Leiomyoma of the mediastinum is rare. We report a case of a 57-year-old woman with a cervical mass diving to the intrathoracic. Chest radiography and computed tomography revealed a mass in the right superior mediastinum. The tumor was enucleated by cervicotomy. Histologically, the tumor was diagnosed as leiomyoma. Leiomyoma of the mediastinum is extremely rare. Only 12 cases of solitary leiomyoma that developed from small vessels in the soft tissue of the mediastinum wall have been reported in the English literature [A 57-year-old woman African origin and Moroccan nationality was admitted with a history of a slowly enlarging, painless mass in the right supraclavicular region and shortness of breath on exertion. Upon physical examination, there was a hard right supraclavicular mass adherent to the deep structures of the neck. Uterine examination revealed normal. Biochemical and hematologic analyses were normal. Chest radiography displays a large opacity in the right apical thorax that displaced the trachea to the left side . The cerLeiomyomas mediastinal are extremely rare tumors which develop from smooth muscle, usually in the esophagus and large vessels . In our case, there was no tumor dependency to the neighboring structures. Although leiomyoma pathogenesis is obscure, estrogen and traumatic theories were suggested . PrimaryResection is the adequate treatment of primary mediastinal leiomyoma. If the tumor is vasculared, an embolisation should be performed prior to operating in order to control the bleeding during surgery. The local recurrence is uncommon, but if the mass recurs, the possibility of leiomyosarcoma should be explored .The primary mediastinal leiomyoma is exceptionally infrequent. It develops from small vessels in the soft tissue of the mediastinum wall. The tumor is surgically removed, and no local recurrence has been reported so far.


Endometriosis is a benign condition affecting females of reproductive age. Although intestinal endometriosis is common it is rare for the condition to manifest as an acute bowel obstruction secondary to ileocaecal and appendicular endometriosis. This case is important to report as it highlights the diagnostic difficulty this particular condition presents to an emergency surgeon.We present the case of a 33 year old female of Asian origin who presented with symptoms and signs of an acute small bowel obstruction. A right hemicolectomy for suspected malignancy was performed with an ileocolic anastomosis. Histological examination demonstrated extensive endometriosis of the appendix and ileocaecal junction.Enteric endometriosis should be considered as a differential diagnosis when assessing females of reproductive age with acute small bowel obstruction. A high index of suspicion is required to arrive at a diagnosis of this elusive condition. Endometriosis is a benign condition, affecting 4 to 17% of menstruating women. It has a peak incidence in the third and fourth decade. Its aetiology is unknown, although there is a high incidence in sterile females as well as in those who have a family history . It is cA 33 year old woman of Asian origin was admitted to our Colorectal Unit with a one day history of absolute constipation and haematochesia. This was associated with a one week history of emesis that had gradually increased in severity. The patient was complaining of a one month history of generalised colicky abdominal pain. On the day of admission, the pain was described as severe and was scored as 10 out of 10. The constipation had commenced a month prior following her menses and had insidiously increased in severity. The patient's past medical history included three uncomplicated Caesarean sections and was otherwise unremarkable. At the time of admission, she was taking the oral contraceptive pill. She was a non-smoker and drank alcohol very occasionally. There was no family history of bowel cancer or inflammatory bowel disease. On examination the patient was comfortable at rest, haemodynamically stable and afebrile. Inspection revealed a distended abdomen with an obvious Pfannenstiel scar. On palpation, there was generalised tenderness with no rigidity or rebound tenderness. No herniae were found. Auscultation revealed tinkling bowel sounds. Per rectal examination demonstrated soft stool. Laboratory tests revealed a raised white cell count of 12700/mm3, a normal haemoglobin of 13.6 g/dL and an elevated C-reactive protein of 186 mg/dL. The arterial blood gas demonstrated a mild metabolic alkalosis with a pH of 7.461 and a base excess of 1.4. A urine dipstick and pregnancy test were both unremarkable. A supine abdominal radiograph showed dilated loops of small bowel. A CT abdomen/pelvis with oral and intravenous contrast was performed. This was reported as showing small bowel obstruction with a transition point at the terminal ileum which was thickened and stenosed. The CT appearances were suggestive of either Crohn's disease or Tuberculosis. The patient was treated conservatively with nasogastric suction and intravenous fluids. The patient initially responded well eventually regaining bowel function. However, the patient then suddenly redeveloped signs and symptoms of obstruction. Due to a rapid deterioration in the patient's condition a histological diagnosis could not be achieved prior to surgery. After obtaining informed consent from the patient, an emergency lower midline laparotomy was performed. Intra-operatively a dilated proximal small bowel was found with one constricting lesion affecting the ileocaecal junction which seemed to arise from the base of the appendix. The macroscopic appearances were suggestive of a malignancy. No other lesions were found. A right hemicolectomy was performed with a side to side stapled ileocolic anastomosis. Histological examination of the specimen was found to show a macroscopic the ileocaecal valve was compressed by outside mass and the mucosa showed an 8 mm fibrotic nodule occupying the appendiceal base which was on microscopy diagnostic of extensive endometriosis (see figures Interestingly, although intestinal involvement in endometriosis is common, it rarely causes acute intestinal obstruction . The repThe aetiology of endometriosis remains unknown and controversial ,7. ThereAcute bowel obstruction is a rare event occurring in less than one per cent of intestinal endometriosis and usually affects the rectosigmoid colon,15,16. TMedical treatment with hormonal therapy such as OCP, Danazol or Gonatrophin antagonists can be attempted for intestinal disease when there is no obstruction ,2,4. ThiAcute bowel obstruction secondary to intestinal endometriosis remains a difficult condition to diagnose without an elevated index of suspicion. Endometriosis as a differential should be borne in mind when assessing females of a reproductive age who present with small bowel obstruction. A careful history may elicit symptoms related to the patient's menses and in conjunction with equivocal CT findings should raise the possibility of intestinal endometriosis. If the condition is suspected then a pre-operative MRI small bowel is indicated. Exclusion of bowel malignancy is essential and if in doubt an oncological resection should be performed.CT: computed tomography; MRI: magnetic resonance imaging; OCP: oral contraceptive pill; VEGF: Vascular endothelial growth factorWritten 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.All authors contributed to researching, editing and writing the article. All authors read and approved the final manuscript.


