Dermoid cysts of the head and neck are rare lesions. An unusual dermoid cyst in the subcutaneous tissue of the mastoid region is easily confused with lymph node abnormalities; therefore, diagnosis and treatment are often challenging. We describe a 22-year-old male with an unusual dermoid cyst in the subcutaneous tissue of the mastoid region whose only complaint was a retroauricular lump, which was similar to an intumescent retroauricular lymph node. Final diagnosis was confirmed by pathological examination of the surgically removed specimen. A dermoid cyst in the subcutaneous tissue of the mastoid region is rare and may be misdiagnosed as a retroauricular lymph node. A lump may be the only presenting symptom without hearing loss and vertigo complaints. Complete excision of the tumor must be ensured and pathological analysis is important for the diagnosis. A dermoid cyst is a rare benign tumor, which is principally situated close to the midline of the body. It is a developmental lesion, histologically composed of ectoderm and mesoderm; however, it has no endoderm. In approximately 7% of cases, these cysts affect the head and neck region; they are frequently encountered in the area of the lateral eyebrow, the orbit and the nose (>80%), with the remainder located in the neck, occipital or frontal midline, lip or palate . HoweverThe patient was a 22-year-old male presenting a lump that had grown gradually under the skin of the right mastoid for 2 years. The patient denied any hearing loss or vertigo. There was no pain and no inflammation in the lump. The patient was healthy, with the exception of the lump. The patient had been assessed at the community hospital one year earlier and was treated with antibiotics, which were ineffective. The patient then came to Xijing Hospital  for further evaluation and treatment.On examination, the two ears appeared normal. The patient had normal and symmetrical facial and cervical structures with no deformities of the pinnae or external auditory canals. The ear drums were normal and no air-fluid levels or bubbles were observed behind the drum. Impedance audiometry and audiogram were also normal. There were no significant findings in the remainder of the head and neck examination, with the exception of the tumor, which was initially considered a retroauricular lymph node.−2 m (Surgical biopsy was performed with the patient under local anesthesia. When the skin flap was opened, a pink encapsulated mass was observed occupying the right mastoid surface. The mass extended from the subcutaneous tissue to the mastoid cortical plate without destruction of the mastoid bony wall. The tumor and mastoid cortical plate were umbilicated in the mastoid cavity. The mastoid cavity maintained integrity. The surface of the mass was granulated and a number of hairs were observed in the tumor when it was slivered. The diameter of the tumor was approximately 1.3×10−2 m . FollowiPathological analysis confirmed the diagnosis that the tumor was a dermoid cyst . Two yeaDermoid cysts are tumors composed of two germ layers, ectoderm and mesoderm. They are epithelial-lined cavities with skin appendages, including hair, hair follicles and sebaceous glands. This distinguishes them from cholesteatomas, which are composed only of ectodermal elements, and teratomas, which are composed of ectodermal, mesodermal and endodermal elements.et al reported on a 6-year-old female patient with a dermoid cyst in the middle ear and low-tone unilateral conductive hearing loss (et al reported on an 8-month-old female who was the youngest patient with a congenital dermoid cyst of the middle ear with a moderate conductive hearing loss of the ear in 1998. It was suggested that congenital inclusion may be the cause of the majority of dermoid cysts in the head and neck (et al reported a case of a 1-year-old female who was initially seen with a cutaneous fistula of the frontotemporal region, which revealed an intracranial dermoid cyst (Dermoid cysts may occur anywhere in the body. They primarily occur in the gonads; however, they also occur at extragonadal sites along the midline of the body. The head and neck region is a rare location for such tumors in children and adults. Therefore, the pathological evaluation and clinical management of these tumors is extremely difficult. Toynbee reported the first case of a dermoid cyst of the mastoid in 1866, when hairs were identified in the mastoid cavity surrounded by epidermis . Howie dand neck . ScolozzThe patient in our study was unique in several respects and there are no previous reports of similar cases. The patient had no symptoms, with the exception of the histologically-confirmed dermoid cyst in the subcutaneous tissue of the mastoid region. Patients previously reported often had a dermoid cyst in the mastoid and middle ear and usually suffered from hearing loss and vertigo. However, in this case, the dermoid cyst extended from the subcutaneous tissue to the right mastoid, the mastoid cavity was undamaged and hearing loss and vertigo did not appear. Therefore, prior to pathological analysis, it was easily confused with an intumescent lymph node.As with the majority of cysts, local recurrence at the primary site is common unless the entire wall of the cyst is removed. Therefore, complete excision of the cyst during surgery must be ensured.Although uncommon, dermoid cysts must be considered in the differential diagnosis of lumps in the mastoid region. However, their pre-operative differentiation from a retroauricular lymph node is not easy. Complete resection of the tumor is required and pathological analysis is important for the diagnosis.


Introduction. Giant cell tumor is a benign or locally aggressive tumor of uncertain origin that appears in mature bone, most commonly in the distal femur, proximal tibia which characteristically extends right up to the subarticular bone plate. Case Report. We report here a 35-year-old female presenting with swelling of the left knee. On examination, the swelling was solitary, about 20 × 15 cm in size with the skin over the swelling stretched and glistening. On the fifth day of hospital stay, the swelling burst open and blood tinged pus started pouring out. X-ray and MRI scan showed a well-defined T2 hyperintense expansile eccentrically located osteolytic lesion involving the metaphyseal region of the proximal tibia and extending into the subarticular space and multiple T1/T2 hypointense septations are noted within the lesion suggestive of osteoclastoma. The patient was counseled regarding the tumor and prognosis and various treatment options. She was treated successfully with above knee amputation. The tissue was sent for histopathology which confirmed osteoclastoma. Conclusion. It is important to recognize giant cell tumors early, so that they can be treated promptly with local measures to prevent morbidity and mortality in young adults. Giant cell tumor is so named because it contains profusion of multinucleated osteoclast-type giant cells, giving rise to the synonym osteoclastoma. The term osteoclastoma was first used in Great Britain by Stewart in 1922. It is a benign but locally aggressive neoplasm representing approximately 5% of all primary bone tumors. It usually arises in patients in their twenties to forties. The tumor is slightly more common in female. They are typically epiphyseometaphyseal tumors, with the majority involving the distal femur and proximal tibia. The clinical history of a giant cell tumor usually includes pain and limitation of motion because of the tumor's proximity to the joint space. Swelling occurs late in the course of the giant cell tumor. Patients presenting to the doctor early in the course of the tumor can be treated with local surgery such as thorough curettage and cryosurgery or burring of the cavity with or without phenol installation. The lesional area may then be packed with allograft or polymethyl methacrylate. Such treatment reduces the local recurrence rate to less than 20% and the prognosis is excellent if it does not recur locally [If this tumor is not diagnosed and treated early, it continues to grow in size causing massive local tissue destruction and may get secondarily infected thereby requiring amputation.A 35-year-old female patient presented to our opd with swelling of the left knee for 6 years. Initially, the swelling was small, which gradually increased in size, covering the entire anterior aspect of the knee.Staphylococcus aureus which was sensitive to piperacillin and tazobactam which was started immediately. All routine blood investigations were carried out. The patient was noted to have severe anemia and her random blood sugar level was 385 mg%. Her serum calcium and alkaline phosphatase levels were within normal range. X-ray and MRI scan of left knee showed a well-defined T2 hyperintense expansile eccentrically located osteolytic lesion involving the metaphyseal region of the proximal tibia and extending into the subarticular space and multiple T1/T2 hypointense septations were noted within the lesion suggestive of osteoclastoma (Also, she complained of pain and loss of movements of the knee with inability to walk. There was no history of discharge from the lesion, fever, weight loss, or other joint pains. General physical examination was normal except for pallor. Systemic examination was also normal. On local examination, the swelling was solitary, about 20 × 15 cm in size with the skin over the swelling stretched and glistening. On the fifth day of hospital stay, the swelling burst open and blood tinged pus started pouring out. The pus was sent for culture which showed clastoma . Her hemclastoma . The patclastoma . Sample Distance of ambulation was progressed to patient's tolerance. Exercises were encouraged when the patient was on bed or sitting in chair. Dressings were done on the 3rd, 6th, and 9th postoperative days. Pain was controlled with adequate intravenous analgesics. Intravenous antibiotics were given for 4 days after which oral antibiotics were continued till suture removal which was done on the 12th postoperative day. There was no discharge or gaping at the stump site. Patient was then discharged on the 14th postoperative day after giving her oral hypoglycemic drugs. The patient returned for followup after 1 month and had no residual symptoms with completely healed stump . AmbulatGiant cell tumors are benign but locally aggressive tumors and, at times, can be malignant. They can be well treated with local surgical measures if diagnosed early. Radiation therapy has been used for the treatment of giant cell tumor of bone since 1906 , 3. RadiIn this case, the patient presented late and thus the lesion had progressed beyond a point of conservative management. Limb salvage techniques like curettage cannot be used once there is vascular invasion and surrounding soft tissue destruction or there is superadded infection. Thus we conclude the importance of early diagnosis and management of giant cell tumors so that the limb can be saved.


