a relatively higher metastatic rate.We described four rare cases of NET arising from gastric adenoma and followed benign clinical courses. Recognition of this rare entity is important for accurate diagnosis and treatment.Written informed consents were obtained from the patients for publication of this case report and any accompanying images.The authors have no potential conflicts of interest to disclose.SMA, YKL, KTJ, and CKP made contributions to acquisition of clinical data, and analysis of the histologic features by H&E stain and immunohistochemistry. SML drafted the manuscript. KMK revised manuscript critically for important intellectual content and had given final approval of the version to be published. All authors read and approved the final manuscript.


Extraskeletal myxoid chondrosarcoma (ESMC) is a rare tumor seen more often in men. It is seen to arise from soft tissue of lower extremity or buttocks. We report a case of soft tissue swelling of left labium majus in a 66-year-old female. Patient underwent wide excision with uneventful postoperative course. Histopathology of specimen confirmed it to be ESMC. Patient refused adjuvant therapy. Followup of 1 year has shown her to be disease- and symptom- free. Only two cases arising from vulva have been reported in literature . This is the third case and first from Indian subcontinent. A brief review of clinical features, diagnosis, treatment, and outcome of patients with extraskeletal chondrosarcoma is presented. Extraskeletal myxoid chondrosarcoma (ESMC) was first described by Enzinger and Shiraki –3. Its uWe present a case of ESMC arising from left labium majus in a 66-year-old female. Surgical excision followed by histopathology confirmed the diagnosis.  A 66-year-old female presented with painless lump in the left vulvar region for 2 years. It was initially small and had gradually increased in size. There was no history of fever, trauma, or any other complaints. She has been post menopausal for 20 years. Clinical examination revealed a firm, nontender lump measuring 12 cms × 8 cms arising from the left labium major with smooth surface and well-defined margins . UltrasoESMC is a rare variety of sarcoma with a cytogenetic basis. The EWS-CHN gene fusion is seen in more than 75% cases of ESMC . Our casPreoperative diagnosis is difficult, however there are studies where fine needle aspiration cytology (FNAC) has diagnosed this entity preoperatively . Microscopically, extraskeletal myxoid chondrosarcoma has a multinodular pattern. The tumor nodules consist of round or slightly elongated cells of uniform shape and size in variable amounts of mucoid material. The neoplastic cells are arranged in short anastomosing cords or strands, and less frequently in small, loose aggregates. The individual cells have small hyperchromatic nuclei and a narrow rim of deeply eosinophilic cytoplasm and feature characteristic of chondroblasts . Our casThe treatment of patients with localized extraskeletal myxoid chondrosarcoma should include excision of the primary tumor with a wide surgical margin . ESMC ha


The diagnosis of urethral diverticulum can be challenging given the vague or absent presenting symptoms. In addition, vaginal cancer can present with elusive symptoms--some parallel to urethral diverticula. A case of a bleeding ulcerated mass anticipated to be a vaginal cancer was instead identified as a benign urethral diverticulum. To the best of our knowledge, this is the first case report of a benign urethral diverticulum presenting as a bleeding, necrotic ulcerated mass.A 52 year-old multiparous African-American woman presented with a 2-day history of heavy vaginal bleeding passing large clots and suprapubic pain. A pelvic examination revealed blood clots in the vagina along with a friable, fibrous ulcerated lesion on the anterior suburethral vagina, just left of the midline measuring 4 × 2cm. Initially, this mass was considered to be a vaginal cancer. Intraoperative diagnosis of a benign urethral diverticulum was made.The diagnosis of urethral diverticula based on the vast array of presenting symptoms, is difficult. This original case report may benefit both gynecologic oncologists and female pelvic surgeons and reconstructive surgeons to keep urethral diverticulum in the differential diagnosis when faced with a bleeding midline anterior vaginal mass. This unusual presentation of a urethral diverticulum demonstrates how similarly it may present to a vaginal cancerous mass. A urethral diverticulum is a permanent, epithelialized, non-muscular, sac-like cavity projecting into the periurethral fascia arising from the posterior urethral lumen with an incidence of 1 to 6% in the general population .They present at any age, but more commonly in the second and third decade of life with nonspecific symptoms. Due to vague or absent symptoms, patients are often misdiagnosed. Frequency, urgency, dysuria, dyspareunia, and post-void dribbling are commonly pathognomonic of urethral diverticulum . PalpatiSimilarly, vulvar cancer presents with nonspecific symptoms and is often difficult to diagnose. The most common presenting symptoms include a visible or palpable mass, bleeding, ulceration, vaginal discharge, pain, dysuria, and pruritus. When vulvar cancers are diagnosed in an advanced stage, it is often attributed to the vague presentation and delay in diagnosis. If a vulvar lesion is identified by physical examination, a biopsy is imperative to evaluate for carcinoma .Furthermore, diagnosis can be difficult if a malignant transformation presents within the diverticulum such as several described cases including adenocarcinoma, transitional cell carcinoma, and squamous cell carcinoma .We present the case of a patient who presented with a necrotic, ulcerated, bleeding vaginal mass initially thought to be a vulvar cancer based on the presentation, but found to be a urethral diverticulum.A 52-year-old multiparous African American woman presented from an outside hospital after syncope. The patient had a 2-day history of heavy vaginal bleeding passing large clots and suprapubic pain. She denied any vaginal pain, dysuria, hematuria, or frequency. She had a remote history of a total abdominal hysterectomy and bilateral salpingo-oophorectomy secondary to leiomyomata.The initial pelvic examination revealed blood clots in the vagina along with a friable, fibrous ulcerated lesion on the anterior suburethral vagina, just left of the midline measuring 4 × 2cm. The lesion was actively bleeding and necrotic, but did not appear to extend beyond the sidewall. Biopsies of the specimen were insufficient for diagnosis revealing blood and necrotic material. A computed tomography scan revealed a soft tissue mass within the lumen of the vagina and an enlarged lymph node located within the right ovarian chain Figure . A stagiOn hospital day (HD) 2, the patient underwent an examination under anesthesia with repeat biopsies. With improved visualization, the ulcerated mass was noted to be 4.5 × 2.5cm in the mucosa of the anterolateral vagina left of the midline parallel to the urethra. Beyond the lesion, inspection of the surrounding vaginal anatomy revealed otherwise normal vaginal mucosa. The parametria and cul-de-sac were normal and smooth bilaterally. Based on this examination and clinical presentation, concern was for vaginal squamous cell carcinoma. However, the pathology was again non-diagnostic.During HDs 3 through 5, the patient’s bleeding had decreased significantly, and she was asymptomatic. On HD 6, a second examination under anesthesia again revealed biopsies negative for malignancy. A Tonsil clamp was placed into the urethra and visualized through a breach in the vaginal wall around the vaginal mass. The urology service was intraoperatively consulted, and the diagnosis of urethral diverticulum at the 5 o’clock position was confirmed. Cystoscopy confirmed a urethral defect toward the right distal aspect and another area of communication near the 7 o’clock position. The diverticulum was then excised, and a multilayered repair over the Foley catheter was performed.On postoperative day 9 the patient re-presented with bleeding. An examination under anesthesia revealed several rejected sutures in the necrotic tissue. These were removed, and interrupted mattress sutures were placed to reapproximate the tissue. Final pathology revealed benign squamous mucosa.In the urology clinic 2 weeks later, a voiding cystourethrogram was performed revealing no extravasation from the urethra or bladder. A physical examination revealed proper vaginal healing and no defects.Female urethral diverticula often become diagnostic dilemmas. Acquired or congenital, they usually present in women of reproductive age, unlike this post-menopausal patient. Most diverticula result from repetitive or chronic infections of the periurethral glands, which are unresponsive to multiple courses of oral antibiotics, leading to chronic inflammation. This chronic inflammation explains why some cases (such as this presentation) may demonstrate bleeding at the lesion, even in the absence of a urethral stone or malignancy. Huffman  likened The diagnosis of diverticula based on the vast array of presenting symptoms is difficult. Classically, the milking of purulent discharge from the urethra after compressing the suburethral area during examination is highly specific, although poorly sensitive. A urine analysis and culture should be sent; women often complain of recurrent urinary tract infections . Cystoscet al. [Presenting symptoms vary including dysuria, dyspareunia, or stress urinary incontinence. Romanzi et al.  describeet al. .Several case reports have presented malignant diverticula . The preThis unusual presentation of a urethral diverticulum emphasizes the importance of keeping a broad differential diagnosis when faced with an anterior midline vaginal mass with symptoms such as bleeding and necrosis. Gynecologic oncologists, female pelvic surgeons, and general gynecologists can all benefit from an understanding of this rare diagnosis and the possibility of vague symptoms upon presentation. Furthermore, this original study highlights that urethral diverticula may mimic other disorders and that symptoms refractory to therapy should prompt consideration of this diagnosis.Written informed consent was obtained from the patient for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors have no conflicts of interest or financial disclosures.MB performed background research, data collection, and manuscript writing and was one of the surgeons during the first operating room case. RJ performed background research, data collection, manuscript writing, editing, and submission, and was a surgeon at the time of diagnosis. AH was the surgeon who diagnosed the urethral diverticulum. KR was the surgeon who initially took the patient to the operating room and performed biopsies. All authors read and reviewed the final manuscript.


