A rare case of congenital bipartite lunate in a child is reported. Carpal variants are very uncommon as independent entities, with only three previous reports of this condition in the English literature.An 11-year-old Caucasian boy presented with pain in the left wrist after a fall. Radiographs in the emergency department demonstrated a lunate that was divided into palmar and dorsal parts, causing a misdiagnosis of fractured lunate. Magnetic resonance imaging was then used to differentiate between the two diagnoses.Very few cases of bipartite lunate have been reported in the literature, and unless awareness is raised about congenital anomalies such as this variant, confusion may arise. The bipartite lunate is a rare congenital variation of the carpal bones. While anomalies such as a bipartite scaphoid, bipartite hamate and carpal synostosis are well described in the literature -4, to ouAn 11-year-old Caucasian boy presented to the emergency department after a fall onto the outstretched left hand. Standard posteroanterior and lateral radiographs of the wrist were performed, which demonstrated a lucent line through the lunate. On examination, the patient reported pain over the dorsum of the distal radius and in the anatomical snuffbox, but no point tenderness over the lunate. On the basis of this clinical presentation, he was provisionally diagnosed with a fractured lunate and an undisplaced scaphoid fracture  was performed to differentiate between a traumatic lunate fracture, a bipartite congenital variant of the lunate or an atypical presentation of Kienböck's disease. Axial MRI images correlated with the plain radiographs showed a linear hypointense signal between the well-defined rounded bony margins of the palmar and dorsal parts of the lunate Figure . In the The lunate begins to chondrify at Streeter's 18th and 19th horizons of embryonic development [As previously mentioned, congenital bipartite lunate is a rare occurrence and may lead to diagnostic difficulties in the setting of acute wrist trauma and pain. However, we did not consider the divided lunate to be a fracture, because the MRI and plain radiograph images showed that each part was well corticated with no evidence of callus formation. We concluded that our case was also unlikely to be pseudoarthrosis after a previous lunate fracture because there was neither a history of trauma to the wrist nor evidence of degenerative change. Last, the patient did not display any radiographic signs suggestive of Kienböck's disease, such as lunate fragmentation due to devascularized, necrotic bone and decreased T1-weighted signal intensity on MRI images .Injuries to the wrist and carpal bones are extremely common, and unless there is awareness about congenital anomalies such as the bipartite lunate, scaphoid and hamate, confusion and misdiagnoses may arise.The authors declare that they have no competing interests.Written informed consent was obtained from the parents of 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.EE identified the patient and performed the initial investigation and examination. BL undertook further investigation and wrote the manuscript. JH was the supervising consultant and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


Brown tumors are rare focal giant cell lesions of the bone caused by primary hyperparathyroidism (HPT). Brown tumor was reported in 1891; it presents as the end-stage findings of HPT. Common involvements are skull and pelvic girdle. We describe a case of 46-year-old female hemodialysis patient, with secondary HPT in whom multiple masses lesions of the left maxillary sinus and frontal bone were radiologically suspected to be brown tumor. This unusual manifestation of secondary HPT can be expected to occur with increased longevity of patients with renal failure and illustrates the need to include brown tumor in the differential diagnosis. A 46-year-old woman with end-stage renal failure from hypertensive nephrosclerosis that had been treated by hemodialysis for 11 year presented to the Emergency Room at EMMS Nazareth Hospital with vomiting after head injury. Immediate investigations including laboratory testing and head imaging (noncontrast computed tomography—CT scan) were performed to exclude intracranial hemorrhage. The patient recovered from vomiting in the next day, and she was asymptomatic regarding the lesions in the skull.Our patient's past medical history reveals that she was in relatively good health until the age of 26 when she started to complain of severe and colicky pain, radiating from the back, down the flank, and into the groin not improved by changes in position accompanied by nausea and vomiting. In April 1993, clinical diagnosis and evaluations revealed ESRD due to nephrolithiasis, and chronic hemodialysis treatment was started. In 2004, she received a kidney transplant from a cadaveric donor. In April 2009, she developed chronic allograft rejection, and hemodialysis treatment was restarted.In December 2012, the patient was referred to the emergency room at our hospital after head injury. Extended investigations excluded intracranial hemorrhage and fractures; on the other hand, other accidental findings were observed which include expansive sclerotic mass lesion within the left maxillary sinus (2.2 cm in diameter) without periosteal reaction  and an eThe lytic type of this entity mimics metastasis, and therefore diagnosis requires a systemic investigation and good radiological knowledge of this entity for lesion differentiation.The laboratory results were as follows. Her serum calcium level was 8.5 mg/dL . Her serum phosphorus level was 4.1 mg/dL . Her alkaline phosphate concentration was 406 UI/l , and her parathyroid hormone level was 1282 pg/mL , not to mention the fact that the patient refused to undergo surgical treatment. Other hormone levels were within their normal ranges. Renal functional tests showed a blood urea level of 126 mg/dL , a creatine level of 6.68 mg/dL, a uric acid level of 5.2 mg/dL , and a potassium level of 5.13 mEq/lt . The patient was treated for secondary hyperparathyroidism with vitamin D, calcium, phosphate binders, and reduced phosphate intake.Histological examination of the frontal bone lesion revealed giant bone cells and osteitis fibrosa cystica. Considering the clinical, radiographic, histopathological, and blood investigation findings, we reached a final consensus that our patient suffers from brown tumor.Brown tumor is a rare benign reactive, destructive process of bone caused by a clinical consequence of untreated severe primary, secondary, or tertiary hyperparathyroidism (HPT). A “brown tumor” is not a true neoplasm but a term commonly used for osteitis fibrosa cystica . In factThe term “brown tumor” is derived from the characteristic appearance of brownish material within the cystic lesion, which is caused by the vascularity, haemorrhage, and deposits of haemosiderin .It is well known that brown tumors, or osteoclastomas, are caused by localized, rapid, osteoclastic removal of bone secondary to the direct effects of PTH on the bone; it is actually a giant cell lesion and often appears as an expansile osteolytic lesion of the bone. They represent localized bony accumulations of fibrous tissue and giant cells that can occur in patients with primary, secondary, and tertiary HPT. Common involvements are long bones, ribs, clavicle, hand, skull, pelvic girdle, and mandible. While the mandible is the most frequently involved bone in the head and neck region, atypical involvement of the cranium in the area of the maxillary sinus and the frontal bone simultaneously, as presented in our case, is rare and had never been reported before in the literature .In 1891, Von Reckling Housen recognized the clinical impact of HPT and described osteitis fibrosa cystica as the pathognomonic bone lesion of this entity. In 1962, Mandle operated on a parathyroid tumor in a patient with hypercalcemia and radiological changes of osteitis fibrosa cystica and demonstrated postoperative regression of the bone disease and biochemical abnormalities.Incidence of primary HPT has been reported as approximately 5 per 10,000 populations per year. Among patients over 60 years of age the frequency is even higher, approaching nearly 1 per 1000 in men and twice that in women [HPT is a condition caused by high circulatory levels of parathyroid hormone. The various types of HPT manifest different laboratory findings. In primary HPT, the serum calcium level is usually elevated, with low or normal serum phosphate level. Patients with secondary HPT, on the other hand, usually present with hypocalcaemia and hyperphosphataemia. These findings are variable in tertiary HPT. In addition, HPT is also classified according to severity into 4 types: primary due to hyperplasia, benign, or malignant neoplasia of one or more of the parathyroid glands; secondary, when the parathyroid gland is stimulated to produce increased amount of hormone to correct abnormally low serum calcium levels in different physiologic or pathologic conditions resulting in parathyroid hyperplasia; Tertiary, when long-standing secondary hyperplasia becomes autonomous in spite of correction of the underlying stimulant; and a fourth type is ectopic HPT observed in patients with malignant disease .A tight relationship between chronic secondary HPT, and chronic renal failure (CRF) is well known. Most patients with CRF present some degree of secondary HPT and modifications of parathyroid glands begin early during the development of the renal disease . SecondaThe incidence of skeletal brown tumors in chronic renal failure (CRF) ranges from 1.5 to 13% but is expected to decrease with improvements in the medical care of these patients . This unHistologically, brown tumors are nonencapsulated and are characterized by abundant stroma consisting of fibrous connective tissue, with important proliferation of fibroblasts and several multinucleated osteoclast-like giant cells containing variable numbers of nuclei. Calcified material and areas with extravasations of red blood cell and hemosiderin in histiocyte can be found . The cysHistological features alone cannot establish a certain diagnosis of a brown tumor. Indeed, on clinical examination and using only routine panoramic radiography, the lesions may resemble osteosarcoma, bone metastases of a carcinoma, multiple myeloma, Langerhan's cell histiocytosis, Paget's disease, osteomyelitis, or osteonecrosis secondary to bisphosphonate therapy. The differential diagnosis is based on the clinical history and widespread skeletal involvement; pathological fractures and renal stones may suggest the presence of primary HPT. The diagnosis of HPT should be established by determination of the serum calcium, phosphorus, ALP, and PTH level rather than by histological examination of focal lesion alone. The main challenge in diagnosing brown tumor (as was the case of our patient) is that it can be seen in normocalcemic patients as well .Radiological examination: on CT scan, brown tumors appear as a lytic or sclerotic lesion without periosteal reaction. The lesion may be hyperdense or heterogeneous and well-circumscribed or expansile lucent lesion with a rim of calcification and remodeling of surrounding bone. After injection of contrast medium, brown tumors appear as heterogeneously enhancing masses . There iThe most significant point about the case described here is the simultaneous appearance of brown tumors in the left maxillary sinus “sclerotic pattern” and in the left frontal bone “lytic pattern” with amorphous calcifications and the general sclerosis of the cranial bones.In conclusion this case should attract the attention of general practitioner dentists, oral and maxillofacial surgeons, endocrinologists, and especially radiologists whose consultation is essential in including this entity in the differential diagnosis to avoid unnecessary surgical removal. Accurate diagnosis enabled the proper treatment to be carried out, avoiding unnecessary harm to the patient.