Gastrointestinal stromal tumors of the duodenum are uncommon. They can reach a large size. Diagnosis can be elusive and managing them can be difficult. Our case report aims to increase awareness and highlight some issues related to the diagnosis and management of duodenal gastrointestinal stromal tumors.We present the case of a 38-year-old Middle Eastern woman with a large, slowly-growing gastrointestinal stromal tumor of the duodenum. Her complaints were minor epigastric discomfort and swelling. A pancreaticoduodenectomy with complete tumor excision was performed. She was doing very well with no evidence of disease recurrence when she was last seen 34 months after her operation.Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis. There is more than one surgical approach available; however, complete surgical excision, with negative margins, is the absolute requirement. Preoperative imatinib mesylate can be considered in unresectable or borderline resectable cases. The most common sites for gastrointestinal stromal tumors (GIST) are the stomach and, to a lesser extent, the small intestine . Small iWe describe the case of a large duodenal GIST including its presentation, diagnosis, and the type of surgery performed, as well as a review of issues related to GIST in the duodenum.A 38-year-old Middle Eastern woman presented with a slowly enlarging abdominal mass of 12 years duration. According to the patient, a surgeon had attempted to resect the mass 12 years earlier, but could not do so due to excessive bleeding from the tumor. She was offered no further treatment.At presentation, her main complaint was epigastric discomfort. She also gave a history of some mild back pain and occasional abdominal pain. Her appetite was good and she had not lost weight. There was no history of vomiting, change in bowel habits or melena. She had been diagnosed with a peptic ulcer many years ago.On examination she looked healthy with no clinical jaundice or pallor. Abdominal examination revealed a large upper abdominal mass with thinned overlying skin. It had minimal mobility and was not tender. The rest of the examination was normal. Her hemoglobin level was 10.8 g/dL, with hypochromic microcytic red blood cell indices. Otherwise, all blood tests were normal. A computed tomography (CT) scan of the abdomen revealed a 20 cm retroperitoneal mass in the region of the head of the pancreas  was performed with the dissection kept outside the pseudocapsule of the tumor, taking care not to rupture the tumor. The patient tolerated the procedure well and had an uneventful recovery. Histopathological examination revealed a 22 cm tumor arising from the second part of the duodenum. The tumor showed moderate cellularity and mildly atypical spindle cells arranged in fascicles with a low mitotic count (1/50 high power field) and no necrosis (Figure GISTs are the most common mesenchymal tumors of the gastrointestinal tract . They arDiagnosis can be made with upper gastrointestinal endoscopy . The tumThe treatment of choice for duodenal GIST is complete surgical excision. This can be performed by local or segmental duodenal resection with preservation of the pancreas for small tumors . As for The outcome depends on the pathological features of the tumor and the completeness of surgical resection. Large tumors with high mitotic counts behave much worse than small tumors with low mitotic counts, which are considered benign . Local rDuodenal GIST should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of this diagnosis. There is more than one surgical approach available, but the absolute requirement is complete surgical excision. Preoperative IM can be considered in unresectable or borderline resectable cases.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BM performed the literature review, collected the photos and wrote the article. FA collected some papers for review and provided input for the article. All authors read and approved the final manuscript.


Synovial osteochondromatosis of the distal radioulnar joint is a rare entity with only 14 cases reported in the literature. Malignant transformation of synovial osteochondromatosis is the most worrisome complication of the disease. It has been described in joints such as the hip and knee but never for the distal radioulnar joint. We report a case of synovial osteochondromatosis of the distal radioulnar joint which presented with radiographic features which were worrisome for malignant transformation and required a comprehensive preoperative workup. Discussed are the preoperative management, surgical treatment, and a literature review of this rare disease. Synovial osteochondromatosis of the wrist is a rare, progressive disorder characterized by benign hyperplastic metaplasia of the subsynovial cells resulting in cartilaginous nodules which often shed loose bodies into the synovial cavity . Common There have been fewer than 15 previous reports of synovial osteochondromatosis of the distal radioulnar joint . Herein,A 28-year-old, right hand dominant female presented with a 3-year history of an enlarging mass in her left distal forearm which had become increasingly painful. Her wrist was initially sore only while typing but later also became painful at rest. The patient did not experience any systemic symptoms such as weight loss or night sweats and had no history of sarcoma or other tumors. She was otherwise healthy with a benign medical history, no previous surgeries, and no prior injuries to her left hand or wrist.On physical examination, she had a palpable mass, approximately 3 cm × 3 cm in diameter, located over the dorsal and volar aspects of the left distal wrist. Flexor and extensor tendons appeared to be superficial to the mass, and she had full range of motion of the wrist with the exception of a loss of 20 degrees of pronation. Neurovascular status of the left hand was normal. Radiographs showed a mass with dystrophic, amorphous calcifications centered in the interosseous membrane, just proximal to the distal radioulnar joint . There wDue to concern of a potential malignant process, a CT guided biopsy of the mass and a CT chest were ordered to help rule out sarcoma. Histopathology of the biopsy showed fragments of a well-differentiated hyaline cartilage with focal calcifications, and a preliminary diagnosis of synovial osteochondromatosis was made.Surgical indication for excisional biopsy was made as the patient described pain, loss of motion, and concern about the mass. Both volar and dorsal incisions of the forearm were required to excise the mass as it was located on both sides on the interosseous membrane in a dumbbell-shaped configuration . The mosConsistent with the initial biopsy, histological analysis of the excised specimen showed that the majority of the tumor was encapsulated and demonstrated hyaline cartilage with focal calcifications. A final diagnosis of synovial osteochondromatosis was made.The patient has done well postoperatively, with no evidence of recurrence and a return of full range of motion of her left wrist 2 years postoperatively. Synovial osteochondromatosis is a benign condition of unknown etiology, whereby a metaplasia of the synovial membrane results in an accumulation of intra-articular cartilaginous nodules. Common clinical features include mild pain, swelling, and stiffness of the affected joint. Locking and clicking due to loose bodies have also been reported . It has The differential diagnosis for synovial osteochondromatosis in the wrist is broad and includes benign and malignant tumors. The latter includes synovial chondrosarcoma and malignant fibrous histiocytoma, and the former includes synovial osteochondromatosis, rheumatoid or osteoarthritis, and chondrocalcinosis . A tissuClinicians must rely primarily on clinical exam and imaging when diagnosing synovial osteochondromatosis preoperatively. While the radiographic features of this condition are variable depending on the maturity of the tumor, abnormalities can be appreciated in most cases. Initially, the tumor may present with an effusion and noncalcified loose bodies, which may not be visible on radiographs but can usually be visualized on CT or MRI . As the The current standard for the treatment of synovial osteochondromatosis involves the removal of any nodules and loose bodies from the synovial cavity and excision of diseased synovium , 10. Pos


In rare condition, combined thymic epithelial tumors showing either type A or type B thymomas areas combined with thymic carcinoma components may occur in thymus. Mucoepidermoid carcinoma (MEC) of the thymus is rare in thymic carcinoma, and so far there is no report to describe a combined epithelial tumor of thymus with MEC component. We report an unusual case of combined thymic MEC/type B2 thymoma in a middle-aged male occurring in a mass of anterior mediastinum. Case report: A 51-year-old Chinese male patient presented with a 6-month history of right ptosis and progressive muscle weakness. Computed tomography (CT) examination revealed a solitary, well-circumscribed mass was in the anterior mediastinum with mild heterogeneous enhancement. Histologically, the mass contained two separated components and displayed typically histological features of low-grade MEC and type B2 thymoma, respectively. There was no gradual transition of these two components observed in mass, and no enlarged lymph node was found in the surrounding tissues. A diagnosis of combined thymic MEC/type B2 thymoma was made. The patient received thymectomy to resect the mass totally. After surgery, chemotherapy with regiments of cisplatin and mitomycin, and radiotherapy of the main tumor bed were performed on the patient. There was no evidence of tumor recurrence during the period of 12 months follow-up.To our best knowledge, this is the first report of combined thymic epithelial tumor with MEC component. Although this tumor is rare, the diagnosis of a thymic MEC should be taken into consideration when a combined epithelial tumor is occasionally encountered in thymus.http://www.diagnosticpathology.diagnomx.eu/vs/9721397571157894The virtual slide(s) for this article can be found here:  Primary thymic carcinoma is a rare tumor of the anterior mediastinum. Mucoepidermoid carcinoma (MEC) of the thymus is rare in thymic caircinoma, and comprises approximately 2% of published thymic carcinoma ,2. Up toA 51-year-old Chinese male presented with a 6-month history of right ptosis and progressive muscle weakness. The patient had been diagnosed as myasthenia gravis at local hospital 6 months before, but for unknown reasons, he failed to receive workup and management at that time. Before one day the patient was admitted to our hospital, he suffered with a sudden onset of dysarthria. Therefore, the patient referred to our hospital for further examination and treatment. Physical examination showed right-sided partial ptosis. Diplopia was noticed on right lateral gaze due to right lateral rectus weakness. The patient did not have dysphagia or dyspnoea. But he was having generalized weakness in all his extremities, both proximal and distal, with marked diurnal variation in the form of more weakness during the evening. A confirmatory electrophysiological study with repetitive nerve stimulation showed decrement in amplitude of action potentials with further reduction post exercise and recovery after 15 minutes. Acetylcholine receptor (AChR) binding antibodies were markedly elevated. However, other routine laboratory test, including blood count, differential, liver and renal function, were within the normal range. A chest computed tomography (CT) scan demonstrated a solid mass, measuring 5.4 × 3.7 × 2.6 cm, with focal heterogeneously enhancement, in the right anterior mediastinum. The tumor was observed to adhere to the wall of aorta but did not infiltrate it. Minimal amount of right pleural effusion and pericardial effusion were associated, but neither enlarged lymph node nor remarkable image finding was noted in the lung parenchyma /CT study to search for the potentially secondary tumor, but no abnormality was found. The patient was on regular follow-up for 12 months after discharging from hospital. He had remained asymptomatic, and there was no evidence of tumor recurrence during the period of postoperative follow-up.Combined thymic epithelial tumors are rare and characterized by at least two distinct areas each corresponding to one of the histological thymoma and thymic carcinoma types . The etiMEC is a relatively common neoplasm of the salivary glands, which rarely arises in other sites, including esophagus, anal canal, skin of the breast, lachrymal sac, thyroid gland, or uterine cervix -23. PrimThe establishment of a preoperative diagnosis of thymic MEC is difficult because of the rarity of this tumor and the fact that there are no specific landmarks in the radiologic examinations. Therefore, percutaneous biopsy is needed for this tumor to obtain a definite diagnosis preoperatively . In the Histologically, MEC in thymus and other anatomical sites are characterized by squamoid (epidermoid), mucin-producing and cells of intermediate type with varying proportion and architectural configuration in and between tumors . The preAvailable data on combined thymic epithelial tumors suggest that the most aggressive component of tumor determines the clinical outcome. As to thymic MEC, high-grade tumors have been demonstrated more invasive and shown stronger tendency for metastasis compared with low-grade tumors ,19. AmonIn summary, we herein first reported a rare case of combined thymic MEC/thymoma in an anterior mediastinal mass. The mass contained two separated components and displayed typically histological features of low-grade MEC and type B2 thymoma, respectively. The precise mechanism of coexistence of MEC and thymoma in the same nodule remains unknown, and longer period of follow-up and more case investigation are necessary to better clarify the biological characteristics and clinical outcomes of this unusual tumor. Although this tumor is rare, the diagnosis of a thymic MEC should be taken into consideration when a combined epithelial tumor is occasionally encountered in thymus.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.SGW and YL made contributions to acquisition of clinical data, and analysis of the histological features by H & E staining and immunoassays. They are joint first co-authors and made an equal contribution to this work. BL carries on the immunohistochemical and special staining. XYT drafted the manuscript. ZL revised manuscript critically for important intellectual content and had given final approval of the version to be published. All authors read and approved the final manuscript.