A rare case of thoracic malignant schwannomma, in an adult, is presented here. This case shows an aggressive, rapid progression, which is characteristic of the disease. In spite of the best surgical and chemotherapy treatment, the patient died within four months of diagnosis. Schwannomas (or neurilemommas) and neurofibromas are the most common mediastinal tumors. They are benign, slow-growing neoplasms that frequently arise from a spinal nerve root but may involve any thoracic nerve. Malignan32-year-old male presented with chest pain and mild dyspnea of 20 days duration. He was a laborer and non-smoker with no history of exposure to any occupational or inorganic dust. Chest pain was retrosternal and pricking in character with no radiation and not related to the meals. Examination revealed averagely built person with BMI of 22, and no clubbing and lymphadenopathy. Respiratory system examination revealed diminished movements on the left hemi-thorax. There was stony dullness on the left hemi-thorax, and breath sounds were absent. No adventitious sounds were heard. Chest radiograph revealed a uniform density homogenous opacity in the left hemi-thorax occupying the full hemi-thorax except at the left apex. The mediastinum was shifted to the right side .Routine blood investigations were within normal limits. A Computed tomography (CT) of the thorax revealed a large well defined heterogenous mediastinal mass in the left hemi-thorax. There were some areas of necrosis and calcification within the mass. The mass was extending up to the pleural surface, and infiltrating into the subcutaneous tissues in the chest wall . The diaAs the diagnosis was inconclusive, the patient was taken up for thoracotomy with excision of the tumor. Intra-operatively it was observed that there was a large firm mass in the mediastinum on the left side with invasion of the great vessels and involvement of mediastinal lymph nodes. The lymph nodes involved were: left hilar group, subcarinal group, aorto-pulmonary window group, right hilar group and left interlobar group of lymph nodes. The mass was seen infiltrating the great vessels, pericardium and the chest wall. Hence, de-bulking of the tumor was done to the extent possible. Complete excision of the tumor could not be done as it was infiltrating the great vessels and the pericardium. Histologically, the specimen revealed sheets of pleomorphic spindle shaped cells, with enlarged hyperchromatic twisted nuclei and amphophilic moderate eosinophilic cytoplasm. The neoplastic cells were arranged in fascicles that closely resembled the architecture of leiomyosarcomas . They haNeurogenic tumor is the most common cause of a mass lesion in the thoracic para-vertebral region in the general adult population. Schwannomas are rare in people less than 20 years of age and are largely asymptomatic. SchwannoMalignant schwannomas typically arise from a simple or plexiform neurofibroma, but rareApproximately half of the cases occur in individuals with neurofibromatosis and the incidence of sarcomatous degeneration is seen in five per cent cases. Malignant schwannomas may occur sporadically or be induced by radiation exposure. Most of Differential diagnosis of the dense homogenous density lesion in the left hemithorax, similar to the present case, includes bronchogenic carcinoma, lymphomas (Hodgkins or non-Hodgkins disease), pleural mesothelioma, thymic tumours, teratomas, and tumours arising from sympathetic ganglia like ganglioneuroma, ganglioneuroblastoma and neuroblastomas, and encysted pleural effusion. Radiologically, schwannomas appear as a well circumscribed, round masses which are homogenous, soft-tissue density on plain CT images, with clear preservation of surrounding fat planes. OccasionIn this case, diagnosis could not be reached following routine procedures, and hence exploratory thoracotomy was performed. It confirmed malignant schwannomma. In this case, wide excision of the tumor was done. Complete excision was not possible due to the invasion of the great vessels and chest wall. He was also given adjunct radiation therapy and chemotherapy. However, due to rapid progression of the tumor, the patient succumbed to the disease within four months of diagnosis.


Splenic tumors are rare and present a diagnostic dilemma. Metastatic carcinoma to the spleen is unusual. Visceral metastases in patients with ovarian cancer represent hematogenous spread of the disease; capsular involvement resulting from serosal and peritoneal seeding is more common. We present a patient with intraparenchymal splenic metastasis from ovarian carcinoma. This case demonstrates a rare etiology of an intraparenchymal solid splenic mass.An 85-year-old woman presented with left upper quadrant pain. During her evaluation, a computed tomography scan revealed intraparenchymal splenic masses. An elective splenectomy was performed, during which ovarian cancer, which had not been revealed by the pre-operative computed tomography, was detected. There was no involvement of the splenic capsule by direct extension of the tumor, as is usually the case for ovarian cancer, but only intraparenchymal metastases. This mode of metastasis to the spleen has been described but is quite rare, and ovarian cancer presenting as a splenic mass is even more so.Splenic metastasis is a relatively rare event. It is often asymptomatic and is usually detected as part of multiorgan metastases. Symptomatic cases, though rare, do occur, and as in our patient, a thorough clinical evaluation is important to help direct the treatment plan. This case is a reminder to be cognizant of one of the less likely differential diagnoses of an intraparenchymal solid splenic mass. Splenic tumors are rare and present a diagnostic dilemma. The differential diagnosis of splenic tumors includes hemangioma, lymphangioma, hamartoma, hemangiosarcoma, malignant lymphoma, and metastatic carcinoma.Metastatic carcinoma to the spleen is unusual. Visceral metastases in patients with ovarian cancer represent hematogenous spread of the disease, and are present in 2% to 3% of patients . CapsulaWe present a rare case of intraparenchymal splenic metastasis from ovarian carcinoma.An 85-year-old woman originally presented to her primary care physician with vague pain in her left upper quadrant. She described it as a sharp, intermittent pain, not associated with meals. The pain was exacerbated by different body positions and by activity. She experienced loss of appetite and five pounds of weight loss. She had no significant past medical history, but had recently had both upper and lower endoscopies, which were normal.Physical exam revealed tenderness in her left upper quadrant. No organomegaly was identified, and no other tenderness or palpable masses were present on examination. A computed tomography (CT) scan of her abdomen was ordered by her primary care physician, and this showed multiple masses within the spleen which were suspicious for malignancy Figure . BecausePre-operatively, a complete blood count and electrolytes were within normal limits. A laparoscopic splenectomy was initiated but due to extensive adhesions and multiple tumor implants, the procedure was converted to an open approach. On exploration, numerous