Angiomyolipomas are benign tumors of the kidney. Typical angiomyolipomas are usually recognized by identifying fat components before any intervention. On the contrary, solid renal masses without evident fatty components but containing calcifications on the computed tomography scan are suspicious for malignancy. However, as in this rare case, rules of diagnostic imaging are of exceptions.A 40-year-old man presented with left flank pain. The plain X-ray showed multiple coarse calcifications of 4.0 x 3.2 cm in diameter on the left upper quadrant abdomen. Computed tomography scan further revealed a solid renal mass and inside the mass there were calcifications. The size of the tumor was 5.6 × 5.5 × 6.3 cm. We performed a radical nephrectomy, and the histopathology showed a minimally fat-contained angiomyolipoma of multiple calcifications. The patient was free of recurrence or metastases after a follow-up period of 3 years.An angiomyolipoma containing calcification is rare. An angiomyolipoma with minimal fat concomitant with calcifications is an even rarer presentation. It is very difficult to differentiate a minimal-fat angiomyolipoma with calcifications from a renal cell carcinoma preoperatively. In such a circumstance, a well-planned partial nephrectomy may be optimal for the patient, regardless of the tumor size. Angiomyolipomas (AMLs) of the kidney can be diagnosed mostly based on intratumoral fat components on computed tomography (CT) examinations. AMLs coA 40-year-old man presented with intermittent left flank pain for one month. He denied having fever or any voiding symptoms. The physical examination was unremarkable. There was no weight loss, night sweat, or other constitutional symptoms.A plan X-ray showed multiple amorphous calcifications in the patient’s left upper abdomen; the calcifications were 4.0 × 3.2 cm in diameter on the left upper quadrant abdomen. Intravenous urography (IVU) disclosed a mass-like defect with multiple calcifications in the middle of the left kidney but without any obstruction. Incomplete ureteral duplication was also found incidentally in the right kidney. CT scans further revealed a 5.6 × 5.5 × 6.3 cm, relatively well-circumscribed, heterogeneous, bulging mass lesion with same coarse calcifications in the middle of the left kidney Figure . The denWe therefore performed a transperitoneal left radical nephrectomy. Grossly it was an encapsulated solid tumor measuring 6.0 × 6.0 × 5.2 cm mixed with reddish and yellowish material Figure a. The hyAn AML is a benign tumor lesion of the kidney composed of adipose tissue, smooth muscle and thick-walled blood vessels. The incAMLs contain a variable proportion of fat as the leading presentation on CT examination and this is the most important characteristic for diagnosis. On T1-wRenal epithelioid angiomyolipomas (EAMLs), also known as epithelioid PEComas, were distinct variants of AMLs, and have been reported to be less fat-contained, and more aggressive than the other common AMLs. In a report by Froemming and colleagues, 6 out of 9 cases of EAMLs had small amount of fat seen by preoperative imaging. They concluded that EAMLs can be indistinguishable from a RCC preoperatively,11.There are only four case reports on AMLs with calcification-5. RCCs Merran et al. suggested that when the fat components fill most of the renal tumor, an AML is the more likely diagnosis; on the contrary when the predominant component is calcification, a RCC is more likely than an AML. DiffusiThe unique nature of this case is that a preoperative CT examination showed a heterogeneously contrast-enhancing mass lesion occupying the left kidney with obvious calcification. The calcifications may be the result of previous hemorrhage, but this is only our speculation. We also did IVU and MRI to evaluate the possibility of renal stones or other renal malignancy. However, these examinations did not show other significant findings.An AML containing small amount of fatty components along with multiple coarse calcifications is extremely rare. It is very challenge to differentiate minimal-fat AMLs from RCCs preoperatively, as is seen in our case herein. With this radiological pattern, epitheloid AMLs should be included in the differential diagnosis in the future.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 of this journal.The authors declare that they have no competing interest.All authors participated in the patient’s diagnosis and medical management. Chen JL drafted the first version of the manuscript and Cha TL drafted the revised manuscript. All authors have read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2369/14/160/prepub


Abdominal wall hematomas are an uncommon cause of acute abdominal pain and are often misdiagnosed. They are more common in elderly individuals, particularly in those under anticoagulant therapy. Most abdominal wall hematomas occur in the rectus sheath, and hematomas within the oblique muscle are very rare and are poorly described in the literature. Here we report the case of an oblique muscle hematoma in a middle-aged patient who was not under anticoagulant therapy.A 42-year-old Japanese man presented with a painful, enlarging, lateral abdominal wall mass, which appeared after playing baseball. Abdominal computed tomography and ultrasonography showed a large soft tissue mass located in the patient’s left internal oblique muscle. A diagnosis of a lateral oblique muscle hematoma was made and the patient was treated conservatively.Physicians should consider an oblique muscle hematoma during the initial differential diagnosis of pain in the lateral abdominal wall even in the absence of anticoagulant therapy or trauma. Abdominal wall hematomas are an uncommon cause of acute abdominal pain and are often misdiagnosed. They result from rupture of the epigastric vessels or the deep circumflex iliac artery (rarely), or from tears of the fibers of the rectus abdominis or lateral oblique muscles,2. They 3 cells/μl, prothrombin time was 11.8 s, international normalized ratio was 0.99, and activated partial thromboplastin time was 32.6 s.A 42-year-old man presented with a painful, enlarging, lateral abdominal wall mass, which appeared after playing baseball. He gave no history of direct abdominal trauma such as collision with another player. He had a history of hyperuricemia; however, he had not undergone any therapy for the same. There was no family history of bleeding diathesis or hematological diseases. On physical examination, his vital signs were as follows: temperature 36.8°C, blood pressure 108/69 mm Hg, pulse rate 58 beats/min, and respiratory rate 12 breaths/min. A firm mass was palpable over the left lateral abdominal wall and it was tender. The skin color of the area was normal. Blood biochemistry on laboratory examination was within normal ranges and the platelet count was 174 ×10Ultrasonography (US) showed a heteroechoic, well-defined mass in the patient’s left lateral abdominal wall Figure. An emerAbdominal wall hematomas are one of the causes of acute abdominal pain. A rectus sheath hematoma caused by the rupture of the epigastric artery is a rare, but well-described, manifestation of abdominal hematoma. On the other hand, an oblique muscle hematoma caused by a rupture of the deep circumflex iliac artery is very rare. The mosMany risk factors have been reported for abdominal wall hematomas. These include aging, anticoagulant therapy, platelet disorders, trauma, recent surgery, injection procedures, and physical exercise as well as increased intraabdominal pressure from coughing, sneezing, vomiting, or straining during urination, defecation, or labor. In the An abdominal wall mass with ecchymosis is the most important diagnostic finding for suspicion of a hematoma. However, abdominal wall ecchymosis is a late sign and the average time between its presentation and its onset takes about 4 days, as reported in the literature. FurtherThe diagnosis of an oblique muscle hematoma is made by combining medical history, laboratory examination findings, and US and/or radiological findings. US and CT scans can provide useful information for differential diagnosis to avoid unnecessary surgery. US can Although there is no grading for an oblique muscle hematoma because of its rarity, the following grading system has been established for a rectus sheath hematoma on the basis of CT findings. Grade I is an intramuscular hematoma with an observable increase in muscle size. Grade II is also an intramuscular hematoma but with blood between the muscle and transversalis fascia. Grade III hematoma may or may not affect the muscle and blood is seen between the transversalis fascia and muscle in the peritoneum and prevesical space that results in a drop in hemoglobin. Grade IConservative treatment including bed rest and analgesics are appropriate in most patients with abdominal wall hematomas. Although most are self-limiting because the bleeding usually stops without intervention, some patients show significant morbidity and the overall mortality rate is reported to be 4%. Surgical intervention or transcatheter arterial embolization is recommended when conservative management fails,4. In ouAn oblique muscle hematoma is a very rare condition. In the clinical setting, abdominal US and contrast-enhanced CT are useful for a diagnosis. A correct diagnosis is important to avoid increasing morbidity or unnecessary surgical intervention. Treatment is mainly conservative and includes pain management. Physicians should consider an oblique muscle hematoma in the initial differential diagnosis of abdominal pain even in the absence of history of anticoagulant therapy or obvious trauma.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.MS wrote the manuscript. TK contributed to the diagnosis and revised the manuscript. MK and KS reviewed the literature. All authors contributed intellectual content, have read and approved the final manuscript.


Intussusceptions in adults is rare. Gastrointestinal lipomas are rare benign tumors and intussusceptions due to a gastrointestinal lipoma constitutes an infrequent clinical entity. Lipoma may develop as a benign tumor in all organs and rarely in large or small intestine. The present report describes a case of jejunojejunal intussusceptions in an adult with a history of colicky upper abdominal pain. Ileo-ileal invagination was diagnosed by computed tomography scan. Exploratory laparotomy revealed jejunojejunal intussusceptions secondary to a lipoma which was successfully treated with segmental intestinal resection. A review of the literature is also performed regarding this rare association revealing the diagnostic and therapeutic debates that exist.L’invagination chez les adultes est rare. Les lipomes gastro-intestinaux sont de rares tumeurs bénignes et l’invagination intestinale due à un lipome gastro-intestinal constitue une entité clinique trés rare. Le lipome peut se développer comme une tumeur bénigne dans tous les organes et rarement dans l’intestin grêle ou le colon. Le présent rapport décrit un cas d’invagination jéjunojéjunale chez un adulte avec une histoire de douleurs abdominales. Iléo-iléale invagination a été diagnostiquée par tomodensitométrie. Une laparotomie exploratrice a révélé l’existence d’une invagination jéjunojéjunale secondaire à un lipome qui a été traitée avec succès par une résection intestinale segmentaire. Une revue de la littérature est également effectuée au sujet de cette association rare révélant les débats diagnostiques et thérapeutiques qui existent. Intussusceptions was reported for the first time in 1674 by Barbette of Amsterdam. In contA 35-year-old man was admitted to the emergency department in a tertiary referral hospital with 4 months history of intermittent upper abdominal pain accompanied with nausea. The patient had no past history of peptic ulcer disease, alteration in bowel habits, melena or weight loss. On examination, he was apyrexial and hemodynamically stable. His abdomen was distended and no palpable abdominal masses; bowel sounds were hyper audible. Initial A rectal examination revealed no masses or blood. Laboratory blood tests were normal. Abdominal radiography revealed prominent dilatation of the small bowel with air fluid levels Figure . AbdominIntussusceptions in adulthood is unusual, with an incidence of approximately 2-3 cases per population of 1 000 000 per year. The mosThe lipoma is a rare benign tumor of the digestive tract. The diagnosis of intussusceptions in adults can be difficult because of atypical and episodic symptoms. A high level of clinical suspicion and an abdominal CT scan are most useful tools for making a timely diagnosis. Surgical resection remains the treatment of choice and produces an excellent prognosis.Written informed consent was obtained from the patient for publication of this case report and accompanying imagesCT: Computed tomography; MRI: Magnetic resonance imaging; CS: Colonoscopy; ECS: Enema contrast study; EGD: Esophagogastroduodenoscopy; US: Ultrasonography; ND: Not described.The authors declare that they have no competing interests.All of the authors were involved in the preparation of this manuscript. OM performed the operation and revised the manuscript. HH was an assistant surgeon and made substantial contributions to conception and design. LC described histological finding and was involved in drafting the manuscript. All authors read and approved the final manuscript.