Introduction. Leiomyoma uteri is one of the most common benign conditions for which women undergo hysterectomy every year. Fibroids found retroperitoneally are a rare entity, especially, primary retroperitoneal fibroid. Case Presentation. We report a case of 42-year-old para 1 who presented to our hospital with recurring retention of urine, lower abdominal and pelvic pain, and dyspareunia . Provisional diagnosis on the basis of examination and imaging was large subserosal fibroid with mild right-sided hydroureteronephrosis, due to pressure effect of the fibroid. Abdominal hysterectomy was done for the patient, and intraoperatively, a bulky uterus was found with multiple small fibroids on anterior and posterior walls, and a large fibroid approx. 10 × 8 cm was found arising from the posterior surface at the level of internal os retroperitoneally, which was confirmed by histopathology as leiomyoma. Conclusion. Retroperitoneal fibroids are rare neoplasms and treatment is surgical removal. Preoperative imaging can only give provisional diagnosis and can be misguiding. Final diagnosis of retroperitoneal fibroid can be made only intraoperatively. Leiomyomata is a common benign conditions arising from smooth muscle cells. Approximately 20%–30% of women older than 35 years have uterine leiomyomata that are manifested clinically , 2. RetrA 42-year-old female patient, presented to our gynaecology outpatient department with complaints of lower abdominal pelvic pain and backache, dyspareunia, and difficulty in passing urine for the last 6 months. She had suprapubic pain with constant backache and heaviness in the perineum. Patient took medication from local practitioners for symptomatic relief. She presented to us when already catheterised with self-retaining Foley's catheter from outside for retention of urine for one day. There was history of similar episodes of retention of urine 4-5 times in the last 6 months for which she had to be catheterised once and again for about 5–7 days each time. The catheter was removed after toning of bladder.Patient was para one with one live issue 20 years old conceived spontaneously and delivered by normal vaginal delivery and had three spontaneous first trimester abortions, thereafter. Menarche was attained at the age of 13 years. Patient had irregular menstrual cycles occurring at every 15–20 days with bleeding lasting for 4-5 days; the amount of blood flow was normal. There was no previous illness or surgical intervention. Patient was a vegetarian, nonsmoker, nonalcoholic with normal bowel habits. She was moderately built and nourished. General condition of the patient was stable with mild pallor. Vitals were normal. Abdominal examination revealed a firm mass of 16-week size arising from the pelvis deviated more towards the left side. The mass had smooth surface with restricted mobility. On pelvic examination, cervix was hitched below the pubic symphysis, bleeding was present through the cervical os. Foleys catheter was in situ. Uterus was acutely retroverted; a mass of about 16-week size, irregular, firm in consistency was felt through posterior and the left lateral fornix. The uterus could not be differentiated from the mass. Base line investigations and renal function tests of the patient were normal. Ultrasonography revealed a large uterine fibroid of size 14 × 9 × 8 cm, which was subserous with normal bilateral adnexae. Right-sided hydroureteronephrosis was also reported for which IVP was done showing right-sided ureteric obliteration with bladder neck compression. Considering the above clinical findings and investigations, provisional diagnosis of subserous fibroid was made and abdominal hysterectomy was planned. A vertical midline infraumbilical incision was employed. Intraoperatively, uterus was found to be just bulky. A large retroperitoneal firm mass of size 10 cm × 8 cm was found arising from the posterior surface at the level of internal os . Total aThe postoperative period of the patient was uneventful. The stitch removal was done on the seventh postoperative day. The stitch line was healthy with good healing. Histopathological examination confirmed the smooth muscle cell origin of the retroperitoneal mass consistent with diagnosis of retroperitoneal leiomyoma .Uterine fibroids are the most common benign solid pelvic tumours in women  and are Retroperitoneal leiomyomata is a rare neoplasm and thorough imaging, though, helpful in operative planning may also miss the exact site of these fibroids. Final diagnosis is made intraoperatively on direct visualisation. Complete excision with or without abdominal hysterectomy is the treatment of retroperitoneal fibroids.


Non-traumatic hemoperitoneum may be catastrophic if it is not promptly diagnosed and treated. It is critical to identify this clinical picture and treat any active bleeding. We report the first case in the literature (to our knowledge) of spontaneous hemoperitoneum caused by a cystic splenic hemangiopericytoma. Hemangiopericytomas represent a small subset of soft tissue sarcomas. They rarely originate in the spleen as a primary tumor, with only ten cases having been previously described. The difficulty of predicting the prognosis and clinical behavior of these lesions has been repeatedly stressed. The literature concerning this rare and unusual neoplasm is reviewed. Non-traumatic hemoperitoneum may occur spontaneously or may be iatrogenic. This uncommon and often unsuspected condition may be catastrophic if it is not promptly diagnosed and treated. The possible causes include hemorrhage from a highly vascular neoplasm, pathological splenic rupture, hemorrhage or rupture of an ovarian cyst, rupture of the gestational sac or other affected anatomic part in an ectopic pregnancy, bleeding from a vascular lesion, anticoagulation therapy, blood dyscrasias, surgery and invasive procedures. It is critical to identify the clinical picture and treat any active bleeding promptly . We repoA 70-year-old man was admitted to our Urology Department with a 2-month history of left-sided abdominal pain. There was no significant past medical or family history. Previous ultrasonography had shown a 10-cm cystic lesion of the spleen and left hydronephrosis, and a further contrast-enhanced computer tomography evaluation confirmed a 12-cm cystic lesion located at the upper pole of the spleen suspected to be a hemorrhagic cyst and a suspected urothelial lesion of the pelvic ureter causing hydronephrosis.An electrocardiogram performed at admission documented an unknown atrial fibrillation with high ventricular response. The patient began pharmacological cardioversion with amiodarone, beta-blocking therapy in order to slow the heart rate, and anticoagulation therapy with enoxaparin 12,000 IU/day.Two days later, the patient developed two episodes of nausea, vomiting, confusion, and sweating associated with abdominal discomfort. The general examination was normal except for pallor, and his hemodynamic parameters were stable with a blood pressure of 110/80 mmHg, pulse of 70/min, and O2 saturation of 99% on room air. Blood tests revealed that his hemoglobin had dropped to 10.2 g/dl from the initial value of 14.5 g/dl. Urgent ultrasonography and computed tomography showed active bleeding in the splenic cystic lesion associated with signs of a recent massive hemorrhage and hemoperitoneum , a high number of mitotic figures (>4 mitoses/10 high-powered field), cellular atypia, presence of necrosis, and/or hemorrhage can help to differentiate the malignant form from the benign form ,5. The r cm, a hiSurgical resection, ideally with negative microscopic margins, is the treatment of choice. Local recurrences have been reported in one-third of patients . OncologWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Financial or other relationships that might lead to a conflict of interest: none.The manuscript has been read and approved by all the authors, the requirements for authorship have been met, and each author believes that the manuscript represents honest work.


Corticosteroid-induced reduction in contrast enhancement on radiographic imaging is most commonly associated with lymphoma but has been reported in other entities, including glioma. This finding may represent a diagnostic dilemma. Concern that steroid-induced cytotoxicity obscures histological diagnosis of suspected lymphoma may lead to postponement of a biopsy. If glioma is not considered in the differential diagnosis, reduction in tumor contrast enhancement may be misinterpreted as disease regression rather than a transient radiographic change. We report a case of a patient with an enhancing right temporoparietal mass adjacent to the atrium of the lateral ventricle. After treatment with dexamethasone was started, the mass exhibited marked reduction in contrast enhancement, with symptom improvement. The clinical course suggested lymphoma, and surgery was not performed. Subsequent screening for extra-axial lymphoma was negative. Two weeks later, the patient developed worsening symptoms, and repeat T1-weighted imaging showed interval increase in size and enhancement. The findings suggested a possible diagnosis of malignant glioma. The patient underwent a stereotactic-guided craniotomy for excision of the right temporoparietal mass lesion. Final histological diagnosis was glioblastoma multiforme, World Health Organization grade IV. Patients who harbor an intracranial mass lesion are frequently treated with corticosteroids to reduce tumor-surrounding edema and symptoms associated with mass effect. Often patients experience substantial improvement in symptoms within 24 hours after corticosteroid administration. Occasionally, the mass lesion may demonstrate a marked decrease in size on contrast-enhanced magnetic resonance (MR) imaging or computed tomography (CT) scans. This feature is most commonly attributed to primary central nervous system lymphoma (PCNSL)  but has A 57-year-old woman presented with a two-week history of short-term memory loss, headaches, subtle left-sided weakness, and unsteady gait. MR imaging of the brain with a T1-weighted fast spin echo (FSE) sequence showed an enhancing right temporoparietal mass adjacent to the atrium of the lateral ventricle . The refThe patient underwent a stereotactic-guided craniotomy for excision of the right temporoparietal mass lesion. Gross examination of the lesion revealed yellow abnormal tissue of a firm consistency. Biopsy samples were sent as frozen sections to the surgical pathologist. Preliminary results were consistent with malignant glioma. An ultrasonic aspirating device was used to remove the dominant lesion. Tumor tracking from the splenium of the corpus callosum to the contralateral ventricle was not removed. Final histological diagnosis was glioblastoma multiforme, World Health Organization grade IV .At 1-month followup, the patient reported better cognitive function, resolution of headaches, and improvements in left-sided weakness and gait imbalance. She continued to require 1 mg of dexamethasone daily for symptom management. Neurooncological treatment for glioblastoma continued with a combined regimen of temozolomide and whole-brain radiation therapy. At 2-month followup, she was tolerating her cancer therapy regimen well and remained clinically stable.Corticosteroid-induced regression of cerebral mass lesions is a characteristic most often associated with PCNSL  but is sThe use of corticosteroids in suspected but unconfirmed cases of PCNSL is controversial. Current clinical practice is to refrain from corticosteroid administration to avoid rendering a tissue biopsy nondiagnostic  and delaFor glioma, corticosteroid-induced contrast reduction has been observed on both CT-  and MR-iThe amount of tumor pseudoregression can be substantial. Cases of gliomas with complete disappearance of contrast enhancement on radiographic imaging after steroid treatment have been reported , 4, 5. DTraditionally, the Macdonald criteria have been used to assess treatment response in gliomas . MeasureThis case highlights the importance of obtaining a tissue diagnosis in intracranial mass lesions that change in radiographic appearance on gadolinium-enhanced MR imaging. Although most commonly attributed to PCNSL, marked reduction in contrast enhancement may also be seen less commonly in high-grade glioma. Confirmatory testing to ensure an accurate diagnosis is imperative because corticosteroid-induced radiographic change may represent pseudoregression caused by nontumoric effects in glioma or actual disease regression from cytotoxicity in PCNSL.