omental, mesenteric, peritoneal, and diaphragmatic implants were identified. The splenic capsule, however, appeared to be uninvolved. A splenectomy, omentectomy, removal of peritoneal nodules, and right oophorectomy were performed. The left ovary was not identifiable due to the dense adhesions and implants matted in that area.Pathologic examination of the gross specimen Figure  revealedA CA-125 level checked postoperatively was markedly elevated at 685.9 units/mL . The patient made an uneventful postoperative recovery, and was discharged home. After a consultation with oncology, she is currently undergoing chemotherapy.Metastatic carcinoma to the spleen is rare ,3. The iLam and Tang conducted a 25-year-long clinicopathologic study where they concluded that metastatic disease involving the spleen may not be readily identified for several reasons. First, many splenic metastases are asymptomatic, making their detection more difficult. Symptomatic lesions tend to be larger, and thus are more likely to be part of a more widely metastatic tumor. Also, some patients in the study had splenic lesions that were so small that they could not be easily identified on gross examination during an abdominal exploration. Lastly, many of the grossly detectable lesions were solitary or diffuse rather than multiple, making them more likely to be confused with primary splenic tumors such as lymphomas or hamartomas [The location of the primary tumor appears to affect the frequency of metastases to the spleen. For example, one series found that pancreatic primary tumors metastasized to the spleen more often than to other primary sites . The proLate metastases to the spleen are seen more often in melanomas, choriocarcinomas, and breast carcinomas. Symptomatic lesions, when compared to asymptomatic lesions, were larger and were found more often in women and younger patients . Those aMetastases to the spleen are frequently found as part of a widely metastatic primary tumor. In one study, all patients found to have metastatic splenic tumors eventually died of complications of their primary tumors . SplenecInvolvement of the splenic capsule in epithelial ovarian cancer usually occurs with widespread tumor dissemination , and is Pathology from our case revealed metastatic poorly differentiated adenocarcinoma from the splenic lesions and poorly differentiated papillary mucinous cystadenocarcinoma from the right ovary. There were several parenchymal metastases in the spleen, and intra-abdominal dissemination of the tumor. The capsule of the spleen in our patient was not involved by direct invasion. Parenchymal metastases may represent hematogenous spread of the disease, whereas capsular involvement represents peritoneal seeding .Solitary splenic metastasis of carcinoma of ovarian origin is exceedingly rare, with fewer than 25 reported cases. Splenic involvement, even parenchymal involvement excluding direct invasion into the capsule, is usually associated with widespread intra-abdominal dissemination of the tumor. This, in fact, was the case with our patient, who had widespread visceral, peritoneal, and diaphragmatic tumor implants. In the rare cases of solitary splenic metastasis, splenectomy may have value beyond providing tissue diagnosis and preventing splenic rupture. Splenectomy, combined with oophorectomy and an appropriate chemotherapy regimen, can be part of a therapeutic and not merely a palliative procedure. Laparoscopic splenectomy, if achievable, is preferred over open splenectomy in these cases [Our patient had multiple parenchymal splenic metastases and was symptomatic. In fact, her initial presentation was because of the discomfort caused by her affected spleen. She had an elevated CA-125 level and there were visible parenchymal splenic lesions on CT imaging. Splenectomy, omentectomy, removal of peritoneal nodules, and a right oophorectomy were performed. In reported cases, an open surgical splenectomy was performed except in two patients where a laparoscopic approach was used . The advIn conclusion, splenic metastasis is a relatively rare event. It is often asymptomatic and is usually detected as part of multiorgan metastases . SymptomWritten 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.AG provided the case information, and was a major contributor to the discussion section of the paper. ZH interviewed the patient, reviewed the medical records and wrote the case presentation. DC provided major contributions to the case presentation and discussion sections, and edited the final manuscript. SP researched the subject and provided major contributions to the discussion section. DJ researched and wrote the pathology portion of the manuscript and prepared the histology figures. All authors read and approved the final manuscript.


Primary lymphoma of the bladder is a rare condition with less than 100 cases reported in the literature.Here we present the case of a 64 year old woman with a 9 month history of haematuria, frequency, urinary incontinence and weight loss. Cystoscopy revealed a solid tumour throughout the whole bladder wall and a vesico-vaginal fistula. This was confirmed on CT scan which also showed no other organ involvement and bilateral hydronephrosis. Trans urethral biopsies taken at cystoscopy revealed Non-Hodgkin's Lymphoma. The patient had a nephrostomy inserted and is currently receiving a course of chemotherapy.We believe this is the first documented case of primary bladder lymphoma causing a fistula and therefore we suggest that lymphoma should be included in the differential for any patient with a fistula involving the bladder. Non-epithelial tumours of the bladder are extremely rare. The most common amongst these are rhabdomyosarcomas in children and leiomyosarcomas in adults. PrimaryPrimary lymphoma of the bladder was first described by Eve in 1885 and to dConsiderable debate remains as to the origin of primary bladder lymphoma. This is largely because normal bladder microscopic sections fail to reveal routinely the presence of lymphoid follicles. A populLymphoma of the bladder is more common in women,3. The mA 64 year old woman presented to our outpatients clinic with a 9 month history of painless haematuria, frequency and urinary incontinence. On further questioning she admitted to suffering from night sweats and 3 stones of weight loss over the past year. She had been repeatedly treated for urinary tract infections by her general practioner. There was no other past medical history apart from abdominal hysterectomy.There was nothing abnormal to find on physical examination of the abdomen or other systems. The urea and electrolytes were normal as was the patient's peripheral blood film.An abdominal ultrasound scan revealed bilateral hydronephrosis, a large solid pelvic mass with the dimensions 70 mm × 72 mm × 70 mm and no visible bladder despite attempts to fill with water. Cystoscopy showed solid tumor throughout the entire bladder with minimal lumen and a vaginal fistula. This was confirmed by a CT and showed a diffuse 30 mm circumferential thickening of bladder  chemotherapy. Four months later a repeat CT showed that changes in the region of the bladder were less marked  declare that they have no competing interests.