Intestinal malrotation is an uncommon cause of abdominal pain and normally presents during infancy. Familial cases of malrotation are extremely rare in the absence of other congenital malformations. We present the case of a 22-year-old woman with undiagnosed chronic abdominal pain and her previously well 16-year-old brother who presented within 18 months of each other with acute midgut volvulus secondary to intestinal malrotation. Clinicians should be aware of this rare but serious cause of abdominal pain. Intestinal malrotation is a well recognised cause of abdominal pain in children with a described incidence of 1 in 500 live births.The majority of patients with malrotation present in the neonatal period with bilious vomiting and a diagnosis can be confirmed with barium studies.Cases of familial intestinal malrotation are rare and normally occur in conjunction with other genetic abnormalities including gut atresias, biliary, cardiac and pancreatic malformations.A 22-year-old woman presented to our department with severe abdominal pain shortly after being discharged from another hospital. She had a long history of abdominal pain and had seen multiple paediatricians and gastroenterologists. Over the following few days her clinical condition deteriorated and she developed peritoneal signs and tachycardia. Urgent computed tomography (CT) was performed, which demonstrated the presence of midgut volvulus with an occluded superior mesenteric artery. The patient proceeded to emergency laparotomy. This revealed intestinal malrotation and a midgut volvulus. Unfortunately, there was a large amount of necrotic small bowel with a combined total of 15cm of viable proximal jejunum and terminal ileum.Following a subtotal small bowel resection and mucous fistula formation, a second look laparotomy was performed the following day and gut integrity was restored. Recovery was complicated by the development of several intra-abdominal collections treated with radiological drainage and antibiotics. The patient is well and gaining weight 18 months after discharge although she remains on long-term cyclical parenteral feeding.Fifteen months later, this patient’s sixteen-year-old brother presented with a short history of sudden onset central abdominal pain. He had no prodromal symptoms. Although he displayed no evidence of peritonism and his pain had settled somewhat overnight, CT was performed and confirmed volvulus. At laparotomy he was found to have intestinal malrotation with midgut volvulus. Although the bowel was cyanosed, this promptly improved following untwisting of the volvulus and no bowel resection was necessary. As with his sister, the caecum was in the left upper quadrant, the duodenum was intraperitoneal and there was only a very narrow attachment of small bowel mesentery to the posterior abdominal wall around which the volvulus had occurred .Figure The Ladd’s bands were divided, the bowel was untwisted and replaced in the anatomical position with anchoring of the small bowel mesentery to the posterior abdominal wall. This patient made a steady post-operative recovery, which was complicated by a pneumonia. He remains well.To our knowledge this is the first report of familial malrotation presenting with acute volvulus in adulthood in siblings with no other syndromic abnormalities. Studies have identified the transcription factor FOXF1 as playing a key role in the formation of the dorsal mesenteryThe diagnosis of intestinal malrotation can be difficult in young adults as symptoms are often vague and remitting. The lady in this report was seen on an outpatient basis by both paediatricians and gastroenterologists multiple times as a child and young adult and had been labelled as having functional bowel disorder. She had at least three admissions to hospital with acute self-limiting abdominal pain and ultrasonography had confirmed the presence of a small gallbladder polyp, further confusing the diagnostic picture. She had also undergone a barium swallow as a neonate following episodes of vomiting, which was reported as normal. All of these factors, coupled with a reluctance to expose young adults to the radiation associated with imaging such as CT, led to the delay in treatment to the point where extensive bowel necrosis had occurred.Fortunately, when her brother presented with similar symptoms, CT was performed early even though his symptoms had seemingly improved. This undoubtedly led to a quicker diagnosis in his case, which obviated the need for intestinal resection.Clearly, CT of all patients with self-remitting abdominal pain would be both unworkable and hazardous. Acute midgut volvulus in patients with malrotation can cause severe abdominal pain and, where spontaneous untwisting occurs, symptoms may be self-limiting. As ever, one should be cautious in accepting a diagnosis of functional bowel disorder in patients with known chronic symptoms that present acutely. When symptoms do not settle in such patients, an alternative explanation must be sought. Familial malrotation is rare in patients in the absence of other syndromic abnormalities and we would not recommend routine screening for family members of those affected. However, a diagnosis of malrotation with volvulus should be considered if a patient with a family history of midgut malrotation presents with abdominal pain.