Such ca protein , 5.We present a 74-year-old African–American female who presented to another hospital with low-grade periumbilical pain which she described as “bloating,” but she had experienced no weight loss. Computed tomography of the abdomen demonstrated a mass in the head of the pancreas. There was no radiographic evidence See Fig.  of lymphThe specimen contained a poorly circumscribed mass centered within the uncinate process of the pancreas and abutting the vascular groove. It was firm and, on cut section, consisted of yellow–white homogeneous parenchyma. The greatest dimension measured 2 cm in diameter. The lesion grossly appeared to infiltrate the wall of the adherent SMV. The remaining pancreas was grossly unremarkable without evidence of additional lesions or disease.At low magnification, the mass was infiltrative and composed of a polygonal-to-spindle cell population of histiocytes  and scattered small- to medium-sized lymphoid aggregates (darker areas). The histiocytes demonstrated an insidious invasive pattern as they subtly penetrated and entrapped the surrounding normal pancreatic elements and extended through the media of the SMA. The histiocytes had abundant, granular eosinophilic cytoplasm with a round-to-oval nucleus. Emperipolesis was conspicuous and readily identified, particularly within lymphatic-like spaces Fig. , inset. Rosai and Dorfman first described a clinicopathological entity separate from histiocytosis X in 1969 . ClassicOccasionally, patients have had an indolent course where the disease has spread retrograde from the lymph nodes into other organ systems. Notably, one case report described the 17-year-long course of a patient who presented at 11 years of age with nasal disease and ultimately died of diffuse involvement of his lungs, kidneys, and pancreas . AlthougMultiple organ systems have been involved in extranodal disease, notably the head and neck, eyes and orbits, and the central nervous system. The gastrointestinal system is rarely involved, and only two cases of primary pancreatic involvement have been reported. Esquivel first reported a 48-year-old African–American female with a 3.5-cm mass in the body of the pancreas, who underwent a distal pancreatectomy. This patient did not have lymphadenopathy, and preoperative biopsies had been inconclusive [It is important to distinguish patients with RDD from those with autoimmune pancreatitis (AP). In addition to chronic pancreatitis, the characteristic histologic features of AP are dense lymphoplasmacytic infiltrate centered on the pancreatic ducts, venulitis, and interstitial fibrosis. Increased numbers of IgG4+ plasma cells are also seen in most cases of AP . The patPatients who present with a pancreas mass, abdominal discomfort, weight loss, and malaise present a challenge to clinicians evaluating them for malignant process as this rare entity may present in an indistinguishable manner. In patients whose biopsy results are inconclusive or negative for malignancy despite a clinical picture of neoplasm, performing immunohistochemical labeling may be a way to discard RDD from the differential although the vast majority of patients presenting in such a fashion will have their diagnosis made upon resection.


Leiomyoma is a benign smooth muscle tumor which is rarely found in urethra. Only a handful of cases have been reported in the literature. We hereby report a case of urethral leiomyoma in a twenty-seven-year-old female who presented with intermittent hematuria. Mass was completely excised with a rim of normal tissue. Patient remained asymptomatic with no evidence of recurrence in followup. Urethral leiomyomas are rare benign tumors arising from the smooth muscle of urethra. These are more common in women . Approxi A twenty-seven-year-old woman presented with intermittent hematuria and a mass coming out from the vagina. On examination, she was found to have a fleshy mass at the urethral meatus . She didLeiomyoma of urethra is a rare condition affecting women more than men. The tumor is most common in third and fourth decade; the mean age of the appearance is around 41 years , 9. DistA differential diagnosis of urethrocele, a urethral diverticulum, caruncle, and malignancy should be considered. A careful clinical examination, urethroscopy, and radiological examination of the lower urinary tract are essential to distinguish it from urethrocele, urethral diverticulum, and caruncle. However, a microscopic examination is indispensable to exclude possibility of a malignancy although lately transvaginal ultrasound and MRI have been used to indicate the benign nature of the disease .Urethral leiomyomas must also be differentiated from paraurethral leiomyomas, which may be removed without disrupting the urethral muscle or mucosa. No malignant transformation or recurrence has been reported. Local surgical excision is the treatment of choice for urethral leiomyoma .Our case was different as the patient presented with episodic hematuria unlike the previously reported cases. The mass was located in distal urethra which is not the common site of presentation of leiomyoma in females.


Angiosarcoma is a rare disease with a poor prognosis; significantly, patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare. This article describes the case of a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which had increased in severity 2 weeks prior to presentation. After a complicated diagnostic and therapeutic process, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was made by pathological and immunohistochemical examinations. We reviewed previous cases of angiosarcoma described in the English literature to determine their risk factors, diagnosis and treatment, and we found that angiosarcoma is extremely rare, especially in the small intestine. To the best of our knowledge, this may be the youngest case of primary angiosarcoma of the small intestine with metastasis to the liver reported in the English literature. Angiosarcomas, which account for only 1 to 2% of all soft tissue sarcomas, are rare malignant tumors of endothelial origin . PrimaryHere, we report a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which worsened in the last 2 weeks prior to presentation.A 33-year-old gentleman admitted to our hospital on 5 August 2011 presented with a 4-month history of pain in the left lower quadrant of the abdomen, which had worsened in the last 2 weeks before presentation.Four months ago, the patient began experiencing abdominal pain in the lower left quadrant without any apparent cause. The symptoms became increasingly aggravated during the last 2 weeks before presentation. The presenting symptoms included paroxysmal colic in the left lower quadrant of the abdomen, fever, nausea and vomiting. The patient also experienced fatigue and an 8 kg weight loss over the last 4 months. In a nearby local hospital, an abdominal plain film was obtained, which showed intestinal tympanites.For further treatment, the patient came to our outpatient department and was admitted to the Department of Gastroenterology. A physical examination revealed a male patient with tenderness in the left lower abdominal region. There was no hepatomegaly or splenomegaly. Laboratory tests were performed, and the results were normal, including the levels of carcinoembryonic antigen and carbohydrate antigen 19–9. Computed tomography (CT) scans of the whole abdomen showed a significantly thickened intestinal wall located at the end of the jejunum and the proximal ileum, excessive ascites and a few enlarged lymph nodes in the abdomen Figure .An urgent general surgical consultation was required because of sudden severe aggravation of the abdominal pain. Abdominocentesis was performed, which showed a dark red fluid. The patient presented with peritonitis. Therefore, an emergency laparoscopic surgery was performed, which was later converted to a laparotomy following partial enterectomy. The operative findings included intra-abdominal bleeding of 1500 ml and an oedematous small intestine. Additionally, the greater omentum was found adherent to the small intestinal mesentery in the left upper abdomen with inflammation and blood oozing from the small intestine. During surgical exploration, dozens of irregular nodules of various sizes were found scattered on the surface of the liver. In addition, a mass of approximately 5.0 × 6.0 cm in size was found in the small intestine approximately 70 cm distal to the ligament of Treitz and was accompanied by an endoleak. During the operation, the tumor and the local mesentery of the small intestine were resected. Eight lymph nodes were examined, and two were found to be metastatic. On microscopic examination, vascular invasion of tumor tissue could be observed. The blood vessels of the tumor were abundant with tumor cells around them, and the tumor cells were arranged in a slit-shaped pattern Figure . The immAfter surgical resection, the patient was managed with adjuvant chemotherapy and palliative care. Approximately 400 to 500 ml hemorrhagic fluid was drained during the postoperative period through the abdominal drains. Moreover, the volume of hemorrhagic fluid increased to 600 ml on the 6th postoperative day. Therefore, a cytologic examination was performed, which showed malignant cells, confirming the diagnosis of malignant ascites. The patient received frequent blood transfusions due to a progressive drop in hemoglobin. In the early morning hours of the 27th postoperative day, the patient experienced a sudden disturbance of consciousness along with a progressive decrease in blood pressure. Due to the above-mentioned circumstances, further treatment was refused by the patient’s relatives. The patient died on the morning of the 27th postoperative day.Angiosarcoma, a malignant neoplasm derived from the endothelial cells of blood vessels or lymphatic vessels, is characterized by aggressively proliferating and widely distributed tumor cells .In 1879, Langhans and colleagues reported the first angiosarcoma in the spleen . AllisonTo gain a deeper understanding of primary angiosarcoma of the small intestine, we searched the PubMed database. The following search terms were used: (“hemangiosarcoma”[MeSH Terms] OR “hemangiosarcoma”[All Fields] OR “angiosarcoma”[All Fields]) AND  AND (English[Language]). Only 54 eligible articles were retrieved. We excluded articles that described secondary cases and articles describing animal angiosarcoma involving the small intestine. Finally, only 27 articles remained ,5,11-35 Only one younger case, a 25-year-old woman, was reported as having primary angiosarcoma of the small intestine with normal liver, spleen, rectum, urinary bladder, kidneys, uterus and adnexae [Intestinal angiosarcoma often presents with gastrointestinal bleeding, abdominal pain, intestinal obstruction, abdominal distention, weight loss, shortness of breath, anemia and weakness ,10.Young and colleagues reviewed angiosarcoma with a focus on clinical trials and outlined its risk factors . AccordiMagnetic resonance imaging (MRI), CT, positron emission tomography (PET), X-rays, ultrasound, endoscopy, immunohistochemical testing and a pathological examination can contribute to the diagnosis of angiosarcoma ,30,31. MDue to the rarity of randomized trials and prospective studies, the management guidelines for other soft tissue sarcomas tend to be utilized when dealing with angiosarcoma . CompletGenerally speaking, the prognosis of angiosarcoma is very poor. Moreover, several investigators have noted that the site of angiosarcoma tends to affect prognosis , and thePrimary angiosarcoma of the small intestine is an extremely rare and aggressive soft-tissue malignant tumor. The findings of this case report are of extreme significance. To our knowledge, this may be the first report of primary angiosarcoma of the small intestine with metastasis to the liver in such a young patient. In the future, we should focus on similar cases to ensure early diagnosis and proper treatment.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.CT: Computed tomography; GIST: Gastrointestinal stromal tumor; MRI: Magnetic resonance imaging; PET: Positron emission tomography.The authors declare that they have no competing interests.QN searched the database, selected the articles, and wrote the manuscript. DS supervised the methodology, the selection of the articles, and the writing of the manuscript and is the corresponding author of the paper. HP assisted in drafting the manuscript. MR supervised the writing of the manuscript. WB, GL, XG and DS performed the surgeries. All the authors have read and approved the final manuscript.