Scrotal leiomyomas are rare tumours which are essentially benign. Recurrence and malignant transformation to leiomyosarcoma have been reported. However, a specific subgroup with increased bizarre nuclei showing increased mitosis raises the need for a closer follow-up. We report on such a case.We report the case of a 65-year-old man who underwent a scrotal lump excision. Histology showed a well defined leiomyoma. The presence of nuclear pleomorphism and mitoses, just falling short of the criteria for malignancy, made prediction of biological behaviour difficult. The patient remains well on 4-year follow-up.Histological evidence of increased mitosis raises the need for sustained follow-up in view of the malignant potential from the extent of mitosis. Immunohistochemistry helps in identifying those patients warranting close follow-up. Leiomyoma of the scrotum is a rare entity described as a benign pathology. Immunohistochemistry helps differentiate this condition from a leiomyosarcoma. However, we raise the entity of symplastic scrotal leiomyoma with bizarre nuclei and increased mitosis on immunohistochemistry. The pattern of growth in this distinct subset is not known. Theoretically, there is a higher risk for malignant transformation. We discuss this situation and suggest the necessity for close follow-up.A 65-year-old man presented with a single well-defined, soft, non-tender, mobile right scrotal lump, increasing in size for 4 weeks, with no palpable connection to his testis, epididymis or spermatic cord. Herniae and palpable inguinal lymph nodes were absent. An ultrasound of the testes as well as testicular tumour markers were not undertaken as the testes were considered normal on clinical examination with the lesion being a testicular adnexal mass. The lump was excised from the scrotal dartos layer. Histology showed a well-defined leiomyoma made up of spindle cells in which numerous degenerating uni- and multinucleate tumour giant cells  were identified; nuclear pleomorphism and four mitoses/ten high power fields were also noted. The tumour was smooth muscle actin- and desmin-positive, confirming smooth muscle phenotype Fig. . The patScrotal wall leiomyomas are rare, usually asymptomatic tunica dartos tumours, commonly seen in middle-aged Caucasian men . They arThey are often less than 3 cm in diameter and are more commonly solitary than multiple . TypicalSimple surgical excision is curative; surgery for large lesions should be conservative if its cutaneous origin is clearly separate from the testis or adnexal structures . RadiatiIn symplastic scrotal leiomyoma, the presence of nuclear pleomorphism and mitoses, just falling short of the criteria for malignancy, makes prediction of biological behaviour difficult. Immunohistochemistry helps identify this subgroup of patients who warrant close follow-up in view of the malignant potential.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.JP conceived the case report, collected the patient's information and was involved in writing the manuscript. PV collected the patient's information and was involved in writing the manuscript. RM helped collate patient information and was a major contributor in writing the manuscript. JM conceived the case report with JP, performed the histological examination and was involved in writing the manuscript. All authors read and approved the final manuscript.


A 22-year-old woman was admitted to our institute with complaints of fever for the past 5 days; cough, chest pain and progressively aggravating breathlessness for the past 2 days. There was no significant past history. On examination she appeared moderately ill; temperature was 100°F, heart rate was 100/ min and respiratory rate was 22/min. Chest examination revealed decreased breath sounds bilaterally.What are the radiological findings?What is the likely diagnosis?CT scannogram  shows a Preoperative diagnostic aspiration was attempted, which revealed turbid fluid that showed numerous neutrophils on cytology.CT scan findings of a well-defined multilocular cystic mass with areas of soft tissue, fluid and fat attenuation and calcification, and the presence of neutrophils on cytology suggest a diagnosis of an infected mature teratoma.The patient subsequently underwent a thoracotomy. A large complex well-defined mediastinal mass was found, which showed multiple areas of adhesion to the surrounding lung and pericardium. The adhesions were separated and the mass was completely excised. She recovered well from the operation. Postoperative chest X-ray (CXR) showed well-aerated bilateral lung fields. Preoperative lung atelectasis had completely resolved. Histopathological analysis of the resected specimen confirmed the diagnosis of a benign mature teratoma. She has been well for 4 months postoperatively.nd and 4th decades of life, with an equal distribution with regard to sex.[Germ cell tumors are classified into benign forms, which include mature teratomas; and malignant forms such as seminomas, malignant teratomas, embryonal carcinomas, choriocarcinomas and endodermal sinus tumors. Benign td to sex.Teratomas are slow-growing tumors and are mostly discovered incidentally on chest radiographs performed for other reasons. Symptoms arise from compression of adjacent structures, mainly the cardia, great vessels and airways; and from compressive atelectasis of surrounding lung parenchyma. Secondary infection or hemorrhage into the mass or malignant degeneration is known to cause a rapid increase in size of the mass, making it symptomatic. TrichoptImaging is crucial for arriving at the correct diagnosis. Chest radiographs reveal a well-defined round or oval mass in the anterior mediastinum extending to one side of the midline. Areas of calcification, well-formed teeth or bone may be visualized. Chest CT scan establishes the diagnosis. It readily detects fluid, fat, calcification and soft tissue densities. Mature teratoma appears as a well-defined multilocular  cystic mass with areas of soft tissue, fluid and fat attenuation and calcification or a variable combination of the four. Calcification may be focal or rim like; well-formed teeth or bone may also be seen. CT accurately delineates the extent of the mass and also displays its relationship to the adjoining mediastinal structures. Findings on chest MRI include a heterogeneous signal-intensity mass containing a variable combination of fluid, fat, soft tissue and calcium.56 Ultras57Benign teratomas can be differentiated from malignant germ cell tumors by the presence of greater amount of soft tissue, ill-defined margins and invasive features in the latter.The combination of fluid, fat, calcification and soft tissue densities is highly specific of a teratoma and is helpful in distinguishing teratoma from other anterior mediastinal masses such as thymoma or lymphoma.Teratomas can get infected from contiguous foci in the lung parenchyma or via the bloodstream. With onset of infection, there is a rapid influx of fluid and sudden increase in size of the mass, turning it symptomatic as seen in our case.Mature teratomas are considered chemo- and radio-resistant, and surgical excision is the treatment of choice. Complete excision is considered curative.Mediastinal teratomas are usually asymptomatic and discovered incidentally on chest radiographs. This is a rare presentation of a benign teratoma presenting as a huge mediastinal mass with characteristic radiological features, extending into bilateral hemithoraces and coming to clinical attention with acute-onset respiratory discomfort secondary to infection within the mass. The diagnosis was accurately established preoperatively on the basis of characteristic CT scan findings. Such a large mediastinal teratoma has only rarely been reported previously.