Disseminated peritoneal leiomyomatosis is a rare disease. Almost all disseminated peritoneal leiomyomatosis cases described in the literature are associated with a gynecological disorder or a mass in the abdominal cavity. Disseminated peritoneal leiomyomatosis with only chronic constipation has not been reported in the English literature. We present a case of a patient with disseminated peritoneal leiomyomatosis who manifested solely with chronic constipation.A 49-year-old premenopausal nulliparous Caucasian woman was admitted with complaints of abdominal distention and chronic constipation. Open subtotal colectomy with ileorectal anastomosis was performed. There were diffuse nodular and polypoid tumor formations in her colonic mesoderm. Based on morphological and pathological evaluation of the resection material, she was diagnosed with disseminated peritoneal leiomyomatosis.In general, disseminated peritoneal leiomyomatosis is seen in women who are of childbearing age with estrogen hypersecretion. Preoperative diagnosis of disseminated peritoneal leiomyomatosis is almost impossible and it can be confused with disseminated intra-abdominal malignancies. There are no specific methods to diagnose disseminated peritoneal leiomyomatosis in a preoperative period. Disseminated peritoneal leiomyomatosis (DPL) is a rare disease which was first defined in 1952 by Willson and Peale. It is gA 49-year-old premenopausal nulliparous Caucasian woman was admitted with the complaints of abdominal distention and constipation. She indicated that she had been defecating once every 7 to 10 days, frequently with the help of a laxative, and had had serious abdominal pain intermittently since childhood. She had undergone an exploratory laparotomy for ileus 1 year ago. The related surgery report stated that her colon had been extensively dilated and elongated, and there had been massive fecal impactions obstructing her colon. The impactions had been disintegrated and removed, and no other pathology had been observed in the surgery.Some laboratory and screening tests were carried out for differential diagnosis of the patient. Laboratory test results were nonspecific and tumor markers were within normal ranges. Anal manometry confirmed the presence of basal waves and rectosphincteric reflex. A barium enema showed dilation of her entire colon. Her colonic transit time was found to be prolonged. Her mucosal structure had a normal appearance at colonoscopy and there were no extra pathological findings in defecography. Informed consent was obtained and she underwent a subtotal colectomy including ileorectal anastomosis due to chronic constipation. Significant elongation and dilation of her colon were observed in the surgery. There were diffuse nodular and polypoid tumor formations in the colonic mesoderm. Since no early and late surgical complications occurred, she was discharged from our hospital at postoperative day 5 with surgical recovery.The resection material was composed of distal ileum, cecum, ascending colon, transverse colon and sigmoid colon. Multiple nodular formations with a hard elastic consistency were observed in the mesenteric adipose tissue Figure . They haWe report the case of a patient affected with the rare disease DPL. It is difficult to diagnose DPL due to its resemblance to malignant conditions. However, there are some differences to consider such as in the cases of LMS and GIST. LMS has a higher mitotic index and shows nuclear atypia, tumor necrosis and infiltrative patterns. Our casFemale patients with DPL in cases reported in the literature were generally pregnant, using oral contraceptives or had malignant ovarian tumors expressing high estrogen and/or progesterone at the time of diagnosis,8. Moreoet al. found that p53 was strongly positive in transformed malignant cells[Some cases of DPL showed malignant transformation. There cnt cells. Since pnt cells. Surgerynt cells. Almost It is important to diagnose DPL because some cases of DPL can show malignant transformation. Pathological evaluation is critical for diagnosing the disease. Other signs and symptoms, such as chronic constipation, may mask the diagnosis of DPL.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.DPL: Disseminated peritoneal leiomyomatosis; GIST: Gastrointestinal stromal tumor; LMS: Leiomyosarcoma.The authors declare that they have no competing interests.SB, EC and EP were involved in the conception of the report, review of literature, manuscript preparation and submission. HB and SB performed the surgery. SC, MMS and EK were responsible for manuscript critique and review. All authors read and approved the final manuscript.