Sertoli Cell Tumor is a rare sex cord tumor, comprising less than 5% of all sex cord tumors. It usually occurs in women of reproductive age but a few can also occur during early childhood. The usual manifestation among children is isosexual pseudoprecocity.This is a case of a three-year-old girl who came in with a chief complaint of vaginal bleeding. She was born term to a G2P1 mother with no perinatal complications noted. At two years of age, she was noted to have gradual bilateral breast enlargement. There was no breast discharge noted. At two years and four months of age, she started to have vaginal bleeding which the mother claimed to occur on a “monthly” interval lasting for five to seven days. There was no history of trauma or any accompanying urinary symptoms. Initial consult was done in a local hospital where she was also noted to have enlarged head. Cranial CT Scan done was normal. Assessment made was precocious puberty. The patient was subsequently referred to our institution for further evaluation. On physical examination, pertinent findings include increased head circumference , weight  and height  within normal for age, Tanner Stage 3 for breast and Stage 1 for pubic hair. The rest of the physical exam findings were essentially normal. Bone age was appropriate for chronologic age. Hormonal work-up were as follows: FSH: 0.27 (NV:0.27-3.39 IU/L), LH: 0.22 (NV: 0.03-0.55 IU/L), Estradiol: 234 (NV:<10pg/mL), Prolactin: 783 (NV:80-500 microIU/mL), TSH: 9.4 (0.3-3.8 mIU/L), FT4: 22.4 (11-24 pmol/L). Pelvic ultrasound revealed upper borderline-sized uterus with slightly thickened round complex mass in the left adnexal region, to consider an ovarian neoplasm. Alpha-feto Protein, B-HCG, CA-125 tumor marker levels were all within normal limits. The patient subsequently underwent Exploratory Laparotomy with Left Salphingo-oophorectomy. Intra-operatively, the left ovary was noted to be converted to a 5cm x 5cm x 3cm solid mass with smooth surface and intact capsule. The right fallopian tube and ovary appeared normal. Histopathologic studies were consistent with Sertoli Cell Tumor of the left ovary, positive for Inhibin and Calretinin immunostaining. The vaginal bleeding and progressive breast enlargement ceased post-operatively. The estradiol and prolactin levels dropped (Estradiol: 234 to 37.3pg/mL and Prolactin: 783 to 304 microIU/mL) one month after the surgery.


EWSR1 gene rearrangement. The patient had been in a good status without evidence of recurrence 13 months after surgery. Urinary bladder PEComa is an extremely rare neoplasm and seems occur predominantly in middle-aged patients. However, this peculiar lesion can develop in pediatric population and therefore it should be rigorously distinguished from their mimickers.Perivascular epithelioid cell neoplasms (PEComas) of the urinary bladder are extremely rare and the published cases were comprised predominantly of middle-aged patients. Herein, the authors present the first urinary bladder PEComa occurring in an adolescent. This 16-year-old Chinese girl present with a 3-year history of abdominal discomfort and a solid mass was documented in the urinary bladder by ultrasonography. Two years later, at the age of 18, the patient underwent transurethral resection of the bladder tumor. Microscopically, the tumor was composed of spindled cells mixed with epithelioid cells. Immunohistochemically, the tumor were strongly positive for HMB45, smooth muscle actin, muscle-specific actin, and H-caldesmon. Fluorescence in situ hybridization analysis revealed no evidence of http://www.diagnosticpathology.diagnomx.eu/vs/1870004378817301The virtual slide(s) for this article can be found here:  Perivascular epithelioid cell neoplasms (PEComas) are defined by the World Health Organization as “mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells” . The PEC2 sharply circumscribed soft tissue mass, with a wide base. The tumor showed homogeneous intermediate signal intensity on the T1-weighted images, slightly heterogeneous hyperintense signals on the T2-weghted images, and significant inhomogeneous enhanced signals on the T1-weighted images  scan demonstrated a solid mass arising from the left posterior wall of the bladder. Subsequent magnetic resonance imaging (MRI) of the pelvis revealed a 2.5 × 2.2 cmEWSR1 gene) translocation, excluding the possibility of clear cell sarcoma of soft tissue (CCSST). The histopathological features, together with immunohistochemical pattern and genetic studies, indicated that the tumor was a PEComa.A fragmented specimen, 3 cm in aggregate, was subjected to pathological examination. Histologically, the tumor was composed of spindled cells mixed with epithelioid cells arranged in fascicles or nests, with clear to lightly eosinophilic cytoplasm Figure . NotablyThe patient’s symptoms were almost relieved completely and she was discharged on the 5th postoperative day. Six months later, a follow-up CT scan and ultrasound were normal. At the most recent follow-up 13 months following the surgery, the patient had been in a good status without evidence of recurrence.PEComa belongs to a peculiar neoplasm and accounts for an extremely smaller percentage of urinary bladder mesenchymal lesions. A search of the English literature indicated that only 12 previous cases of PEComas of the urinary bladder have been reported thus far -20, wherMicroscopically, all of the 13 cases exhibited classic features of PEComa, with admixture of epithelioid and spindled cells arranged radially around blood vessels. Five of 11 cases exhibited infiltrative growth pattern. The mitotic activity of all of the cases was no more than 1/50 HPF. Necrosis was found in 3 of 10 patients, although the necrosis in 1 of the 3 cases might be attributed to prior embolization . VasculaThe urinary bladder is an extremely rare location for PEComa, especially for pediatric population. The differential diagnosis of this peculiar lesion is broad and sometimes might be very challenging especially in small biopsy samples. This unique lesion must be differentiated from melanotic tumors and other more common types of pediatric bladder mesenchymal lesions, such as smooth muscle tumors, rhabdomyosarcoma, and pseudosarcomatous myofibroblastic proliferation.EWSR1-ATF1) or t(EWSR1/CREB1) fusion can be invaluable in distinguishing CCSST from PEComa [This unique lesion can be confused with CCSST and melanoma because of the strong positivity for melanotic markers, especially when neoplastic cells express S-100 protein. However, melanotic tumors can be distinguished from PEComa in several respects. First, cells of melanotic neoplasms are characterized by large and conspicuous nucleoli. Second, strong S-100 protein expression is usually detected in most melanotic tumors but only in a minority of PEComas . Third, m PEComa .Smooth muscle tumors can mimic PEComa, as both tumor types express muscle markers. Smooth muscle tumors usually arrange in fascicles, demonstrating ‘cigar-shaped’ nuclei and more eosinophilic cytoplasm. Immunohistochemically, the majority of smooth muscle tumors express strong positivity for desmin, whereas PEComas are usually desmin-negative or express desmin only focally. It is noteworthy to mention that very rare true smooth muscle tumors can express HMB45 ; howeverBoth rhabdomyosarcoma and pseudosarcomatous myofibroblastic proliferation are more common in this site, especially for very young patients. Additionally, these two lesions express muscle markers. However, detailed morphologic inspection combined with negativity for melanotic markers in these two lesions can be very valuable in distinguishing them from PEComas -29.TFE3 gene fusions, which usually affect children and young adults [Lastly, pediatric PEComas of the urinary system sometimes may be confused with renal carcinoma associated with Xp11.2 translocations/g adults -32. Of ng adults ,32. HoweIn 2005, a criterion for the classification of PEComas as “benign”, “uncertain malignant potential” and “malignant” was proposed by Folpe et al. . Most PECurrently, the optimal treatment for PEComas is not known. Among the 13 cases, 9 patients underwent partial cystectomy and 4 of them received TURBT. Complete excision seems to be curative and might be necessary to avoid progression. Notably, 1 patient was treated by postoperative adjuvant INF-α immunotherapy [Urinary bladder PEComa is an extremely rare neoplasm and usually occur in middle-aged patients. The present case represents the first one occurring in an adolescent, indicating that this peculiar lesion can affect pediatric population. Therefore, pediatric bladder PEComa should be rigorously distinguished from other more common types of bladder lesions. Bladder PEComas exhibited classic features of PEComa. All of the lesions seem to behave in an indolent fashion although some tumors revealed malignant morphology. Nonetheless,, the behavior of PEComa in this site remains unpredictable. Further studies, including additional large numbers of cases and longer-term follow-up periods are necessary. Additionally, cytogenetic or molecular biological investigation is also warranted to better delineate this peculiar lesion.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.PEComa: Perivascular epithelioid cell neoplasm; AML: Angiomyolipoma; LAM: Lymphangioleiomyomatosis; CCST: Clear cell sugar tumor of the lung; CCSST: Clear cell sarcoma of soft tissue.The authors declare that they have no competing interests.LY made contributions to acquisition of clinical data, pathological analysis and manuscript writing. HB drafted the manuscript. MC and JC carried out the genetic studies. JY participated in the radiological analysis; HZ (Hua Zhuang) participated in the ultrasonography diagnosis. HZ (Hongying Zhang) conceived of the study, and participated in its design and coordination and helped to draft and edit the manuscript. All authors read and approved the final manuscript.This study was supported by National Natural Science Foundation of China (no. 30971148 and no. 81272944).