Mucinous cystadenomas are relatively uncommon benign cystic tumors. Most of them are found in the ovary, pancreas, and appendix. Primary mucinous cystadenoma of spleen is extremely rare and only countable cases have been recorded. These are assumed to arise from heterotopic pancreatic tissue or invaginated spleenic capsular mesothelium. We present a rare case of primary mucinous cystadenoma of spleen without pancreatic tissue in it. Exact tissue of origin could not be determined.Cystadenoma; Splenic cyst; Pseudomyxoma peritonei Mucinous cystadenomas are relatively uncommon benign cystic tumors. Most of them are found in the ovary, pancreas, and appendix. However, they have also been identified in other unusual sites such as retroperitoneum, fallopian tube, lung, urinary bladder, and liver. Primary mucinous cystadenoma of spleen has been reported very rarely. In these cases it was presumed that the tumor arose either from invaginated capsular mesothelium of the spleen or from heterotopic pancreatic ,2 or entA 22-year-old male presented with dull aching pain associated with a slow growing lump in left hypochondrium for 3 months. Physical examination revealed a well-defined ovoid, nontender, non pulsatile, smooth surfaced mass of size approximately 24 x 16 cm, that involved the left hypochondrium, epigastrium and umbilical regions, and was moving with respiration. Ultrasonography (USG) abdomen suggested inferiorly displaced spleen due to a large well-defined hypoechoic cystic mass of size 15 x 10 cm. Barium meal examination showed extra luminal indentation of left lateral wall of stomach. Passage of barium through stomach and duodenum was smooth. Magnetic resonance imaging (MRI) demonstrated a large well-defined, unilocular, cystic, encapsulated lesion of size 20.2 x 19.3 x 15.1 cm in left subdiaphgramatic region, arising form spleen, with hyper intense signal on both T1 and T2, producing marked compression and displacement of adjacent structures . PancreaOn exploratory laparotomy, a tense and cystic mass, of size 2015 cm, arising from the diaphragmatic surface of spleen, involving the whole left hypochondrium with extension into the umbilical region and epigastric region displacing the stomach to right side, was found. Other common sites for cystic lesion like pancreas, appendix, mesentery, and liver were found normal. Splenectomy was done. The cut surface showed unilocular cystic mass with smooth inner surface and profuse insipissated mucus. Microscopically, papillary structure was found on the inner surface of cystic space, which was lined by a single layer of mucin-producing epithelial cells without malignant change. The splenic tissue was outside the cystic wall. Histopathological examination was suggestive of unilocular mucinous cystadenoma of spleen . No pancSplenic tumors are uncommon neoplasms. Morgenstern et al. classified splenic tumors into four categories: lymphoid tumors, non-lymphoid tumors, metastatic tumors, and tumor-like lesions, such as cysts and hamartomas. The most common non-lymphoid tumors are vascular tumors. However, primary splenic tumors arising from epithelial origin are extremely rare .Splenic mucinous cystic tumors are defined as cystic spaces lined by mucin-producing columnar cells and ranged from benign cystadenoma to frankly malignant cystadenocarcinoma. Primary mucinous cystadenoma of spleen is an extremely rare finding ,4,6,7. TSimple mucinous cyst of spleen is a rare and close differential diagnosis, which may rarely present as pseudomyxoma peritonei . DistincIn conclusion,a rare case of primary mucinous cystadenoma of spleen is presented. Tissue of origin may or may not be identifiable, but it is hetertopic pancreas usually or invaginated splenic capsular mesothelium.


Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm that mainly arises from the lymphoid tissues of the immune system. Although this neoplasm typically occurs anywhere along the lymph nodes, it can also be found at extranodal sites, especially in the head and neck. We experienced a rare case of extranodal IDCS in the nasal cavity, a location that has not been previously reported. A 73-year-old woman presented with a polyp-like mass in the nasal cavity and underwent endoscopic sinus surgery. A histologic study confirmed the mass as IDCS by immunohistochemistry with S-100 antibody, and postoperative adjuvant radiotherapy was administered. Although the incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of nasal cavity masses. Tumors arising from dendritic cells, such as follicular dendritic cell sarcoma/tumor (FDCS) and interdigitating dendritic cell sarcoma/tumor (IDCS) (WHO), are extremely rare , 2. Althα, CD21, EMA, and D2-40. Postoperative MRI showed no residual tumor or significantly enlarged cervical lymph nodes. Adjuvant radiotherapy was administered for 2 months at 59.4 Gy at 1.8 Gy per fraction to the tumor bed area. The patient had xerostomia induced by radiation but this was tolerable. Followup PNS MRI or PET-CT revealed no abnormal FDG uptake, suggesting that there was no tumor recurrence or distant metastasis. The patient has been disease-free with no evidence of recurrence after 1.8-year followup.A 73-year-old woman visited our clinic and presented with a nasal obstruction and frequent blood-tinged crust that had persisted for 4 months. Initial endoscopic findings revealed a soft and fungating mass, which appeared as a nasal polyp in the middle meatus of the right nasal cavity. The mass was partially attached to the septum and was more than 1 cm long in its greatest dimension. Although it had no bleeding tendencies, hyperemic mucosa and some blood clots were noted around the mass. Paranasal sinus computed tomography (PNS CT) revealed a 2.2 cm polyp in the right middle meatus without sinusitis . The masDendritic cells are antigen-presenting cells that are heterogeneous groups of nonlymphoid and nonphagocytic immune accessory cells, which are present in lymphoid or nonlymphoid organs. Four types of dendritic cell exist in lymph nodes: follicular, interdigitating, Langerhans, and histiocytic/fibroblastic cells . Among tα, CD21, CD35, CD3, or CD20 [α.The diagnosis of IDCS is confirmed histologically by staining with hematoxylin and eosin as well as various antibodies, which can recognize antigens expressed in follicular and interdigitating dendritic cells. Microscopically, IDCS has large fusiform spindle cells with indistinct cell borders that usually form a storiform or whorled growth pattern. The shape of the nucleoli is oval with finely dispersed chromatin. The nucleoli are small but prominent and sometimes multinucleated . IDCS is or CD20 . In our α-positive status can be used to diagnose follicular dendritic cell sarcoma, rhabdomyosarcoma, or leiomyosarcoma [Many diseases are included in the differential diagnosis of extranodal interdigitating sarcoma in the nasal cavity: sarcomatoid carcinoma, melanoma, malignant peripheral nerve sheath tumor, atypical fibroxanthoma, malignant fibrous histiocytoma, rhabdomyosarcoma, and leiomyosarcoma . These nosarcoma .Usual treatments for IDCS consist of surgical excision, systemic chemotherapy, or radiotherapy. Surgical resection is the mainstay of treatment in patients with localized disease. Radiation therapy, alone or with multiagent chemotherapy, has been used in several cases . HoweverFDCS, a different type of dendritic cell sarcoma, has poor prognostic factors such as intra-abdominal location, size greater than 6 cm, mitotic count greater than 5/10 high-powered fields, coagulative necrosis, significant nuclear pleomorphism, or lack of adjuvant therapy . CompareIDCS is an extremely unusual neoplasm that affects the lymphatic system as well as extranodal sites. Here, we presented a rare case of IDCS in the nasal cavity, which has been successfully treated with combined treatment of endoscopic sinus surgery and adjuvant radiotherapy. Although the incidence of IDCS in the nasal cavity is low, our case suggests that extranodal IDCS should be considered in the differential diagnosis of nasal cavity masses in the head and neck that are confirmed by immunohistochemistry.


Introduction. Concomitant cholecystitis and gallstone pancreatitis is an infrequent clinical encounter, reported sparsely in the literature. Concurrent acute cholecystitis and pancreatitis complicated by gall bladder perforation has not been reported before. Presentation of Case. We report a 39-year-old female presenting with concomitant cholecystitis and acute pancreatitis, complicated by gallbladder perforation. Discussion. There is much controversy surrounding the timing of cholecystectomy following gallstone pancreatitis, with the recent literature suggesting that “early” operation is safe. In the current case, gallbladder perforation altered the “routine” management of gallstone pancreatitis and posed as a management dilemma. Conclusion. Clinical judgement dictated timing of operative management and ultimately cholecystectomy was performed safely. The patient was classed as grade 1 pancreatitis using Ranson's criteria [A 39-year-old female, previously well, was admitted with a diagnosis of acute pancreatitis with concurrent acute calculus cholecystitis. The patient presented with a one-day history of acute onset sharp pain in the epigastrium, associated with nausea and vomiting. She was exquisitely tender in the epigastrium and right upper quadrant, with no peritoneal signs on examination. Laboratory investigations showed a white cell count of 14.2 × 10The patient was initially managed with empiric parental antibiotics; ceftriaxone 1 g daily and metronidazole 500 mg 8 hourly. On day 2 of admission, liver function became increasingly deranged, with bilirubin increasing to 77 U/L. The patient complained of acute exacerbation of pain and repeat sonography showed worsening cholecystitis with a high suspicion of a small gallbladder posterior wall perforation, associated with an increase of pericholecystic fluid collection . ComputeThe patient underwent a semielective laparoscopic cholecystectomy. Intraoperatively, the diagnosis of perforated necrotic cholecystitis was confirmed with 600 mls of bilious free fluid within the peritoneum . Intra-oConcomitant acute cholecystitis and gallstone pancreatitis is common clinical constellation that has been documented in the literature. However, to the authors' knowledge, no such case of gallbladder perforation in conjunction with pancreatitis has been previously documented. The current case had clear biochemical and radiological confirmation of both pancreatitis and cholecystitis. Pancreatitis was established on presentation by clinical symptomatology and serum lipase exceeding four times the upper limit. Later, radiological evidence of peripancreatic fat stranding on CT and dilated pancreatic duct on intraoperative cholangiogram  confirmeCalculus cholecystitis with gallstone pancreatitis has been well documented. Danielle Dietz  reportedCurrent practice for uncomplicated mild biliary pancreatitis is controversial. Historically, early cholecystectomy has been avoided due to the belief that the procedure would be complicated by difficult dissection caused by oedema. However, recent literature suggests early cholecystectomy can be performed with minimal change in morbidity and mortality . UnfortuPatients managed by interval cholecystectomy are at significantly increased risk of representation due to biliary disease, whether biliary colic or repeat pancreatitis. Ito et al. reviewed patients who underwent interval cholecystectomy and noted that up to 31% of patients had recurrent gall-stone events within 2 weeks of discharge . These fIn the current case, radiological confirmation of perforated cholecystitis in the setting of acute pancreatitis was made. These findings ultimately dictated the timing of early cholecystectomy. This is a rare case report of concomitant perforated cholecystitis and acute pancreatitis. We conclude that in the emergency setting, laparoscopic cholecystectomy may be performed in the context of pancreatitis.