Low-grade extraskeletal osteosarcomas (ESOS) are extremely rare.MDM2 and CDK4 oncogenes. Unfortunately, at this time, no surgical treatment was possible due to loco-regional extension, and despite chemotherapy, the patient died one year after diagnosis, five years after the first symptoms.We present the first case of low-grade ESOS of the chest wall, which occurred in a 30-year-old man. Because of initial misdiagnosis and patient's refusal of surgery, the diagnosis was done after a 4-year history of a slowly growing mass in soft tissues, leading to a huge (30-cm diameter) calcified mass locally extended over the left chest wall. Final diagnosis was helped by molecular analysis of We describe the clinical, radiological and bio-pathological features of this unique case, and review the literature concerning low-grade ESOS. Our case highlights the diagnostic difficulties for such very rare tumours and the interest of molecular analysis in ambiguous cases. Osteosarcoma (OS) typically develops in the intramedullary cavity of long bones of adolescents and young adults and is a high-grade tumour ,2. ExtraLow-grade ESOS, whose histology is similar to well-differentiated intraosseous and parosteal OS, is extremely rare. To date only six cases have been reported in the literature. Here, we describe a new case of low-grade ESOS, which developed in the chest wall. Initial diagnosis, based on radiological and histological aspects, was erroneously myositis ossificans. Correct final diagnosis, suspected by the clinical evolution, was confirmed by molecular analysis, but was too late, and the patient died from extensive loco-regional progression. We hope that this case report underlines the diagnostic difficulties of this tumour, and the interest of molecular analysis in ambiguous cases.The patient was a 30-year-old man, Caucasian type, without any specific medical personal or familial history. He was referred to our institution in November 2006 for diagnosis and treatment of a huge tumour of the left chest wall, detected for the first time 4 years earlier. Indeed, in May 2002, he presented a 3-cm painless, mobile, intramuscular mass on the left shoulder near scapula, three months after a benign trauma on this region. Magnetic resonance imaging (MRI) showed elements in favour of a myositis ossificans circumscripta. Percutaneous biopsy showed areas of osteogenesis with mature bone trabeculae in muscle. The intertrabecular space revealed benign-appearing fibroblastic proliferation. The retained diagnosis was myositis ossificans. The benignity of this disease with usual spontaneous stabilisation or regression , and theHowever, the patient was lost to follow-up, and the mass continued to grow slowly during two years. A second percutaneous biopsy was performed in May 2004. Histological analysis showed an intramuscular tumour made of bland fusiform cells with few mitoses and mature bone trabeculae Figure . The morThe patient was then lost to follow-up during 17 months until November 2006, when he was referred to our institution because the mass had kept growing, leading to a shoulder immobilisation. The ECOG status was 1, and the patient presented a frozen left shoulder associated with ipsilateral radial and cubital paralysis and cervical pain. Physical examination found a voluminous, plane, hard and immobile mass extended from the basicervical region to the scapulo-humeral joint and from the left side of the chest wall to the axillary region. Biologically, there was not any abnormality except an elevation of serum alkaline phosphatases. Radiological images were impressive. CT scan showed a huge calcified heterogeneous mass, with kystic and solid elements Figure . The masMDM2 and CDK4 oncogenes, whose amplification had been reported in low-grade intraosseous and parosteal osteosarcomas [in situ hybridization (FISH) analysis showed high amplification for both MDM2 and CDK4 , cisplatinum (P) and ifosfamide (I). The disease was stable after 2 API/AI cycles. After 4 additional API cycles, the disease progressed with extension of cutaneous nodules and tumour mass associated to the apparition of left pleuritis. The ECOG status was 3, and the patient complained of thoracic pain and dyspnoea. Palliative radiation therapy was not possible technically due to the tumour volume. A thoracoscopic talcage was done, followed by trabectedin-based chemotherapy. Three weeks after the first cycle, the patient deteriorated rapidly and died of respiratory failure secondary to loco-regional extension, 5 years after the initial symptoms.Low-grade ESOS are extremely rare with only 6 cases reported to date in the English literature since 1953 -14. To oThese cases are summarised in Table However, differential diagnosis may be problematic at early stages with other ossified lesions of soft tissues: benign lesions such as myositis ossificans circumscripta (MOC), ossifying lipoma, soft tissue osteoma or chondroma, and ossifying fibromyxoid tumour, as well as malignant lesions such as classical high-grade ESOS, parosteal OS, mesenchymal chondrosarcoma, and synovial sarcoma. MOC is a benign heterotopic ossification of soft tissues characteristically associated with direct trauma . It is oSAS, CDK4 and MDM2, is relatively frequent in osteosarcoma, notably in low-grade parosteal OS, making them suitable as markers for distinguishing them from benign ossifying [MDM2 and CDK4 were done on 2006 from paraffin-embedded tumour samples biopsied on 2004. They showed the overexpression and amplification of these two oncogenes, thus ruling out the diagnosis of myositis ossificans and confirming that of sarcoma. To our knowledge, it is the first case of low-grade ESOS with documented molecular alterations.Today, molecular analyses may resolve the diagnostic dilemma between low-grade ESOS and benign lesions. Amplification of genes in the 12q13-15 region, such as ssifying -9. In ouMDM2 and CDK4) to help diagnosis in ambiguous cases.Our case is the seventh case of low-grade ESOS reported in literature, and the first case located in the chest wall. It highlights the importance and difficulty of early diagnosis of this very rare tumour, which may be confused with numerous benign diseases, notably myositis ossificans. Misdiagnosis may be fatal, as surgery is the only curative approach for these patients. In this context, it is worth noting the importance of molecular analysis (amplification and/or ovexpression of Written informed consent was obtained from the patient's relatives for publication of this case report and any accompanying images.The authors declare that they have no competing interests.RS and FB conceived and designed the study, and wrote the manuscript. FB, PJW, PV, AS and IBR participated in patient's management. CB, GdP, FP, BC made the pathological and molecular explorations. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/10/645/prepub