Leiomyosarcoma of the anal canal is an uncommon neoplasm of the gastrointestinal tract. We report a 45-year-old lady with anal canal leiomyosarcoma. In view of its rarity, we report its presentation and management. In the setting of a recurrent tumor with high-grade histological appearance, local excision would be deemed unsafe. Leiomyosarcoma is the most common non-epithelial gastrointestinal malignancy, seen generally in the fifth/ sixth decade of life. It occurs most frequently in the stomach, followed by small bowel and colon. It may arise from the smooth muscle of muscularis propria, muscularis mucosa or the blood vessel wall. In addition, arrectores pilorum muscle of the subcutis may be another site of origin, especially in the anal canal.We report a case of recurrent leiomyosarcoma of the anal canal. The case is being reported for its rarity of occurrence.A 45-year-old lady presented with history of mass descending per rectum and occasional bleeding per rectum of 10 months’ duration. No definite history of constipation or alteration in bowel habits was present. She had consulted a surgeon 6 months ago, with similar complaints, who had diagnosed spindle cell tumor of anal canal based on a cytology report. In view of the ‘apparent benign appearance,’ local excision was done by the surgeon, but the tumor recurred within a month. At this point, she was referred to us.She was of average build and nourishment. General systemic examination was unremarkable. Per abdomen, there was no organomegaly or mass demonstrable. On rectal examination, there was a 5 × 4 cm polypoid, mobile mass confined to the posterior wall, about 2 cm from the anal verge. There was no induration of the mucosa around the base of the mass.Fine-needle aspiration cytology of the mass revealed a spindle cell tumor with high mitotic activity, probably leiomyosarcoma. Chest X-ray and ultrasound abdomen did not reveal any visceral metastases. Contrast CT scan revealed the tumor to be confined to the anal canal, without metastases or spread to the adjacent viscera. Hence she was planned for an abdomino-perineal resection.Intraoperatively, the tumor was found to be confined to the anal canal, without nodal/ visceral/ peritoneal deposits. She had an uneventful post-op course. Grossly  the reseStromal tumors of the rectum and anal canal are rare, representing 0.02% to 0.03% of malignant neoplasms in the region. Even in the large intestine, they are unusual neoplasms, comprising less than 0.1% of all malignancies of the colon and rectum. Current advice in their management is treatment by abdomino-perineal resection.[There has been an apparent increase reported in females. Palpable mass, hemorrhage and pain/ discomfort were the most common symptoms reported at presentation. With regard to size, majority of the leiomyosarcomas measured 5-9 cm at diagnosis.et al.[Age at presentation varied from 44 to 76 years  in the series of 8 patients with isolated anorectal leiomyosarcoma reported by Tjandra In our patient, abdomino-perineal resection was done in view of recurrence after local excision and the cytological appearance of high-grade leiomyosarcoma. Limited local excision of small tumors without high-grade cytology appears justified if sufficiently wide, since malignancy of leiomyosarcoma appears to remain circumscribed over long periods. However, this limited procedure runs the risk of local recurrence, with the need for abdomino-perineal resection later. Local ex


Intracranial lipomas are congenital malformations. These uncommon lesions have an incidence of 0.1 to 1.7% of all intracranial tumors. Most cases are located at midline and 5% are along the sylvian fissures. If symptomatic, seizures are the most common symptom. These tumors are slow growing and have favorable outcome. We report a case of a 25-year-old man whose CT and MRI revealed a lesion in right sylvian fissure suggesting a lipoma with abnormal vasculature and overlying cortical dysplasia. Intracranial lipomas are very rare lesions whose development is poorly understood. These lesions are neither hamartomas nor true neoplasms, but are congenital malformations. They may be considered mal-differentiated subarachnoid spaces. Lipomas at sylvian fissure are exceedingly rare. The unusual location of this lipoma prompted the reporting of this case.We report a 25-year-old man who presented with episodic headache for 5 months. He was evaluated because of increasing frequency and severity of the headaches. He had no history of seizures or other significant event. His neurological examination was unremarkable. The patient underwent unenhanced CT of the brain. On CT, a large well defined homogeneous lesion was seen in the right sylvian fissure with attenuation characteristics of fat (-90 to -110 HU). Incomplete peripheral calcification was also seen. The lesion was surrounded by abnormally thick cortex along its medial border . For bet1 Most cases are located at midline. 45% of intracranial lipomas are seen along the interhemishere, 25% along quadrigeminal/superior cerebellar cistern, 14% along suprasellar/interpeduncular cistern, 9% along cerebellopontine cistern, and 5% along the sylvian cisterns.2 These are congenital malformations that are thought to develop from abnormal persistence and mal-differentiation of the meninx primitiva during the formation of the subarachnoid cisterns. The sylvian fissure is the first sulcus to appear (16-20 weeks of gestation). Redundant meninx primitive can be found in such neural tube flexion sites. Therefore, a lipoma may develop in sylvian fissure. The folding and growth of the surrounding tissues continue until the opercula completely cover the insula to meet in a line which forms the definitive sylvian fissure. Hence, the lipoma may interfere with the development of the adjacent cortical tissue during ongoing formation of the sylvian fissure.3 Varying degrees of associated brain malformations are seen in 55% of the lesions, and 36% of the lesions show traversing intracranial vessels and nerves through them.2 The associated CNS malformations are agenesis or dysgenesis of the corpus callosum, absence of septum pellucidum, cranium bifidum, spina bifida, encephalocele, myelomeningocele, hypoplasia of vermis, and cortical dysplasias.3 The possible explanations of abnormal vasculature are manifold. One explanation of aneurysm formation is congenital structural deficiency, as the artery shares the same malformative origin with lipoma. Another explanation is that lipoma might secrete some tumoral factors which weaken the arterial wall. A third explanation may be that lipoma itself causes degeneration of arterial wall by interfering with arterial nutrition. The cerebral artery does not have vasa vasorum and derives its nutrition from CSF by diffusion. Some lipomas may show amorphous calcification. The bony components are foci of osseous metaplasia. In such a case, the diagnosis of osteolipoma is made that shows ossification histopathologically.4Intracranial lipomas are uncommon lesions with an incidence of 0.1 to 1.7% of all intracranial tumors.1 Rokitansky reported the first case of intracranial lipoma as an accidental finding at autopsy in 1856. The first case was diagnosed in a living patient in 1939 by Sosman. In 1983, Hatashita et al. reported the first case of sylvian fissure lipoma, in which the lesion was partially removed and diagnosis surgically verified.5 Intracranial lipomas are often asymptomatic. The most common symptom is seizures. However, the patient may also present with headache, emotional instability, hemiparesis or intellectual disorder.6 Clinical progress in the form of onset and worsening of symptoms is thought to be caused by tumor growth. If the lesion is causing no sign or symptoms and is diagnosed incidentally, the patient should be managed expectantly. Surgical intervention is indicated if the lesions cause symptoms. These lesions are attached inadvertently to the surrounding structures. An attempt to totally resect the lesion may injure adjacent brain. Therefore, in most cases, only partial removal is contemplated. These tumors are slow growing with favorable outcome. Therefore, the primary aim of surgery is adequate decompression.Sylvian fissure lipomas are the least common of all the intracranial lipomas with only 13 cases reported until 2007 and the angiomatous variety still rarer.On CT, the lipomas appear as well-defined homogeneous lesions with CT attenuation characteristic of fat. Calcification may be seen. There is no contrast enhancement except in the angiomatous variety, in which enhancing vessels may be seen within the lesion. On MRI, these lesions demonstrate fat signal with a short T1 and T2 relaxation times; that is, bright homogeneous signal on T1-weighted images and intermediate signal on T2-weighted images. Moreover, the signal is bright on FSE/TSE T2 sequences. Intracranial lipomas need to be differentiated from other intracranial fat containing lesions. Dermoids are less homogeneous and have higher attenuation values. Furthermore, with fat suppressed sequences, lipomas exhibit more signal intensity suppression than do dermoids. Teratomas are heterogeneous and may show contrast enhancement.Sylvian fissure lipomas are rare lesions, and with angiomatous component and cortical dysplasia, these lesions are even rarer. Our patient is managed without surgery with the diagnosis made on radiological grounds. If the lesion becomes symptomatic or diagnosis is in question, surgery will become mandatory.