Well differentiated liposarcoma (WDLS) is the commonest subtype of liposarcoma. Recognised subtypes of WDLSs are lipoma-like, sclerosing, spindle cell and inflammatory. The inflammatory variant of WDLS also known as “lymphocyte-rich liposarcoma” is rare. We present a case of inflammatory WDLS occurring in the retroperitoneum, in a patient with a past history of non-Hodgkin lymphoma. We outline the histological features, discuss the differential diagnoses and highlight the diagnostic pitfalls in interpretation of this lesion on fine needle biopsy. Well-differentiated liposarcoma (WDLS) is the commonest subtype of liposarcoma, accounting for 40%–45% of all cases . BecauseIn 2005, a 67-year-old man of Uruguayan origin, with a background history of ischaemic heart disease, psoriasis, and reflux oesophagitis, underwent resection of a retroperitoneal inflammatory and sclerosing WDLS after investigation of abdominal pain. In 2006, diffuse large B cell lymphoma (DLBCL) was subsequently diagnosed in the femoral head and left ilium and treated with radiotherapy. In 2008 he experienced left-sided abdominal pain and was found to have recurrent nodules in the left renal bed. A computed tomography (CT) abdominal scan showed a mass in the surgical bed of the retroperitoneum containing multiple soft-tissue nodules and fat, suggestive of recurrent retroperitoneal liposarcoma. A fine needle biopsy (FNB) showed a mixed population of lymphoid cells consisting predominantly of medium-sized and large cells with a lesser number of small lymphocytes . The medMacroscopically, the specimen consisted of a partially encapsulated, circumscribed mass of fatty tissue 130 × 90 × 40 mm. Serial slicing showed that it had a variegated nodular appearance with fibrous septa. Centrally there was light yellow, mostly uniform, fatty tissue, whilst peripherally there were cream-coloured firm nodules measuring from 5 to 12 mm across, some of which coalesced . On micrMost cases of inflammatory liposarcoma have been reported in the retroperitoneum. However, individual case reports of inflammatory liposarcoma have also been described in the pleura , scrotumFNB of the left renal bed in the current case was interpreted as an atypical lymphoid infiltrate (suspicious for recurrent non-Hodgkin lymphoma) rather than recurrent liposarcoma, probably because the FNB represented only sampling of the lymphoid component. In particular, it likely represented sampling of reactive germinal centres, giving rise to a seemingly predominant population of large lymphoid cells. The background history of DLBCL probably also biased the cytological interpretation. The distinction between non-Hodgkin lymphoma and inflammatory liposarcoma is important because the management and prognosis of both entities are so distinctly different that misdiagnosis may lead to an adverse outcome. In view of the microscopic features of the cellular foci of inflammatory WDLS, possibilities such as inflammatory myofibroblastic tumour, lymphoma (either Hodgkin or non-Hodgkin), or Castleman's disease may enter the differential diagnosis. Unless the atypical lipomatous component is identified and the adipocytic nature of the tumour recognised, misdiagnosis is likely to occur. If the lipomatous component is obscured by the intense inflammatory infiltrate   or the cOther differential diagnoses that may be considered microscopically include sclerosing mesenteritis and retroperitoneal fibrosis. In contrast to inflammatory WDLS, the former is characterised by a combination of fibrosis, chronic inflammation, and fat necrosis. The latter, as the name suggests, shows fibrosis encasing retroperitoneal structures, with admixed lymphocytes and plasma cells. Both of these disorders lack the large hyperchromatic cells and lipoblasts of WDLS. It is not surprising that inflammatory WDLS can be confused with inflammatory myofibroblastic tumour, as cellular foci in the former may have a similar appearance. Inflammatory myofibroblastic tumour lacks the atypical adipocytic component of WDLS and shows positive ALK staining in approximately 50% of cases. The so-called inflammatory malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma) may also be considered in the differential diagnosis of inflammatory WDLS. However, the spindle cells of the latter show less cytological atypia. Thorough sampling of the different regions of the tumour to facilitate identification of an atypical lipomatous component should enable a diagnosis of inflammatory WDLS to be established.The recognition of inflammatory WDLS is difficult on limited sampling specimens such as FNB samples particularly if the atypical adipocytic component is not sampled or recognised. However, in many instances, awareness of this rare variant of WDLS, knowledge of its pathological features, and careful clinicopathological correlation will enable accurate diagnosis.


Extrauterine endometrial stromal sarcoma (ESS) is rare and typified by delayed recurrence of primary ESS. Here, we report an unusual case of colonic ESS in a woman with a remote history of hysterectomy. An 80-year-old woman, with a history of hysterectomy and bilateral salpingo-oophorectomy for abnormal bleeding and endometriosis 37 years prior to presentation, was diagnosed with ESS in the colon. She was treated with laparoscopic low anterior resection, followed by megestrol acetate, and has been in remission for more than 4 years. This case highlights the rarity of extra-uterine ESS in the colon, especially in the absence of a known history of primary uterine ESS. The patient's history of endometriosis may have been a predisposing risk factor. ESS in the colon may be treated successfully with surgical resection and progestin therapy. Indefinite surveillance is recommended to monitor for late recurrences. Endometrial stromal sarcoma (ESS) tumors account for approximately 0.2% of all uterine malignancies . These tAn 80-year-old woman, gravida 3, para 2, with a history of total abdominal hysterectomy and bilateral salpingo-oophorectomy for abnormal bleeding and endometriosis performed 37 years prior, presented to the hospital with bright red rectal bleeding and chronic rectal discharge. She denied vaginal bleeding or discharge. She had been prescribed raloxifene for osteoporosis prevention and intermittent treatment with vaginal conjugated estrogen cream for urogenital atrophy, but had not seen a gynecologist since her hysterectomy. Colonoscopy and biopsy were negative for malignancy. Further evaluation with computed tomography (CT) imaging demonstrated a 5 cm soft tissue mass in the pelvis involving the sigmoid colon. She underwent a laparoscopic-assisted low anterior colon resection and biopsy of left pelvic sidewall and omental nodules. The sigmoid colon mass revealed a low-grade endometrial stromal sarcoma, involving the mucosa, muscularis, and adjacent peritoneal tissue, including three pericolonic soft tissue nodules and the left pelvic sidewall nodule. The proximal and distal margins, 18 regional lymph nodes, and omental biopsy were negative for malignancy. The tumor showed the characteristic morphology of a low-grade spindle neoplasm with uniform proliferation of small round to oval cells, minimal nuclear atypia or mitoses, and small nucleoli . Small, The patient was treated with megestrol acetate 80 mg by mouth twice a day, decreased to 40 mg daily and eventually discontinued after 3 years, due to the patient's inability to tolerate the side effects. The patient has been followed closely with examination, CT imaging and endoscopy, and she has remained without evidence of recurrent ESS for over 4 years. This case is one of only two reported cases of extra-uterine low-grade ESS diagnosed more than 30 years after hysterectomy for benign disease. The other case was a patient with a remote history of hysterectomy for leiomyoma 38 years prior to the diagnosis of low-grade ESS in the small bowel . The eti ESS of the colon is exceedingly rare, with only 7 reported cases in the literature –8. DocumPrimary extra-uterine ESS is more common in premenopausal women , 9–11, sIn summary, we report a rare case of extra-uterine ESS in the colon diagnosed 37 years after hysterectomy reportedly for abnormal bleeding and endometriosis. Endometriosis was not confirmed histologically at the time of this patient's colonic resection. However, based on the strong association with extra-uterine ESS and endometriosis observed in the literature this patient's tumor may have arisen from transformation of pelvic peritoneal endometriosis. We acknowledge that the unavailability of the pathological slides from the previous hysterectomy and lack of endometriosis on the colonic resection are the biggest limitations in making this assessment. Nevertheless, the possibility of ESS should be considered in the diagnosis of solid stromal tumors in the gastrointestinal tract in women with a history of endometriosis since the majority of cases in the literature report this association. Management with surgical resection is recommended, and adjuvant progestin therapy is reasonable. As in this case, long-term remissions can be achieved. However, continuous followup is recommended because of the risk of delayed recurrence.