Appendiceal adenocarcinoma is rare with a frequency of 0.08% of all surgically removed appendices. Few cases of appendiceal carcinoma infiltrating the bladder wall for spatial contiguity have been documented.A case is reported of a 45-years old woman with mucinous cystadenocarcinoma of the appendix with bladder metastasis. Although ultrasonography and voided urinary cytology were negative, abdomen computed tomography (CT) scan and cystoscopy and subsequent pathological examination revealed a mass exclusively located in the anterior wall of the bladder. Histopathology of the transurethral bladder resection revealed a bladder adenocarcinoma  with focal mucinous aspects penetrating the muscle and perivisceral fat. Laparotomy evidenced the presence of a solid mass of the appendix  extending to the loco-regional lymph nodes. Appendectomy and right hemicolectomy, linfoadenectomy and partial cystectomy were performed. The subsequent pathological examination revealed a mucinous cystadenocarcinoma of the appendix with metastatic cells colonising the anterior bladder wall and several colic lymph nodes.The rarity of the appendiceal carcinoma invading the urinary bladder and its usual involvement of nearest organs and the posterior bladder wall, led us to describe this case which demonstrates the ability of the appendiceal cancer to metastasize different regions of urinary bladder. The appendiceal adenocarcinoma invading the urinary bladder is extremely rare -4, and iin situ were also identified  with some episodes of gross hematuria. The ultrasonography and voided urinary cytology, and the physical examination were negative. The patient never had any gastrointestinal symptom and presented a negative regress medical history. The enhanced abdomen computed tomography (CT) scan evidenced a solid mass  at the level of the anterior bladder wall Figures  and 2. Cd Figure . In addii.e. ovary, colon and rectum) may invade the urinary bladder [Appendiceal adenocarcinoma is rare with a frequency of 0.08% of all surgically removed appendices. To our best knowledge it was reported few cases of appendiceal carcinoma infiltrating the bladder wall for spatial contiguity -11. It i bladder ,11. In t bladder . All pat bladder -9. In ouThe rarity of the appendiceal carcinoma invading the urinary bladder and its usual involvement of nearest organs and the posterior bladder wall, led us to first describe this case which demonstrates the ability of the appendiceal carcinoma to metastasise different portions of urinary bladder.The authors declare that they have no competing interests.GT, MC, MS, AB performed all surgical procedures and drafted the manuscript. PC confirmed all histological findings. FG, AP, GG, PAZ, PG helped to draft the manuscript. All authors read and approved the final manuscript.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 pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/10/62/prepub


Hepatic embryonal sarcoma (HES) is a rare but aggressive primary tumor of the liver occurring most frequently in childhood.We report a case of a 52 year old woman having previously undergone treatment for ovarian serous papillary carcinoma who subsequently presented with a large solitary mass in the liver. Initially this was presumed to be metastasis from the ovarian primary however, on further examination it was shown to be a primary hepatic embryonal sarcoma.Primary liver tumors should be considered in differential diagnoses in patients with ovarian cancer who subsequently present with liver tumors. This is particularly important when there is no direct evidence of recurrence of ovarian cancer. Hepatic embryonal sarcoma (HES) is rare primary tumor of the liver usually occurring in childhood. It is generally considered an aggressive tumor with a poor prognosis. Approximately 60 cases have been reported in adults. On the contrary, ovarian carcinoma frequently tends to metastasize to the liver . We repoen bloc subtotal colectomy for bilateral high-grade ovarian serous papillary carcinoma infiltrating the sigmoid colon. She also had peritoneal and diaphragmatic seedlings which were debulked concurrently. After the resection, the residual disease was estimated to be less than 0.5 cm2. Subsequently, she received 6 cycles of adjuvant chemotherapy with carboplatin and taxol. Her CA125 level which was elevated at 149 U/L prior to resection later returned to 8 U/L . Apart from chemotherapy, there was no past history of exposure to radiotherapy or any carcinogens.A 52 year old woman presented with pain and a palpable mass in the right upper quadrant of the abdomen 18 months after completing treatment for ovarian carcinoma. She had previously undergone bilateral salpingo-oophorectomy and en bloc. Post-operatively, she made a good recovery without any complications.During this presentation, the only abnormality on routine hematology and biochemistry was a slightly elevated alkaline phosphatase at 178 U/L . The CA125 level was within the reference range (7 U/L) and no other tumor markers were assayed. A Computerized Tomography (CT) scan revealed a large heterogeneous mass almost completely replacing the right lobe of the liver (Figure Macroscopically, the tumor was a large, solid and cystic mass that included areas of necrosis and cystic degeneration measuring 18 × 12 × 8 cm Figure  with focAfter remaining well for approximately 6 months she developed progressive shortness of breath and a follow-up CT scan demonstrated a large recurrent tumor contiguous across the right hemithorax and abdomen. A percutaneous biopsy of this mass confirmed the recurrence of the primary hepatic sarcoma. Palliative chemotherapy with ifosfamide and doxorubicin was commenced but had to be stopped after 3 cycles due to severe side effects. She received palliative care for gradual deterioration and died 6 months later.Ovarian cancer usually presents with widespread intra-peritoneal metastasis . A minorApproximately 20% of the patients with epithelial type of ovarian cancer have been reported to have normal levels of CA125 ,5. DurinPrimary liver sarcomas are rare tumors and represent only 0.2% of all primary liver tumors . This grThe presentation of liver sarcomas is usually non-specific with symptoms such as abdominal discomfort, anorexia, fever or weight loss . HepatomThere are a number of radiographic features which distinguish HES from mesenchymal and other primary hepatic tumors. The myxoid stroma typical of HES appear as large central areas of hypointense signal on T1-weighted images that have high intensity signal on T2-weighted images. Similarly, in the solid peripheral areas of the mass heterogeneous contrast enhancement is seen on both CT and MRI scans .et al reported on a series of 8 primary liver sarcomas including two HES managed with surgical resection and adjuvant chemotherapy [et al identified 17 children with HES treated with initial conservative surgery and multi-agent chemotherapy followed by second-look surgery for any residual disease [HES have previously been associated with poor outcome. In 1978 Stocker reported a series of 31 patients with HES with mean survival of less that one year after diagnosis . Althougotherapy . Followiotherapy ,15. Biso disease . They reIn this patient HES masqueraded as a metastatic ovarian carcinoma. This case illustrates that the possibility of a primary liver tumor should be considered in patients with ovarian cancer who subsequently present with liver tumors, particularly when there is no direct evidence of recurrence of ovarian cancer.Written informed consent was obtained from the patient's next of kin 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.PK and AJ wrote the manuscript, CS reviewed the histology and contributed to the manuscript, NJ, EH critically reviewed the manuscript and RP performed the hepatectomy. All authors read and approved the manuscript.