Odontomas are the most commonly occurring odontogenic tumors, which are considered by many to be hamartomas rather than neoplasms. These clinically asymptomatic tumors are classified into complex and compound odontomas. They are usually discovered in radiographs and rarely cause bony expansion or infection. This paper discusses a case report of a complex odontoma exhibiting all the structural features and defects of enamel, dentine, and cementum in succession, with an overview on its etiology. Hamartomas of tooth forming tissues are termed as odontoma. They are the most common tumor of epithelial and mesenchymal origin and account for 22% of all odontogenic tumors . This noA 28-year-old male patient reported to a private clinic with a complaint of pain in the right lower posterior tooth region for about one week. On intraoral examination, a partially erupted tooth-like structure was seen. The pain was intermittent and was aggravated on chewing. Radiographic investigation revealed the presence of near-spherical opaque mass resembling calcified tissue measuring about 2 cm in diameter. The radiopaque mass with a density greater than bone and equal or greater than that of tooth was surrounded by a radiolucent rim in all areas except the erupted portion distal to normally erupted second molar .Removal of the mass was planned under local anaesthesia. Mucoperiosteal flap was raised distal to 47, and the calcified mass was removed. The spherical mass was about 2 cm in diameter with small irregular areas of indentations. The inferior side of the mass showed a hollow invagination giving the appearance of a small cup . The speGround sections were done on the dissected halves of the hard tissue mass. The ground section showed areas of enamel, dentine, and cementum in succession . The enaThe dentinoenamel junction was regular and scalloped in some areas. The underlying dentine showed “S” shaped dentinal tubules. Primary and secondary dentine types were observed with clearly visible incremental lines . HypocalPaul Braco in 1867 was the first to coin the term odontoma and defined the term as tumors formed by the overgrowth or transition of complete dental tissue . It is aAccording to WHO, this lesion is “a malformation in which all the dental tissues are represented, individual tissues being mainly formed but occurring in more or less disorderly pattern.” When the calcified tissues are simply arranged in an irregular mass bearing no morphological similarity to rudimentary teeth, they are termed complex odontoma. A compound odontoma comprises calcified structures arranged in an orderly pattern that result in many teeth-like structures, but without morphological resemblance to normal tooth . Our casThe etiology of odontoma is not clear, and various causes like local trauma, infection, hereditary anomalies like Gardner's syndrome, Hermann's syndrome, odontoblastic hyperactivity, and alteration in genetic components responsible for tooth development are also considered . HitchinSources of cells for odontomas could be mature ameloblasts, cell rests of Serres, or extraneous odontogenic epithelial cells , 10–12.  Environmental factors like trauma, growth pressure, and infection may play a role in the pathogenesis of odontoma. A vertically directed force in the form of trauma which is directed to the permanent tooth bud through the deciduous tooth can lead to morphological changes in the permanent tooth bud leading to formation of odontomas . StudiesGrowth pressure due to inadequate space is been quoted as the etiology in some odontomas. This theory by Hitchin states that abnormal pressure from the deciduous roots over the developing tooth germ could lead to the formation of odontomas , 15, 16.Odontomas were discovered at any age but the most prevalent age of detection was the second decade of life with a slight predilection for occurrence in males when compared to females . CompounOdontomas are clinically asymptomatic, although occasionally retention of deciduous teeth, noneruption, swelling, pain, tooth displacement, cortical bone expansion, and infection may be noted . AlteratEruption of odontomas is different from a normal tooth as there is lack of periodontal ligament. As there is no root formation, the increasing size of the odontoma leads to sequestration of the overlying bone by causing pressure and eventually occlusal movement and eruption . Bone reDiagnosis of odontomas is usually by radiographs. A routine intraoral radiograph helps in diagnosing the presence of odontoma and the classification is usually by visualization. A compound odontoma, in a radiograph shows well-organized malformed tooth or tooth-like structures in a radiolucent follicle-like space whereas the complex type shows irregularly shaped radiopacity surrounded by a radiolucent rim. In cases where there are numerous tooth-like structures in a compound odontoma, differentiating the two becomes difficult . If bothGround section usually reveals the presence of all hard tissues in succession representing enamel, dentine, and cementum. Hypocalcified areas of both enamel and dentine are seen. In our case, the microscopy showed remarkable features that make for interesting discussion. Treatment of odontomas is usually by conservative surgical removal and preservation of impacted or embedded tooth if present.Odontomas are common odontogenic tumors, which are usually asymptomatic and are diagnosed by routine radiographs. Etiology of this tumor is not clear, and López-Areal in his study has supported the role of injury during childhood for formation of odontomas . In our


A 1-year-old female child presented with distention of abdomen, accompanied with occasional episodes of vomiting and abdominal pain for the past eight months with no history of constipation or fever. The child was vitally stable. On inspection upper abdomen was found distended and mild tenderness in epigastrium on deep palpation. Laboratory investigations were within normal limits. The plain radiograph of abdomen was unremarkable. Ultrasound scan showed a 6.9 cm x 7.5 cm sized cystic area with internal debris at porta hepatis, compressing the liver. CT scan showed a 5 cm x 7 cm sized cyst extending from porta hepatis to the duodenum . The preoperative differentials were duodenal duplication and choledochal cyst.At operation, a cyst medial to the gall bladder, pushing the stomach and the pancreas anteriorly and intimately related to the second part of the duodenum was found .The content of the cyst was clear mucous on aspiration. Intra-operative cystogram was performed that ruled out its communication with biliary and alimentary tracts . The wall of the cyst was opened and stripping of mucosal lining performed after excising resectable portion of the cyst. The cyst was sharing common wall with duodenum and was non - communicating. The child made an uneventful recovery and was discharged on the fifth postoperative day. Histopathology of the specimen showed gastric mucosal lined tissue having smooth muscles in the wall along with ectopic pancreatic tissue .Gastrointestinal duplications may be cystic or tubular in shape with an intimate contact with the adjacent gut, smooth muscles in their wall, and mucosa resembling that of gastrointestinal tract. Duplications can present along any part of gastrointestinal tract, commonly along the ileum; duodenal duplications account for 5% of all gastrointestinal duplications. In 15-25% of cases ectopic gastric mucosa may be found. Few cases of duodenal duplications containing ectopic pancreatic tissue have been reported in literature. Concurrence of ectopic gastric and pancreatic tissues in a duodenal duplication cyst, as found in the index case, is however extremely rare [1-3]. Duodenal duplications may occur along the first and second parts of duodenum and are cystic with no communication with the intestinal lumen in most of the cases. Rarely, they can arise from pancreatico-biliary ducts. These cysts may be confused with choledochal cysts on account of their location between porta hepatis and duodenum. The presentation could be with abdominal pain, palpable epigastric mass, relapsing pancreatitis, and vomiting. In case of ectopic gastric mucosa, there could be intra-cystic hemorrhage or perforation of the cyst with peritonitis .Ultrasound scan, upper gastrointestinal contrast study, CT scan, magnetic resonance cholangio pancreatography (MRCP), and endoscopy are important tools for preoperative diagnosis. Surgical resection is the treatment of choice for alimentary tract duplications. However, in case of duodenal duplications, excision of as much as part of duplication and mucosal stripping of the rest is preferred on account of its close proximity with pancreatico-biliary ductal systems. We proceeded on the same lines in our patient. Intra-operatic cystogram is mandatory to rule out its communication with pancreatico-biliary tree. Similarly, we have ruled out the communication of the cyst with pancreatico-biliary system and gut lumen by performing intra-operative cystogram. Drainage of the duplication cysts into the duodenum or into a Roux limb of jejunum is also an acceptable alternative [1-4]. Source of Support: NilConflict of Interest: None declared


Sinusitis of dental origin is a relatively frequent entity, and odontomas are considered to be the most common odontogenic tumors of the oral cavity. Eruption and infection of odontomas are extremely rare. Here, we report an interesting case where odontoma was found in the wake of the maxillary sinusitis onset. Although sinusitis of dental origin is a relatively frequent entity , maxillaOn the other hand, the occurrence of complex odontomas is not considered to be rare in the jaws –11. MostA 12-year-and-7-month-old female pediatric patient presented with suspicious left odontogenic maxillary sinusitis and no noteworthy personal history. She complained of persistent nasal congestion and tenderness in the left molars. As a disease history, she had pain and swelling of the left cervical lymph node for one month. Symptoms were improved by intravenous infusion of antibiotics by a pediatrician who pointed out the left maxillary sinusitis by CT imaging. On suspecting odontogenic maxillary sinusitis, the patient was referred to our hospital. There was no sign of eruption of the second upper left molar; however, there was tenderness in a considerable part of the gum. Normal X-ray of the skull showed an opaque image of teeth surrounding the impaction of the second molars in the upper left maxillary sinus area . ComputeThe presence of an odontoma in the sinus is exceptional . FurtherOdontomas are the most common odontogenic tumors –13, 15 aEruption and infection of odontoma are uncommon –12, 16,