Although uterine leiomyomas are the most common neoplasms of the female genital tract, this is not the case when referring to women under the age of 20. Only a few cases of uterus leiomyomas have been reported in this age. Preoperative imaging evaluation is mandatory in adolescent women for the accurate detection, localization, and characterization of uterus leiomyomas. We report a case of a 16-year-old girl admitted to our hospital for pain and abdominal distention. The patient underwent multidetector CT examination of the abdomen and MR examination of the pelvis. Both imaging modalities revealed uterine enlargement and the presence of innumerable variably sized leiomyomas. Histopathologic examination following exploratory laparotomy confirmed the presence of uterus leiomyomas. The patient underwent laparoscopic myomectomy two years after the first operation, following MR examination of the pelvis. Leiomyomas, also known as fibroids or fibromas represent the most common uterine neoplasm, occurring in 20–30% of women between the ages of 35 and 50 –4. HowevWe present a case of a 16-year-old girl with fibromatous uterus, evaluated with multidetector CT and MR imaging examination. As to our knowledge, this is the first report of a uterus with multiple fibroids in an adolescent girl in the English literature, although there are few reports of solitary uterus leiomyomas in this age population –11. The A 16-year-old female patient was referred to the emergency unit of our hospital for abdominal pain and distention. Patient's gynecologic history was unremarkable. Menarche occurred at the age of 13, and menses had been regular ever since. From the family history, her mother reported diabetes mellitus.Physical examination revealed the presence of a relatively firm pelvic mass, of probably uterine origin. Laboratory analysis showed a mild anemia and a slight elevation of CA 125 levels (40 U/ml). The possibility of pregnancy was excluded after a negative pregnancy test.Ultrasonography, both transabdominal and transvaginal, showed globular uterus enlargement and multiple hypoechoic or heterogeneous masses, probably representing leiomyomatous cores, causing distortion of the central endometrial echo. Multidetector CT examination of the abdomen on a 16-row CT scanner was followed. On CT, an enlarged uterus, with lobular, deformed contour was detected, filling the pelvis and extending up to the level of the lower pole of the kidneys. Multiple uterus leiomyomas, of variable size were found, heterogeneously enhancing after contrast material administration . NeitherFinally, the patient underwent MR imaging examination of the pelvis on a 1.5 Tesla unit. An enlarged, posteriorly inclined uterus was found . The preImaging findings were diagnostic for the presence of fibromatous uterus. The patient underwent exploratory laparotomy. An extremely enlarged uterus, with multiple and variably sized fibroids, the largest of which about 10 cm in maximal diameter was found in surgery. Frozen section pathologic examination confirmed the presence of uterus leiomyomas. Most of leiomyomas were excised; some left in place due to their close relation to the uterine vessels. For histologic examination, 19 discrete fairly well-circumscribed nodules were received. They measured 1–13 cm in maximal diameter. On cut section, the nodules were well whitish, with whorled appearance and fibroelastic consistency. No areas of hemorrhage or necrosis were found. On microscopic examination, all nodules were composed of relatively uniform spindle cells with vesicular nuclei, arranged mostly in interlacing bundles and embedded within a collagenous stroma . The mitThe patient was instructed to have pelvic follow-up sonograms on 6-months intervals. MR imaging examination performed two years after surgery, when the patient was admitted to the Gynecology clinic with lower abdominal pain, revealed recurrence of fibromatous uterus . LaparosUterus leiomyomas are extremely uncommon in the paediatric and adolescent population –11. ApprEstrogens and progesterone play an important role in the development of these neoplasms , 5–8, 15Leiomyomas in the young population often show histologic features favouring the diagnosis of malignancy; half of the reported cases demonstrated increased cellularity, mitotic activity, and cellular atypia . These pUterine leiomyomas, although rare they should be considered in adolescent women presenting with a pelvic mass and abdominal pain, as in this case, or menstrual disorders and abnormal uterine bleeding. The management of leiomyomas in this age should be conservative for the preservation of fertility. Therefore, the preoperative characterization of the nature of these tumors is extremely important. The diagnosis should be based on imaging findings, that is, sonographic and magnetic resonance imaging features.Ultrasonography is well established as the primary method for the evaluation of the female genital tract , 18. It MR imaging is considered the most accurate technique for the detection and localization of leiomyomas, proved more accurate than US , 19–22. Differential diagnosis of uterus leiomyomas should also include uterus leiomyosarcoma . Althoug


Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate. Surgery is usually performed on large lesions. In the case of adenomatosis or multiple lesions, surgery will be adapted. Close followup is required in all cases.Benign liver tumors are very rare in children. Most focal nodular hyperplasia (FNH) remain sporadic, but predisposing factors exist, as follows: long-term cancer survivor (with an increasing incidence), portal deprivation in congenital or surgical portosystemic shunt. The aspect is atypical on imaging in two-thirds of cases. Biopsy of the tumor and the nontumoral liver is then required. Surgical resection will be discussed in the case of large tumors with or without symptoms. In the case of associated vascular disorder with portal deprivation, restoration of the portal flow will be discussed in the hope of seeing the involution of FNH. HepatoCellular Adenoma (HCA) is frequently associated with predisposing factors such as GSD type I and III, Fanconi anemia especially if androgen therapy is administered, CPSS, and SPSS. Adenomatosis has been reported in germline mutation of HNF1- Liver tumors are very rare in children, accounting for 1 to 4% of all pediatric tumors. Benign tumors account for 30 to 40% of these, with a majority of hemangiomas occurring during infancy. Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCAs) are extremely rare during childhood, and there are few published reported cases and series. Presentation, physiopathology, and management differ from adults.We will successively review the main characteristics of FNH and HCA in children and discuss physiopathology, followup, and therapeutic modalities based on a systematic review of the literature and our experience. Focal nodular hyperplasia (FNH) is not a neoplasm but a nonspecific hyperplasic reaction to vascular abnormalities. It is a well-delimitated lesion without capsules and characterized by hepatocytic nodules separated by fibrous bands. The mass has a central stellate fibrous region containing malformed vascular structures that include large arteries, without portal veins. Bile ductular reaction is usually present at the interface between hepatocytes and fibrous bands and is highly suggestive of the diagnosis of FNH. According to some authors, in FNH, arterial blood flows from the anomalous arteries via capillaries into sinusoids adjacent to the fibrous septa. The blood in the sinusoids drains to the hepatic vein either directly or via perinodular veins. The absence of portal vein branches in FNH leads to the absence of portal blood flow.The precise cause of FNH is unknown. Several theories have been suggested to explain the occurrence of FNH: vascularization by an anomalous large artery, acquired thrombosis, reactive hyperplasia after hepatocellular injury induced by vasculitis, or higher blood flow, either portal or arterial, compared with the surrounding tissues –8.FNH is very uncommon in children. About 200 cases have been reported in the literature, with few short series –14. It rFirst known as an incidental lesion, FNH can also be associated with predisposing factors such as chemotherapy and radiation therapy in children treated for malignancy, and portal deprivation in case of congenital or surgical portosystemic shunts  Figures  and 2. In the group of children with no predisposing factors, the incidence is estimated to be 0.5%. There is a female predominance as in adulthood. Mean age at diagnosis is between eight and 11 years , 2113, 2FNH appears on US as a well-delimitated, lobulated mass that is iso- or slightly hypo- or hyperechoic compared to the surrounding liver. On Doppler examination, it is usually fed by a large artery with a stellate structure of its branches within the tumor. Typical radiologic findings on imaging techniques using contrast enhancement include a solitary, homogeneous, and slightly hypoattenuating mass compared to the surrounding liver on unenhanced CT with rapid homogeneous contrast enhancement at the arterial phase . On venous phase, the mass becomes isodense as compared to the surrounding liver, while the central scar might be enhanced on the late phase. On MRI, FNH is usually hypo- or isointense to the surrounding liver on T1-weighted images and iso- to slightly hyperintense on T2-weighted images. Enhancement after gadolinium injection is similar to that observed on a CT scan , 24. TheThe diagnosis of FNH in children can be challenging as atypical lesions occur in about two-thirds of cases, and multiple lesions are more common in children than in adults . ImagingIn the group of children with a history of chemo- and/or radiotherapy, a major concern is to differentiate benign and malignant processes. Strong enhancement at the arterial or early portal phase is important to differentiate FNH or “FNH-like” lesions from metastases that usually remain hypointense on arterial or early portal phase when compared to the surrounding liver . Even ifIn the literature, few patients had conservative management of FNH. The natural history of these tumors is poorly known in children. All kinds of spontaneous evolution have been described, from spontaneous involution to growth  11]..11].In the literature, 78% of the patients with no history of malignancy or hemopathy had a surgical resection. Most masses were large , 12.In our practice, when the imaging features are typical on MR and/or CT, the patient is asymptomatic and there is no associated vascular disorder, biopsy is not performed, and conservative management is proposed with a prolonged followup by ultrasound to show if there is an increase in size.When the aspect is not typical, biopsies of the mass and the nontumoral liver are performed to assess the diagnosis of the tumor and search for associated abnormalities of the liver that could be part of an unknown predisposing factor for a tumor.Surgery is sometimes performed in the case of very large lesions, symptoms, and/or impairment of physical activities. Recurrence of FHN after surgery has been reported in one case in the literature, and we have a personal case, not published .FNH secondary to CPSS requires special management as the closure of the shunt with restoration of intrahepatic portal flow may lead to shrinkage of the tumor, as shown in previous cases . That isIn the case of SPSS, closure of the shunt should be discussed in regard to the patient's history. Restoration of intrahepatic portal flow by making a mesenterico-rex bypass should be performed when possible, mostly in the case of portal obstruction with cavernomatous transformation .Hepatocellular adenomas (HCAs) are extremely rare during childhood. They are benign liver tumors that represent a heterogeneous group in which histopathological features may vary according to the etiological background. Classically, HCAs are soft, well-demarcated tumors with little or no fibrous capsule. They are composed of liver cell plate mildly thickened or irregular. They are supplied by thin-walled arteries without other portal tract elements .α-inactivated HCA, β-catenin-activated HCA, and two forms of HCA without mutation of HNF1-α or β-catenin presenting either with or without inflammation. These different subtypes display variable clinical behavior, imaging findings, and natural history that have recently been well described [β-catenin mutations and lack of HNF 1α inactivation [In adults, genomic and molecular studies together with the analysis of genotype/phenotype correlations have led to the recognition of four major HCA subgroups: HNF1-escribed –36. To otivation . HCA formation is complex and varies according to the etiological background. Natural history and management vary with the context.According to the rare published pediatric series, HCA occurs in 0 to 21% of pediatric benign liver tumors. Differences in the frequency between series are probably related to the differences in patients' recruitments. The largest pediatric series reported 22 HCA in a 12-year period –41. Most HCAs are diagnosed during the teenage years (the mean age at diagnosis is around 14 years). HCAs reported before the age of one year are exceptional, the youngest patient being three weeks old in a context of multiple congenital anomalies .Sex ratio varies with series, but the female predominance observed in adults is not the rule in children, and male predominance is observed in several series . α gene, and familial adenomatosis polyposis , germline mutation of HNF1- Figures , and 6 [α-mutated HCA that account for 80% of all cases in adult series [Making the diagnosis of HCA by imaging can be challenging. In adults, major improvements in knowledge of HCA have been gained during recent years. Correlations of imaging with the genotype/phenotype classification proposed by the “Bordeaux” experience have made it possible to distinguish specific imaging patterns in relation with the two major subtypes, inflammatory HCA and HNF 1t series , 35, 44.Ultrasonography is usually the first exam performed in children for the evaluation of abdominal disorders and the first screening tool during followup in predisposed children, but it can miss small nodules isoechoic to the liver, especially in a steatotic liver. HCAs are typically heterogeneous and are well-delimited solid masses with vessels within the mass . Magnetic resonance imaging (MRI) is the best technique to depict lesions. HCAs patterns on MRI will depend on the amount of fat, hemorrhage, and necrosis within the mass. HCA are frequently heterogeneous on T1- and T2-weighted images (WIs) but with a high signal on T2 WI. Fat component in HCA is well demonstrated with chemical shift sequences as in “in-phase and out-of-phase” T1 sequence. It can also be shown by sequences with fat saturations that are less sensitive. When present, it can help diagnosis. Early enhancement after contrast injection is observed in most cases. An enhanced pseudocapsule can be visible on delayed acquisition. Washout should not be too early . Diffusiα mutation  and androgen therapy with or without FA, liver tumors can occur and concern about 3% of patients [α-foetoprotein test is not reliable as this biomarker has been found to be increased in about 85% of FA patients [patients . Most arpatients –65. Treapatients . Associapatients . The medpatients . Severalpatients , 67, andpatients , 64. Scrpatients .Heterozygous germline mutations of the hepatocyte nuclear factor-HNF1 alpha are associated with liver adenomatosis and maturity onset diabetes of the young (MODY 3) [α-foetoprotein levels should also be part of the screening. If liver adenomatosis is detected, survey and preventive surgical treatment should be discussed. Criteria that guide treatment include the number and size of the lesions, the presence of symptoms, and the surgical risk incurred by the patient. (MODY 3) , 68–71. (MODY 3) . Cases o(MODY 3) . SystemaCongenital or Surgical portosystemic shunts are associated with HCA, as reported in several cases [al cases , 23, 72.al cases . Regressβ-catenin mutation or uncertain diagnosis. Some authors propose resection of smaller HCA, between 3 and 5 cm, because of reported cases of malignant transformation [It is now well established in adults that complications depend not on the number of lesions but on the histologic subtype and size of the tumor. In adults, complete surgical resection is an effective option for HCA larger than 5 cm or in male or if HCA is associated with GSD. In our personal pediatric experience and in the pediatric literature, HCAs which are frequently larger than 5 cm are resected , 40. Surormation , 73. Theormation , 74, 75.ormation .Embolization can be performed in the case of hemorrhage.With predisposing factors such as GSD, androgen steroid therapy, CPSS, or SPSS, regression of HCA has been observed with metabolic control, androgen withdrawn, and closure of the shunt, respectively.Most FNH remain sporadic during childhood, but predisposing factors exist, as follows: long-term cancer survivor (with an increasing incidence) and portal deprivation in CPSS and SPSS. The aspect is atypical on imaging in two-thirds of cases. Biopsy of the tumor and the nontumoral liver is then required. Surgical resection will be discussed in the case of large tumors with or without symptoms. In the case of associated vascular disorder with portal deprivation, restoration of the portal flow will be discussed. This will be done either by closure of a congenital portosystemic shunt or by making a mesenterico-rex bypass in the cavernous transformation of the portal vein in the hope of seeing the involution of FNH.α. Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate. Surgery is usually performed on large lesions. In the case of adenomatosis or multiple lesions, surgery will be adapted. Close followup is required in all cases.HCA, which is very rare in children, is frequently associated with predisposing factors such as GSD type I and III, Fanconi anemia especially if androgen therapy is administered, CPSS, and SPSS. Adenomatosis has been reported in germline mutation of HNF1-