To describe an unusual anterior chamber lesion found on routine eye examination of a 71 year-old Hispanic gentleman who presented for cataract evaluation. The lesion was biopsied at the time of cataract surgery and its pathology presented.A 71 year-old Hispanic gentleman presented for routine cataract evaluation. We found an unusual lesion in the anterior chamber of the right eye. The patient underwent uneventful phacoemulsification surgery. The lesion was biopsied at the time of cataract surgery and sent for pathology. Clinical photos and its pathology are presented in this article.Despite biopsy and several ancillary testings, the nature of this lesion remains unknown. Only long-term follow-up of the left eye might reveal clues as to the origins of this unusual lesion. We describe an unusual anterior chamber lesion found on routine eye examination of a 71 year-old gentleman who presented for cataract evaluation. The lesion was biopsied at the time of cataract surgery and its pathology presented.A 71 year-old gentleman presented to our clinic for cataract evaluation. His past medical history was negative except for well-controlled type II diabetes. On examination, his vision was 20/70 in the right eye and 20/50 in the left. His intraocular pressures were 15 mm Hg in both eyes. Slit lamp examination revealed a quiet conjunctiva in both eyes, as well as nuclear sclerosis cataracts consistent with his visual acuity. Posterior segment examination did not show any diabetic retinopathy. In addition, we noticed an unusual lesion in the anterior chamber of the right eye. The lesion was pearly white, solid looking and had multiple finger-like projections (Figure Due to the unusual nature of the lesion, an oncology consultation was requested. A plain radiographic film of the orbit was obtained and did not reveal any calcification. Blood levels of calcium and magnesium were normal. The patient underwent an uneventful phacoemulsification surgery of the right eye with excisional biopsy of the lesion at the time of cataract surgery. The lesion was strongly adherent to the posterior corneal stroma and was rubbery in nature, requiring dissection into the cornea. It was removed in small pieces, and sent for pathological identification. Pathology of the mass revealed a paucicellular eosinophilic material, mildly PAS positive, and consistent with connective tissue. Stains for amyloid were negative Figure .A review of the literature using PubMed has revealed previously reported cases of anterior segment epibulbar choristomas containing brain tissue arising from the cornea ,2. HowevQuestions as to the etiology of this lesion remain unanswered. Did this lesion originate in the posterior cornea and then protrude in the anterior chamber, or did it originate in the anterior chamber and got stuck to the posterior cornea? Could it represent some form of hamartomatous proliferation of the posterior stroma, or was it acquired? Only long-term follow-up of the left eye might reveal some clues as to the origins of this unusual anterior chamber lesion.The authors declare that they have no competing interests.SP examined the patient and took pictures of the lesion. PO examined the patient and recommended ancillary testing and appropriate consultations. DN and JL examined the patient and recommended appropriate testing and consultations. They also performed the cataract surgery and biopsy of the lesion. The pathology specimen was sent to Wills Eye Institute to be examined by Ralph Eagle, M.D. DN compiled all the data and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.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.


Ectopic sebaceous glands occur rarely in the esophagus. A 65-year-old man presented with a history of discomfort during swallowing since the last 4 months. On upper gastrointestinal endoscopy, multiple wart-like grayish-yellow projections were detected and two of them were biopsied. Microscopically, they proved to be sebaceous glands in the esophagus. Histogenesis of this rare lesion is discussed in this case report. Sebaceous glands are derived from the ectoderm. Ectopic sebaceous glands have been reported in various sites such as lips and mouth (in Fordyce's disease) eyes, orbits, palms and soles, salivary glands, tongue and larynx. In esophagus, which is an endodermally derived organ, the presence of ectopic sebaceous glands is rare. This conA 65-year-old man presented with a history of discomfort during swallowing since the last 4 months. There was no history of vomiting or hematemesis and weight loss. The patient was not a chronic smoker or an alcoholic. No history of chronic tobacco chewing was reported. General physical examination was unremarkable. Respiratory and cardiovascular systems were normal. Clinically, carcinoma of esophagus was suspected, and upper gastrointestinal (GI) endoscopy was performed.On upper GI video endoscopy, multiple (nearly 20) grayish-yellow nodular projections of 2–4 mm diameter were noticed in the middle and lower esophagus. Endoscopy also revealed features of mild chronic gastritis. Endoscopic diagnosis of papillomata was suggested; punch biopsy from two of the yellowish projections was performed and sent for histopathological examination.Microscopically, the non-keratinized stratified squamous epithelial lining showed minimal hyperplasia. The subepithelial area revealed lobulated sebaceous glands, deep in lamina propria. Each lobule consisted of polygonal cells with small nuclei and abundant clear cytoplasm . No hairMild lymphocytic infiltration was seen in the vicinity of the sebaceous glands. No histological features suggestive of reflux or infectious esophagitis were noted. Histological diagnosis of ectopic sebaceous glands in the esophagus was made.The patient was treated symptomatically with antacids and omeprazole for one month, and he became symptom free and remained so at two years of follow up.The presence of ectopic sebaceous glands was first described in lips and oral cavity by Fordyce in1896 . Since then, it has been described in various other sites, such as eyes and orbits, palms and soles, parotid glands, and larynx.However, in 1962, De La Pava and Pickren first reported the presence of sebaceous glands in an endodermally derived organ, the esophagus.[Ectopic sebaceous glands in esophagus were first observed during endoscopy by Ramakrishnan and Brinker in 1978 2The most interesting aspect of this condition is histogenesis, which is not completely clear. Several hypotheses have been postulated.Metaplasia theory: This is favored by the occurrence of this condition in the elderly, and not in children, and because the arrangement of these glands are similar to that of esophageal submucosal glands.Heterotopia theory : This theory is not supported by endoscopic studies and autopsy studies conducted on infants and children.There were no reports on any significant complications resulting from this condition. Endoscopic follow-up in such cases after a period of 8 months to 5 years showed no significant variation in the number or size of these lesions. MoreoverMost of these lesions appear to be harmless, and only symptomatic treatment or anti-reflux treatment is advised. Ectopic Ectopic sebaceous glands in esophagus are a very rare occurrence. It probably represents a metaplastic change in the esophageal submucosal glands. Here, we reported this rare occurrence in a 65-year-old male.