We report a rare case of nasal glial heterotopia in an adult. After the surgery, frontal lobe cerebral hemorrhage developed. A 58-year-old man had unilateral nasal obstruction that progressed for one year. He had been treated for hypertension, chronic heart failure, and cerebral infarction with aspirin and warfarin. A computed tomography scan showed that the tumor occupied the right nasal cavity and the sinuses with small defect in the cribriform plate. The tumor was removed totally with endoscopy. After the operation, the patient developed convulsions and frontal lobe cerebral hemorrhage. The hemorrhage site was located near a defect in the cribriform plate. Nasal glial heterotopia is a rare developmental abnormality, particularly rare in adult. Only few cases were reported. We could not find any report of adult nasal glial heterotopias that developed cerebral hemorrhage as a complication of the surgery. Nasal glial heterotopia, also known as nasal glioma, represents a collection of normal glial tissue in an abnormal location; the tissue is isolated from the nervous system without intracranial connection , 2. NasaNasal glial heterotopias have no communication with the central nervous system. However, from CT findings, 15%–20% of patients have a defect in the cribriform plate , 5.We report a case of nasal glial heterotopia in an adult man. The tumor was removed endoscopically. After the surgery, frontal lobe cerebral hemorrhage developed. We found that the hemorrhage site was located near a defect in the cribriform plate.A 58-year-old man was referred to our hospital for evaluation of a unilateral nasal obstruction. His symptoms had progressed for one year before the consultation. He had no rhinorrhea or nasal bleeding. He had been treated for hypertension, chronic heart failure, and 2 incidences of cerebral infarction (the latter with aspirin and warfarin). Endoscopic examination revealed a soft, white, and polypoid mass that occluded the right nasal cavity . A compuTotal endoscopic resection of the tumor was performed under general anesthesia. The tumor was found to arise from the frontal sinus and was occupied the anterior ethmoid sinus and nasal cavity. The maxillary sinus was filled with the pus by secondary inflammation from the tumor. The tumor was removed under endoscopic visualization at 0 and 70 degrees of view. No cerebrospinal fluid (CSF) leakage was found during or after the operation.Three hours after the operation, the patient developed convulsions. The CT showed frontal lobe cerebral hemorrhage. He was treated in the intensive care unit with an anticonvulsant sedative. One day after the convulsion, he became fully conscious, without signs of dyskinesia.Histopathologically, the tumor consisted of neuroglial cells, neuroglial fibers, and vascularized connective tissue. The tumor was surrounded by normal respiratory mucosa. The neuroglial cells were stained positive for glial fibrillary acidic protein (GFAP) .For 2 years after surgery, the patient has been free from nasal symptoms, signs of recurrence, and dyskinesia.Nasal glial heterotopia  is a rare developmental abnormality typically seen at birth or in early childhood. Nasal glial heterotopia develops from normal glial tissue, but is located in an abnormal location, and is isolated from the nervous system without intracranial connection. It is thought to be the result of abnormal embryonic development. Approximately 250 cases have been reported , but onlRegarding the location, 60% of the masses were located extranasally, 30% were intranasal, and 10% were both –13. In tThe histology of nasal glial heterotopia is characterized by mature glial cells (astrocytes and oligodendrocytes) in stromal connective tissue, covered by the respiratory epithelium . The difUnilateral nasal polyps and tumors in adults are very common conditions encountered by the otolaryngologist. Differential diagnosis of these lesions is made by determining the form, shape, color, and hardness of the mass. The underlying etiology may be inflammation, chronic fungal sinusitis, benign neoplasm, inverted papilloma, or malignant tumor. A radiological evaluation is mandatory to assess the site, extent, and enhancement effect of the lesion. The presence or absence of bony erosion should also be examined. In particular, when intraglial heterotopia is surrounded by respiratory mucosa, it is difficult to discriminate it from such masses as polyps.In the present case, nasal obstruction had progressed for one year. The CT showed that an isodense shadow occupied the right nasal cavity, maxillary, anterior ethmoid, and frontal sinuses, with no bony erosion, although a small defect in the cribriform plate was noted. The MRI showed that the tumor was solid and had no connection with the meninges or cerebral tissue. We suspected that the tumor was benign or of low grade malignancy. We did not perform biopsy, since the patient had chronic heart failure and cerebral infarction treated with aspirin and warfarin. We decided to perform total endoscopic resection with the additional treatment of low dose heparin. We used low dose heparin  for four days before the surgery instead of aspirin and warfarin.During the operation, the mass was found to arise from the frontal sinus. Defects in the bones surrounding the sinuses or CSF leakage were not observed. However, the patient developed convulsions and frontal lobe cerebral hemorrhage after surgery.Intranasal glial heterotopia has a firm attachment to the nasal vault at the middle or superior turbinate . An intrDiagnosis of intranasal glial heterotopia in adults can be made histologically. The presence of a single, unilateral, nontranslucent, noncompressible mass may arouse suspicion of intranasal glial heterotopia or encephalocele. In either case, one should avoid performing biopsy or surgery before a possible intracranial connection is evaluated by CT and MRI.Nasal glial heterotopia is a rare congenital lesion that is frequently diagnosed in newborns or infants, although this case is found in adults.Total endoscopic resection was performed without the CSF leakage; however, the patient developed frontal lobe cerebral hemorrhage after surgery.We suspected that the force of the surgical procedure was transmitted intracranially, through the bone defect of cribriform plate.