Necrotizing fasciitis in neonates is rare and is associated with almost 50% mortality. Although more than 80 cases of neonates (under one month of age) with necrotizing fasciitis have been reported in the literature, only six of them are identified as originating in the scrotum.We report the case of a four-week-old, full-term, otherwise-healthy Caucasian baby boy who presented with an ulcerating lesion of his scrotal wall. His scrotum was explored because of a provisional diagnosis of missed torsion of the testis. He was found to have necrotizing fasciitis of the scrotum. We were able to preserve the testis and excise the necrotic tissue, and with intravenous antibiotics there was a successful outcome.Fournier gangrene is rarely considered as part of the differential diagnosis in the clinical management of the acute scrotum. However, all doctors who care for small babies must be aware of this serious condition and, if it is suspected, should not hesitate in referring the babies to a specialist pediatric surgical center immediately. Testicular torsion is a surgical emergency and is commonly seen in children between five and 15 years of age. Neonatal torsion of the testis is a well-recognized condition in which an extravaginal torsion of the testis is known to occur before birth and invariably is associated with a necrotic testis [Necrotizing fasciitis in neonates is extremely rare and is associated with almost 50% mortality . AlthougA 10-day-old, full-term Caucasian baby boy presented with viral gastroenteritis to his local hospital. During admission, pediatric surgical services were consulted for left scrotal swelling and a diagnosis of a left hydrocele was made and patient was discharged. Seventeen days later (at four weeks of age), he presented with a 24-hour history of left scrotal swelling, erythema, pyrexia, and a pus-like discharge from an ulcerated area in his left hemi-scrotum. The results of an abdominal examination were normal, and a scrotal examination revealed a left scrotal abscess with a hard swollen left testis. No hernia was identified. A provisional diagnosis of missed neonatal testicular torsion was made and the baby was prepared for immediate surgical exploration. Intravenous antibiotics (amoxicillin with clavulanic acid and flucloxacillin) and fluids were commenced in light of the local scrotal infection.A midline raphe scrotal incision was used. A small amount of pus was evacuated, and a necrotic scrotal wall with an appearance similar to that of Fournier gangrene was excised back to the healthy tissue margin. The excised tissue was sent for histology and microbiology. The tunica vaginalis was thickened, and the testis was found to be edematous with normal blood supply. There was no evidence of testicular torsion. The wound was thoroughly washed with antiseptic solution. The dartos muscle and skin were closed primarily by means of absorbable sutures. The pyrexia was noted to settle soon after surgery. Edema, erythema, and pain settled over the following 48 hours.Enterococcus sp. (ampicillin-sensitive) and Staphylococcus aureus (methicillin-sensitive). The antibiotic regime mentioned above was continued for a total of 14 days . The baby was discharged home on the fifth post-operative day with oral antibiotics. Histology confirmed the intra-operative diagnosis (Figure Wound cultures revealed Fournier gangrene is more common in adults - peak incidence occurs between 20 and 50 years of age - and is extremely rare in children. Only around 80 cases of necrotizing fasciitis have been reported in the literature . FournieWhen the skin barrier is breached, the organisms appear to spread into the subcutaneous tissue and produce fascial necrosis with an obliterative endarteritis leading to further necrosis of tissue. The disease process, like that of our patient, is usually outside the tunica and hence the blood supply via the testicular artery is preserved. Although a left-sided hydrocele was diagnosed 17 days prior to this admission, no obvious hydrocele was noted at the time of surgery. Whether the initial swelling was due to a subacute phase of the disease process or truly was an isolated hydrocele that resolved spontaneously over this short period of time is not clear.Neonatal testicular torsion is a well-recognized condition. Patients present with unilateral, often painless scrotal swelling and erythema of overlying skin. The management of this condition is controversial. Some surgeons recommend immediate exploration whereas others prefer conservative management . ParatesS. aureus. Both Enterococcus and S. aureus have been implicated as the causative organisms of Fournier gangrene in previous reports [Although the initial literature advocates early aggressive surgical debridement of Fournier gangrene wounds, a recent report shows a successful outcome with a more conservative and selective surgical debridement . When sm reports ,11.Fournier gangrene of the scrotum is extremely rare in newborns but should be considered as part of the differential diagnosis in the clinical management of the acute scrotum. If it is considered a possibility, then immediate referral to a dedicated pediatric surgical unit is indicated.Written informed consent was obtained from the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.OZ gathered patient data, performed a literature search, calculated data from previous articles, and wrote the core of the manuscript. SP analyzed the patient's presentation in detail and made a major contribution to the manuscript, especially the Discussion section. MO'S performed a histological examination of specimens, photographed them, prepared the figure and legend, contributed in interpreting histological findings in view of the clinical presentation, and made corrections to the manuscript with special consideration to international histological nomenclature.FQ was the consultant in charge of this case, made the intra-operative diagnosis, directed the management, and finalized the manuscript, shaping the final conclusions. All authors read and approved the final manuscript.