Background. Didelphys uterus is a noncommon finding in women. Till now, few cases with benign mesenchymal tumors in patients with didelphys uterus are described. We present a case of a patient with carcinosarcoma arising in a didelphys uterus. Case. A 73-year-old patient presented with profuse watery postmenopausal bleeding. On examination under anesthesia, left and right cervixes were identified. Tumor extended from the left cervix into the lower third of the vagina and was adherent to the right vaginal sidewall. There was no evidence of parametrial extension. Tissue was sent for biopsy which revealed high-grade uterine carcinosarcoma. Two uterine fundi and two vaginas in keeping with uterine didelphys were identified on imaging. The patient underwent vaginal excision of the protruding tumor measuring 8 × 6 cm with harmonic scalpel followed by total abdominal hysterectomy and bilateral salpingooophorectomy. Although a number of pelvic and paraaortic lymph nodes were also identified on imaging, she was not planned for lymphadenectomy after MDT (multidisciplinary team) discussion because of her comorbidities. The final histology confirmed the diagnosis. Conclusion. According to our knowledge, this is the second case of carcinosarcoma arising in didelphys uterus in the world literature. Persistent Müllerian duct syndrome presenting as didelphys uterus is a very rare anomaly which is estimated to occur in 1/3,000 women . HoweverA 73-year-fold patient, para 5, presented with profuse watery postmenopausal discharge and frank red bleeding per vagina for 2 weeks. On examination under anesthesia, left and right cervixes were identified. Tumor extended from the left cervix into the lower third of the vagina and was adherent to the right vaginal sidewall. The uterus was otherwise mobile, and there was no evidence of parametrial extension. Tissue was sent for biopsy which revealed high-grade uterine carcinosarcoma.After contrast-enhanced computed tomography (CT) of the chest, abdomen and pelvis showed two uterine fundi and two vaginas in keeping with uterine didelphys . Small vPositron emission tomography (PET) CT demonstrated a metabolically active tumour with intense metabolic activity corresponding to local uterine disease sites seen on MRI. PET CT also detected metabolically active nodes in the left supraclavicular fossa, mediastinum, abdomen, and pelvis in keeping with metastatic disease.Although a number of pelvic and paraaortic lymph nodes were identified on imaging, she was not planned for lymphadenectomy after MDT discussion because of her past medical history which included coronary artery bypass graft 3 years ago, hypertension, and unstable diabetes mellitus. The patient had three laparotomies as a child, the third of which resulted in a bowel resection due to anatomical duplication of her gastrointestinal tract. On examination, the patient had reduced exercise tolerance (angina at 400 yards) and also had evidence of reflux on induction of anaesthesia and respiratory wheezing. The patient underwent vaginal excision of the protruding tumor measuring 8 × 6 cm with harmonic scalpel followed by total abdominal hysterectomy and bilateral salpingooophorectomy which was achieved after mobilizing the uteri from the adjacent bladder and bowel which were adherent in the midline between the right and left uteri. Two separate uterine bodies were found, each with its own cervix and attached fallopian tube and ovary. The area of the tumor in the vaginal wall was marked with clips as the patient would have adjuvant treatment postoperatively. It should also be mentioned that the caecum was found double in size. Dilated small bowel loops were also found. The patient had an uneventful postoperative recovery.The final histology confirmed the diagnosis of carcinosarcoma stage IIIb. More specifically, the vaginal tumour was a polypoid necrotic tumour consisting of malignant columnar epithelial cells within a stroma consisting of highly atypical spindle-shaped cells. The epithelial component consisted mainly of high-grade serous carcinoma and showed marked atypia with prominent mitoses. The stromal component consisted of markedly atypical spindle and stellate cells. Occasional foci consisted of smaller ovoid cells with hyperchromatic nuclei and granular chromatin. No heterologous components were seen. The features were of a high-grade carcinosarcoma. Some residual foci of vaginal epithelium and stroma were also seen. Regarding the uteri, tubes, and ovaries, the right horn of the uterus showed a carcinosarcoma with features similar to those of the vaginal tumour. Within the corpus this consisted mainly of high-grade serous adenocarcinoma with very focal sarcomatous differentiation. Psammomatous calcification was also seen. The tumour invaded into the outer half of the myometrium and was less than 1 mm from the serosal surface. Extensive lymphovascular space invasion was seen. The right cornu, right tube, and ovary also showed tumour involvement. Tumour was present at the endocervical resection margin. The right and left horns showed tumour involvement. Tumour was also seen at the parametrial margins, bilaterally. The left ovary contained a focus of calcification and foreign body giant cell reaction but no definite tumour involvement. The left horn of the uterus showed a benign endometrial polyp. The background endometrium showed foci of simple hyperplasia without atypia. Some occasional foci of adenomyosis and benign leiomyomas were also present. The cytology of the washings revealed a cellular specimen consisting of cohesive groups of atypical cells, with prominent nucleoli. The cells aggregated to form glandular structures. Background reactive mesothelial cells and histiocytes were also present. The features were of metastatic carcinoma. The final histopathology was discussed at the Gynae Oncology MDT, and palliative radiotherapy was recommended.Fusion uterine anomalies of various kinds are not uncommon. Such anomalies result from differing degrees of failure of fusion of the two Müllerian ducts at about 9 weeks of gestation. A congenital anomaly syndrome with didelphys uterus and ipsilateral renal agenesis was first reported in 1922 . Since tMost of these anomalies do not reduce the female fertility, and this was demonstrated in our patient as she had five normal deliveries. However, complete duplication of the uterus and cervix may prevent descent of the fetal head in late pregnancy or obstruct labor by the nonpregnant horn, something that was mentioned in our patient's medical history.Usually, women with a congenital uterine anomaly have an increased risk of renal anomalies and should undergo renal tract imaging. Our case was not correlated with significant renal anomalies, but she had a history of gastrointestinal tract anomalies (duplicate colon). The other finding from the genitourinary tract was that our patient had normal urethra, as well as a second “blind” urethra. Uterine malformations may cause a delayed diagnosis of gynecological malignancies. Imaging studies including ultrasound, 3D ultrasound, CT, and MRI could help in the diagnosis of uterine malignancies in cases of didelphys uterus . We usedA Pubmed and Google search was conducted for internet-based resources and open access publications with the terms didelphys uterus or double uterus and cancer or sarcoma or malignancy and we identified 17 cases with endometrial cancer and another case with carcinosarcoma between the years 1952 and 2012 –21. So aThe question of why the patient underwent MRI could be raised as the role of MRI is to identify patients likely to be at risk of nodal disease and thereby select patients for lymphadenectomy. CT or PETCT alone would have sufficed for the detection of distant disease, whilst local staging was surgical. However, we performed first the imaging and after that the MDM decided to avoid lymphadenectomy. Carcinosarcomas are also called mixed Müllerian tumors which are characterized by a typical biphasic pattern with carcinomatous and sarcomatous elements (either homologous or heterologous). In our case, no heterologous components were found. The behavior of carcinosarcomas is characterized mainly by the carcinomatous component, and so mainly they give lymph node metastases. In our case, pelvic and paraaortic lymph nodes were identified, but because of the comorbidity, a lymphadenectomy was not performed. Radiotherapy is suggested in the literature as another option for treatment of uterine didelphys malignancy . In geneDidelphys uterus is a rare finding in women. It is even more rare to be complicated with carcinosarcoma. According to our knowledge this is the second case of carcinosarcoma arising in didelphys uterus in the world literature.


Differentiation of cystic mass lesions of the sellar and parasellar regions may pose a diagnostic dilemma for physicians, neurosurgeons, radiologists and pathologists involved in treating patients with these entities. A considerable number of tumors previously identified as craniopharyngiomas may, in fact, have been xanthogranulomas. We report a case of pituitary dysfunction caused by xanthogranuloma of the intrasellar region.A 47-year-old man of Japanese descent presented to our institution with a tumor located exclusively in the intrasellar region which manifested as severe hypopituitarism. MRI revealed a clearly defined intrasellar mass that was heterogeneously hyperintense on T1-weighted images and markedly hypointense on T2-weighted images. We preoperatively diagnosed the patient with Rathke's cleft cyst or non-functioning pituitary adenoma. Although the tumor was completely removed using a transsphenoidal approach, the improvement of the patient's endocrine function was marginal, and continued endocrine replacement therapy was needed. Postoperatively, a histological examination revealed the tumor to be a xanthogranuloma of the intrasellar region. His visual field defects and headache improved.Because diagnosis depends on surgical intervention and xanthogranulomas of the intrasellar region are very rare, the natural history of xanthogranuloma is still unknown. Therefore, this entity is difficult to diagnose preoperatively. We suggest that xanthogranuloma should be included in the differential diagnosis, even in the case of sellar lesions, to formulate appropriate postoperative management and improve endocrine outcomes. Intracranial xanthogranulomas arise most commonly in the choroid plexus, almost uniformly in the trigone of the lateral ventricle, whereas xanthogranulomas of the sellar region are quite rare . In someA 47-year-old man of Japanese origin presented with a history of headache, general fatigue and appetite loss of 3 months' duration. He was 178.2 cm in height and weighed 57 kg. His blood pressure was 90/50 mmHg, and his pulse was 64 beats/minute. His clinical examination showed the patient to be alert with initial signs of upper-visual-field defects. His physical examination revealed no absence of axillary or pubic hair and no neurological abnormalities. The results of the initial laboratory examinations conducted to determine hormone status are presented in Table MRI showed a clearly defined, intra- and suprasellar lesions with a diameter of 12 × 18 × 15 mm. The lesion appeared as mainly isointense to gray matter with some hyperintense areas and showed inhomogeneous contrast enhancement in the T1-weighted images Figures  and 1B. On the basis of the above-mentioned examinations, our preoperative diagnosis was Rathke's cleft cyst (RCC) or non-functioning pituitary adenoma associated with hypophysitis. The lesion was completely removed by surgical exploration via transsphenoidal selective tumorectomy 5 months after clinical onset. Our pathological examination of the pituitary tumor revealed that the tumor was composed of abundant small epithelial cells with foamy macrophage or lymphoid infiltration, some of which were undergoing necrosis Figure . HemosidPostoperatively, the patient's visual field defects and headache improved. However, he needed to continue endocrine replacement therapy (20 mg/day of hydrocortisone and 25 to 50 μg/day of thyroxine). MRI showed that the mass had been completely removed and revealed no evidence of tumor remnants.et al. described and classified xanthogranuloma of the sellar region as a distinct entity, after which the World Health Organization accepted that xanthogranuloma of the sellar region differs from classical craniopharyngioma in several ways: age at onset, anatomical site and localization of the lesion, and symptoms and prognosis [The spectrum of cystic pathology occurring in the sellar region includes craniopharyngiomas, RCCs, colloid cysts, arachnoid cysts, cystic pituitary adenomas, epidermoid cysts, dermoid cysts and xanthogranulomas ,11. Xantrognosis ,12.et al. [The histogenesis and pathogenesis of xanthogranuloma remain unclear, and these details are controversial and probably heterogeneous. It is generally thought that the xanthogranulomatous reaction is induced mainly by chronic inflammatory processes in circumscribed areas of craniopharyngiomas or at the cyst wall and adjacent tissue of RCCs ,13. Degeet al.  proposedet al. ,5,6,9, aTypical features of a xanthogranuloma of the sellar region include cholesterol clefts, lymphoplasmacellular infiltrates, marked hemosiderin deposits, multi-nucleated foreign body giant cells around the cholesterol clefts, accumulation of macrophages and only small epithelial cell clusters . These fFor xanthogranulomas, no typical radiological signs exist . AlthougA MEDLINE English-language literature search commencing from 2000 revealed eight separate reports of cases of sellar xanthogranuloma -7,9,15. A xanthogranuloma of the sellar region usually causes symptoms of an endocrine nature, despite favorable survival outcomes for patients with xanthogranulomas. In this case, a good outcome is defined as a socially independent life without impairment but no recovery of endocrine function. The appropriate therapeutic strategy for most cases of xanthogranuloma of the sellar region appears to be to correct the underlying endocrine problems first. Patients often need to receive long-term endocrine replacement therapy to improve their symptoms. In the eight cases of xanthogranuloma in the literature that we reviewed, no examples of recurring xanthogranuloma of the sellar region were noted, and the overall outcome for patients with this condition appeared to be favorable Table . HoweverBecause xanthogranulomas of the sellar region are rare, their nature and clinical course remain somewhat unclear. The seriousness of the hypopituitarism, the hyperintensity of T1-weighted MRI or hypointensity of T2-weighted MRI, and the absence of calcification should alert clinicians to the possibility that the tumor may be a xanthogranuloma. Therefore, xanthogranulomas should be included in the preoperative differential diagnosis of intrasellar tumors. Moreover, we suggest that such tumors should be treated as soon as possible, within a minimum of 3 to 4 months, especially in the case of tumors occupying the intrasellar region, to improve endocrinological outcomes, even if the tumors seem small.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.TN and KM was major contributor to the writing of the manuscript. TN, KM, HI and TI analyzed and interpreted the patient data regarding endocrinological disease. YK and RH performed the histological examination of the xanthogranuloma. NK and TT performed neurosurgery on the patient. All authors read and approved the final manuscript.