Phytobezoars are concretions of poorly digested fruit and vegetable fibers found in the alimentary tract. Previous gastric resection, gastrojejunostomy, or pyloroplasty predispose people to bezoar formation. Small-bowel bezoars normally come from the stomach, and primary small-bowel bezoars are very rare. They are seen only in patients with underlying small-bowel diseases such as diverticula, strictures, or tumors. Primary small-bowel bezoars almost always present as intestinal obstructions, although it is a very rare cause, being responsible for less than 3% of all small-bowel obstructions in one series. Jejunal diverticula are rare, with an incidence of less than 0.5%. They are usually asymptomatic pseudodiverticula of pulsion type, and complications are reported in 10% to 30% of patients. A phytobezoar in a jejunal diverticulum is an extremely rare presentation.A 78-year-old Pakistani man presented to our clinic with small-bowel obstruction. Upon exploration, we found a primary small-bowel bezoar originating in a jejunal diverticulum and causing jejunal obstruction. Resection and anastomosis of the jejunal segment harboring the diverticulum was performed, and our patient had an uneventful recovery.Primary small-bowel bezoars are very rare but must be kept in mind as a possible cause of small-bowel obstruction. Phytobezoars are concretions of poorly digested fruit and vegetable fibers found in the alimentary tract . PreviouDifferent types of phytobezoars have been reported in the literature. The most common type is the diospyrobezoar, which occurs as a result of the ingestion of persimmons . PharmacSmall-bowel bezoars normally come from stomach, and primary small-bowel bezoars are very rare. They are seen only in patients with underlying small-bowel disease such as diverticula, strictures, or tumors . PrimaryJejunal diverticula are rare, with an incidence of less than 0.5% . They arA 78-year-old Pakistani man was brought to our clinic with a three-day history of epigastric and central abdominal pain associated with vomiting and abdominal distension. He had no history of any loss of weight or appetite. He had no significant medical or surgical history, except for recurrent, vague upper abdominal pain for the past few years that was treated as pain related to dyspepsia.Upon admission, his blood pressure was 130/90 mmHg, his pulse was 100 beats/minute, and he was afebrile. An abdominal examination revealed fullness in the epigastrium and a vague, mobile mass with mild tenderness was palpable in the right hypochondrium. His bowel sounds were sluggish, and rectal examination revealed an empty rectum with no palpable mass. His laboratory investigation results were unremarkable. A supine abdominal X-ray Figure  showed aOn the basis of the clinical and radiological findings, a diagnosis of small-bowel obstruction was made. After resuscitation of the patient, an exploratory laparotomy was performed with the interesting finding of a 7 cm ×5 cm jejunal diverticulum arising from the antimesenteric border of the jejunum about 25 cm distal from the duodenojejunal junction Figure  and 4. TPost-operatively, we noticed that the patient had near total loss of dentition. In response to our inquiry about his dietary habits, he said that he was a fast eater and did not chew food properly, the characteristic feature of an abdominal masticator. The patient had an uneventful recovery.Histopathology showed a 7 cm × 5 cm × 3 cm diverticulum arising from the antimesenteric border of the jejunum with marked inflammation and necrosis of the mucosa. This was a true diverticulum containing all the layers. There was no evidence of any malignancy or any ectopic gastric or pancreatic mucosa in the diverticulum. Upon his discharge from the hospital, he was counseled about dietary habits and proper mastication to avoid future problems.The origin of word "bezoar" derives either from the Arabic term "badzehr" or the Persian word "padzahr," both of which denote counterpoison or antidote. This word was applied to a greenish, hard concretion found in the fourth stomach of the Syrian goat. The stone was felt to prevent poisoning and came to Europe as the bezoar stone, which was highly prized for its medicinal properties ,13.In our patient, abnormal mastication habits as well as a jejunal diverticulum were the precipitating factors for phytobezoar formation that led to small-bowel obstruction. Although the commonest cause is previous gastric surgery, in our patient there was no such history. Phytobezoars usually occur as single entities, but multiple phytobezoars have been reported in the stomach in 17% of patients and in the intestine in 4% of patients ,14.et al. [et al. [Krausz et al.  reported [et al.  reportedCommon causes of small-bowel obstruction 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 has been observed . Primaryet al. [A number of diagnostic modalities have been used for the detection of abdominal bezoars. Rippolés et al.  reportedet al. , which iSmall-bowel bezoars are usually treated surgically. It is mandatory to explore the whole gastrointestinal tract to avoid synchronous bezoars and the recurrence of intestinal obstruction due to retained bezoars. Other described treatment options include enzymatic breakdown and endoscopic fragmentation for gastric bezoars ,20.In summary, we have described a case of an elderly man with a small-bowel obstruction due to a phytobezoar complicating a jejunal diverticulum. He had a combination of two rarities, that is, jejunal diverticulum and a phytobezoar as a cause of small-bowel obstruction.A search of the surgical literature revealed only one case of an obstructing phytobezoar arising from a proximal jejunal diverticulum .Primary small-bowel bezoars are very rare but must be kept in mind as a possible cause of small-bowel obstruction.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent (in Urdu) is available for review by the Editor-in-Chief of this journal.MT performed the surgery and wrote the main part of the manuscript. FK reviewed the manuscript and made valuable changes. FR performed the histology in this case and also wrote the pathology part of the manuscript. MK performed the histology and revised the manuscript.All authors read and approved the final manuscript.


Gastric cancer often carries a poor prognosis, with an estimated 740,000 deaths from the malignancy occurring yearly worldwide . The mortality of disease is largely dependent on the extent of tumor spread, as gastric cancer has a predilection to metastasize to other visceral secondaries via hematogenous and lymphatic dissemination. Direct invasion of a gastric adenocarcinoma to adjacent organs secondary to gastric wall perforation does occur; however, it is often present in the setting of advanced disease. Rarely does direct tumor invasion to adjacent organs from a gastric adenocarcinoma present as the initial manifestation of extranodal tumor spread. We present a case of a 40-year-old male with direct tumor extension to the liver as an initial presentation of extranodal tumor spread from a gastric adenocarcinoma. Clinicians should be aware of such an occurrence, as treatment modalities in direct liver extension from a gastric adenocarcinoma vary and may be directed towards palliation rather than curative intent. Gastric cancer is the second most common malignancy worldwide . AlthougA 40-year-old Polish male with no significant medical history presented to the emergency room with complaints of dysphagia and weight loss for nearly 3 months. The patient began noticing difficulty in swallowing liquids, eventually progressing to dysphagia for both liquids and solids over the last several weeks. He described the dysphagia as difficulty passing a food bolus through his lower esophagus and denied any difficulty initiating a swallow. The patient also noticed an unintentional weight loss of nearly 30 pounds over the past 3 months. He denied any nausea, vomiting, abdominal pain, melena, hematemesis, fevers, or chills. The patient denied the use of any medications, illicit drugs, alcohol, or tobacco. Family history was noncontributory, including the absence of any malignancy, gastrointestinal, or neurologic disorders.Physical examination was remarkable for pallor and mild midepigastric tenderness without guarding or rigidity. Digital rectal examination did not reveal any mass lesions or evidence of gross bleeding. Laboratory evaluation was significant for hemoglobin of 8.9 gm/dL with hematocrit of 27%. Liver transaminases were within normal range; however, there was an increased alkaline phosphatase noted to be 205 U/L. The patient was admitted to the medical ward where he underwent an esophagogastroduodenoscopy (EGD). EGD revealed a large, ulcerated, fungating gastric cardia mass with overlying exudates suspicious for neoplasm . SeveralGiven the size and extension of the gastric mass, the patient was deemed a poor surgical candidate. After an oncology evaluation, patient underwent chemoradiation therapy with paclitaxel and carboplatin. The patient was discharged and continued to follow with the oncology service for further chemoradiation therapy. The patient returned to the medical emergency room 2 months later after the abrupt onset of large volume hematemesis. Emergent EGD revealed the invading gastric mass was unchanged from previous endoscopic evaluation 2 months prior. Also observed was the presence of blood clots in the fundus, with no evidence of active bleeding. Repeat CT of the abdomen was suggestive of increasing tumor invasion into the liver parenchyma compared to previous imaging done prior to chemoradiation therapy. The patient refused further diagnostic and therapeutic measures. His hospitalization was complicated by repeated massive hematemesis and multiple organ failure leading to his eventual death on hospital day 6.Gastric cancer is the second most common cancer worldwide, with the incidence varying with geographical location . GastricThe most common sites of visceral secondaries from a primary gastric adenocarcinoma include the lung and liver with metastases to the brain and bony structures occurring less frequently . Tumor sThe classification of gastric adenocarcinoma has proven to be essential in the evaluation of prognoses in patients with this malignancy. According to the Lauren classification, gastric adenocarcinomas may be differentiated into two subgroups; intestinal type and diffuse type . GastricDirect tumor invasion to liver parenchyma from a gastric adenocarcinoma is infrequent and occurs in the setting of advanced disease , 3. In aSurgical interventions have been attempted in patients with gastric adenocarcinoma with direct liver invasion. In a study by Kunisaki et al., 10 patients with direct liver invasion from a gastric adenocarcinoma were evaluated with 3 patients undergoing curative combined resection of the gastric and liver tumor. Of these 3 patients, median survival time was only 13 months after the procedure with the median survival time without curative surgical intervention being 11 months . The theChemotherapeutic options are available to patients with gastric adenocarcinoma and direct invasion to adjacent organs; however, results are not promising. Randomized trials on combined chemotherapy agents, such as, cisplatin along with bleomycin or etoposide have demonstrated statistically significant improved survival rates over single chemotherapy agents , 9. ClinAlthough surgical intervention and chemotherapeutic options appear to have only a nominal benefit on median survival times, they often aid in palliation of symptoms. Noncurative surgical resection may alleviate symptoms of abdominal pain, dysphagia, nausea, and vomiting. Palliative chemotherapy can result in tumor shrinkage, control of neoplastic proliferation and altering tumor biology and metabolic activity; thus systemic and local symptom alleviation may be achieved , 12. GivPatients with a gastric adenocarcinoma and direct tumor extension to the liver have a dismal prognosis with 5-year survival rates thought to be less than 15 percent , 3. Alth


Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. The pathogenesis of conjunctival EMZL remains unknown. We describe an unusual case of EMZL arising from reactive lymphoid hyperplasia (RLH) of the conjunctiva. A 35-year-old woman had fleshy salmon-pink conjunctival tumors in both eyes, oculus uterque (OU). Specimens from conjunctival tumors in the right eye, oculus dexter (OD), revealed a collection of small lymphoid cells in the stroma. Immunohistochemically, immunoglobulin (Ig) light chain restriction was not detected. In contrast, diffuse atypical lymphoid cell infiltration was noted in the left eye, oculus sinister (OS), and positive for CD20, a marker for B cells OS. The tumors were histologically diagnosed as RLH OD, and EMZL OS. PCR analysis detected IgH gene rearrangement in the joining region (JH) region OU. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD, arising from RLH. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy. Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. EMZL is common among malignant conjunctival tumors and has a quite indolent course, but relapses can be seen. The patA 35-year-old woman had suffered from minor irritation in both eyes, oculus uterque (OU), and was treated with topical eye drops based on a diagnosis of allergic conjunctivitis at an initial clinic. Since her conjunctival lesions did not improve, she was referred to our hospital on 17 December 2010. Her visual acuity was 20/20 OU with a normal intraocular pressure. Slit-lamp examination demonstrated fleshy salmon-pink tumors in the upper and lower conjunctival fornix that extended into the inferior palpebral conjunctiva  kappa and IgG lambda antibodies , at room temperature for two hours. Positive signals were visualized using diaminobendizine as a substrate.Histological examination demonstrated a collection of small lymphoid cells in the stroma FigureC OD. Immin situ.In this case, the diagnosis of EMZL was made OS, based on typical histological findings and B-cell monoclonality with IgH gene rearrangement. In contrast, histological findings revealed RLH OD, although IgH gene rearrangement was detected using the PCR method. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD. Previous studies reported that RLH of the orbit and eyelid developed systemic lymphomas in the selected cases,3; howevWhile EMZL had been thought to be induced by infection and/or chronic inflammation, the pathogens have yet to be determined. EMZL may arise at sites of chronic antigenic stimulation due to autoimmunity  but theConjunctival lymphatic tissue develops after antigen stimulation. ChronicThe results in this case suggest that conjunctival lymphoma may arise from reactive lymphoid hyperplasia. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy.EMZL: extra nodal marginal zone B-cell lymphoma; H & E: haematoxylin and eosin; Ig: immunoglobulin; OD: oculus dexter (right eye); OS: oculus sinister (left eye); OU: oculus uterque (both eyes); PCR: polymerase chain reaction; RLH: reactive lymphoid hyperplasia.The authors have no proprietary interests in any aspect of this study.JF, SK and MN proposed the study. JF and SK obtained images, wrote the manuscript provided and reviewed the pathological images. SI, KI and TY conducted the literature search. All authors read and approved the final manuscript.


Schwannoma (neurilemmoma) is a benign peripheral nerve sheath tumor that occurs in a wide variety of locations; however, its finding in the uterine cervix is extremely rare. We report a case of an incidental primary benign cervical schwannoma in a 48-year-old woman. In the English literature, a few cases of primary schwannoma of the cervix have been reported, which include seven cases of primary malignant cervical schwannoma and only two that are benign. These cases are reviewed in the following discussion. Cervical schwannoma is a very rare entity in the field of gynecologic pathology. In this paper, we describe an incidental benign cervical schwannoma in a 48-year-old woman. A 48-year-old woman presented to the emergency department with a sharp, severe left lower quadrant pain. Ultrasound imaging of the abdomen and pelvis demonstrated an enlarged uterus with multiple masses (highly suggestive of leiomyoma), an 8.7 cm left adnexal complex mass, and minimal fluid in the pelvis. Subsequent computed tomography (CT) scan of the abdomen and pelvis confirmed the above findings.Pelvic examination revealed a posterior cervix with an enlarged, boggy uterus, but no tenderness. The cervix could not be visualized on speculum exam due to a distorted uterus. No palpable inguinal lymph nodes were identified. The remainder of the physical exam was unremarkable. Laboratory workup was notable for an elevated CA-125 level (125.9 U/mL).The patient's past medical history was significant for breast cancer status after lumpectomy, radiation therapy, and Tamoxifen therapy. Her past gynecologic history was remarkable for laparoscopic myomectomy (12 years prior to this encounter). She resumed having regular cycles every 30 days and denied any recent abnormal bleeding, weight loss, and gastrointestinal or genitourinary symptoms.The patient was advised that the best approach would be to remove the uterus and ovaries and send them for intraoperative consultation during the surgery. Subsequently, the patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH/BSO).Exploration of the pelvis at the time of surgery revealed a large multilobated uterus that was about 16 weeks in size, as well as hemorrhagic cysts on both ovaries. The left ovary was no longer 8 to 9 cm, but was instead around 4 to 5 cm. Additionally, there was also some blood in the pelvis. Pelvic washings were obtained, and a representative section from the left ovary was submitted for frozen section examination. The diagnosis was reported as a benign hemorrhagic cyst. The surgery and the postoperative course proceeded without complication and the patient was discharged home on postoperative day 4.On gross examination the left ovary was remarkable for a 2.0 × 1.8 × 1.4 cm hemorrhagic cyst containing necrotic material. The right ovary was remarkable for a 2.5 × 2.0 × 1.5 cm hemorrhagic cyst. Both fallopian tubes were unremarkable.The enlarged and distorted uterus (628.9 gram and 17.8 × 11.0 × 10.5 cm) had multiple subserosal, intramural, and submucosal leiomyomas ranging from 0.1 to 4.0 cm in greatest dimension. Also, within the uterine fundus attached to the anterior and posterior walls were multiple tan-pink focally hemorrhagic fleshy polyps ranging from 5.0 to 2.7 cm in greatest dimension, which all appeared to be confined to the mucosa.Gross examination of the cervix revealed a 0.9 × 0.4 × 0.3 cm rubbery nodule on the posterior endocervical canal which had a homogenous gray-white cut surface. There was also a 1.3 × 0.4 × 0.4 cm tan-white soft polyp arising from the anterior endocervical canal. The remainder of the cervix was unremarkable.Histologically, the white rubbery nodule of the endocervical canal was a well circumscribed lesion composed of a spindle cell proliferation with localized nuclear palisading and abundant thick-wall hyalinized blood vessels Figures . MitoticAdditional microscopic findings were ovarian endometriosis, hemorrhagic corpus luteum, uterine leiomyomas and adenomyosis, endometrial polyps, and a benign endocervical polyp.Schwannoma (neurilemmoma) is a benign peripheral nerve sheath tumor that occurs in a wide variety of locations. Most commonly they occur in middle age, and without a clear sex predilection. They usually present in superficial soft tissues of the extremities, perhaps relatively most often in the head and neck region and the distal parts of the extremities. Certain schwannomas, especially the bilateral vestibular ones, are associated with neurofibromatosis type 2 (NF2) syndrome and hereditary NF2 gene mutations. Uncommonly, large tumors are found in the posterior mediastinum or the retroperitoneum. Typical schwannomas can also involve visceral sites primarily, such as the GI tract, kidney, and breast; however, its finding in the uterine cervix is extremely rare. In the English literature there have been less than ten reports of a primary schwannoma of the uterine cervix listed in Gwavava and Traub reported the first case in 1980 [The clinical presentation in the second reported case of benign schwannoma by LeMaire et al. in 2002 [The first case of pigmented melanocytic schwannoma of the uterine cervix was reported in 1990 by Terzakis et al. . It was The largest series of the malignant schwannoma or malignant peripheral nerve sheath tumor (MPNST) was reported by Keel et al. in 1998 . In thisPrior and after the Keel's report, five more cases of the malignant schwannoma of the cervix were reported (the first one in 1988 and the last one in 2005) that all of them were presented with the similar clinical presentation of abnormal vaginal bleeding. The patients were 47, 41, 51, 65, and 27 years old. The tumors were ranging from 1 to 4.4 cm in dimension, and histologically all of them were composed of mitotically active hypercellular schwannoma with nuclear atypia [Lesions that should be considered in the differential diagnosis in this case are neurofibroma, leiomyoma, angiomyofibroblastoma, and desmoplastic melanoma. The absence of staining for CD34 and NFP, but strong positivity for S-100, supports the diagnosis of schwannoma as opposed to neurofibroma. Moreover, the presence of thick-wall hyalinized blood vessels favors the diagnosis of schwannoma. Positivity for S-100 protein and negativity for desmin and actin argue against leiomyoma and angiomyofibroblastoma. Finally, a negative pan-melanoma stain essentially excludes desmoplastic melanoma.


Bladder herniation within the inguinal canal is a relatively uncommon finding. We report an even less-common occurrence of transitional cell carcinoma located within a portion of inguinally herniated bladder. Fewer than 20 reports exist in the literature describing this scenario. Bladder herniation into the inguinal canal is uncommon. Previous reports have shown bladder to be present in 0.36–4% of inguinal hernias , 2 thoug The presence of bladder carcinoma within a herniated portion of bladder is exceedingly rare, with less than 20 reports in the literature over the past 50 years –8. Treat A 90-year-old man was seen in consultation for complaints of acute onset of gross hematuria. The patient reported that for roughly one month he had intermittent “tomato juice” colored urine. He had no previous reports of urologic malignancy or hematuria and denied any weight loss, lower urinary tract symptoms, or irritative voiding symptoms. Upon consultation, urinalysis was positive for blood, and a review of urine labs performed in the previous three months demonstrated microscopic hematuria. Physical examination revealed bilateral inguinal hernias, the right larger than the left, but neither causing symptoms. All pertinent biochemical labs were within normal limits.CT urogram revealed a number of bladder masses suspicious for malignancy. A large right-sided bladder herniation extended down into the right inguinal canal and scrotal sac, consistent with physical exam findings. Due to patient positioning, this herniated portion did not contain any contrast on delayed phases of CT; however, wall thickening was observed and a mass was noted in the neck portion of the diverticulum that extended through the inguinal canal Figures , and 3.  The presence of carcinoma within a herniated portion of bladder can present unique therapeutic challenges. Given that most bladder hernias are asymptomatic, the majority are discovered at the time of surgery . If susp Ideally, including the current case, herniations are reducible at the time of cystoscopy, providing means for adequate transurethral resection of tumor and future treatments. Care must be taken to avoid perforation, especially if a bladder diverticulum is suspected on radiological imaging.  Few reports have discussed the management of such cases though a small number of them have described bladder sparing approaches with the removal of the TCC—containing portion of bladder , 10–12.  Bladder herniation and scrotal cystocele are rare entities. Cancer contained within these findings is even more uncommon and treatment presents a unique challenge. Here we describe a patient who presented with gross hematuria and was found to have transitional cell carcinoma contained within an inguinally herniated portion of bladder. Pathologic diagnosis is crucial in determining the proper course of treatment and reduction of the herniation may provide visualization for adequate transurethral resection and future intravesical treatments.