Windsock deformity (WD) is a rare anomaly. A case of double jejunal web with WD causing neonatal intestinal obstruction is being reported. Jejuno-ileal atresias have been categorized into type I , type II (atretic fibrous cord), type IIIa (V-shaped mesenteric defect), type IIIb (‘apple peel atresia’), and type IV (multiple atresias). WindsockA three-day-old full-term male neonate was admitted with a history of abdominal distension and bilious vomiting since birth. The baby had not passed meconium since birth. Epigastric distension was noted on abdominal examination. The abdominal radiograph revealed a few gaseous shadows in the proximal gut and completely gasless lower abdomen. An ultrasonography and echocardiography ruled out any associated genito-urinary and cardiac anomalies.At laparotomy, dilatation of the proximal 5 cm of jejunum, duodenum, and stomach were seen. The small gut loops distal to the obstruction were completely collapsed. A longitudinal enterotomy was performed just distal to the site of obstruction along the antimesenteric border. A windsock web causing total obstruction of the jejunum was noticed proximal to the enterotomy site . The webWindsock deformity of the jejunum due to congenital web is a very rare anomaly with scant literature. Furthermore, to the best of our knowledge, the presence of two webs in the jejunum has not been reported till date in the English literature and hence the importance of this case.et al. treated a one-year-old boy with high jejunal membranous stenosis successfully by antimesenteric longitudinal enterotomy over the diaphragm, excision of the latter, and transverse closure of the bowel.[et al. reported a case of jejunal web with a central perforation situated 8 cm from the duodeno-jejunal junction in a four-year-old emaciated male child with history of intermittent episodes of bilious vomiting, abdominal pain, and failure to thrive. Enterotomy with excision of web was done and the child had an uneventful post-operative recovery.[Andrews and Stem (1981) reported a case of jejunal web in a 48-h old Arab female neonate. The newbhe bowel. Kothari recovery. Our caseThe treatment for jejunal web remains surgical excision even though variable approaches may be used. Endoscopic laser therapy has been successfully tried for duodenal web. Simultaneous laparotomy with endoscopy has also proved useful. Our pati


Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm of soft tissue without bone marrow involvement or other systemic characteristics of multiple myelomaA 42 year-old woman presented with intermittent dry cough of 10 months duration. Her breathing sound was slightly coarse without rales or rhonchi on auscultation. CT scan revealed a right anterior mediastinal shadow with multiple pulmonary nodular lesions. A video-assisted thoracoscopic surgery (VATS) was performed. Histopathology showed it to be a myeloma.This is the first presentation of EMP with a mediastinal mass with multiple pulmonary nodules. Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm of soft tissue without bone marrow involvement or other systemic characteristics of multiple myeloma -3. It isA 42 year-old woman presented with intermittent dry cough of ten months duration. No fever, chest tightness, bone pain, anorexia, dyspnea on exertion or body weight loss was noted. She took some medicines at a local clinic to control her cough. However, blood-tinged sputum appeared one month prior to hospital admission. No bone pain or lymphadenopathy was noted on examination. Her breathing was slightly coarse without rales or rhonchi on auscultation. A chest X-ray Figure  and compTracing back her history, she was a non-smoker, without occupational or environmental exposure to air pollutants or micro-organisms. No family members had any similar clinical manifestations nor had any died of cancer before. No related travel history was noted during this time.After admission, a bronchoscopic examination revealed no endobronchial lesion or abnormal secretions. A pulmonary function test showed a mild restrictive ventilatory defect. The whole body bone scan was negative for tumor involvement. A CT-guided biopsy was recommended but the patient refused to undergo this procedure. The hemogram, leukocyte differentiation count, and coagulating profile were all within normal ranges.A biopsy through video-assisted thoracoscopic surgery (VATS) was then indicated to confirm the diagnosis. A 3 cm working incision and a 1 cm scope port were designed for performing this procedure. Removing a wedge of lung tissues including the masses from two separate sites were performed smoothly and the microscopic examination showed a solid mass made up mostly of plasma cells Figure , but negAfter confirming the diagnosis, the patient received a series of related evaluations such as serum calcium, urine Bence-Jones protein and plasma electrophoresis for M protein detection. However, all of the above examinations were negative. The skull, spine and pelvis X-ray revealed no osteolytic lesions. The bone marrow biopsy revealed normal patterns of cell distribution.She received adjuvant chemotherapy, as originally planned, including mephalan and steroids, and her symptoms improved after two months of treatment.Plasma cell neoplasm can be classified into the following types: multiple myeloma (bone marrow and other systemic involvements), solitary myeloma (bone plasmacytoma), extramedullary (soft tissue) plasmacytoma, and plasmablastic sarcoma . ExtrameDiagnosis of undetermined pulmonary nodular lesions can be accomplished by transbronchial, CT- or sono- guide needle biopsy, as well as surgical biopsy through the VATS or open thoracotomy ,16. VATSThe EMP should be differentiated from other plasma cell tumors. As described in the above paragraph, there should be no systemic signs to exclude the possibility of multiple myeloma. In addition, the EMP should also be differentiated from other types of plasma cell tumor, such as reactive plasmacytoma and plasma cell granuloma, or lymphoma  ,18. AlthLike other plasma cell tumors, EMPs are highly radiosensitive with 80 to 100% of patients successfully achieving local control and with a 50 to 65% ten-year disease-free survival rate ,20. SurgWe presented a case of EMP with a mediastinal mass with multiple pulmonary nodules. After reviewing the literature, this has never before been reported as a clinical manifestation of EMP.The author(s) declare that they have no competing interests.SPL – literature review and write clinical part of this manuscript.YSL – pathological diagnosis, collaborate the completion of the pathological part of this manuscript.CHT – pathological diagnosis and write pathological part of this manuscript.TCT – completion of the clinical part of the manuscriptAll authors read and approved the manuscript


Angiosarcoma of the head and neck is an uncommon, aggressive malignant entity most commonly found in elderly Caucasian males. We present a case in a young black female with co-existing HIV. The atypical gender, age and race of the patient reflect the unusual clinical presentation of this case of angiosarcoma, attributable to the patient's HIV status.A 22 year old patient presented with a large unresectable lesion over the occiput with surrounding ulceration, satellite lesions and associated lymphadenopathy. She is HIV-infected with a CD4 count of 360 cells/μl. She was not on antiretroviral treatment based on South African treatment guidelines advocating antiretroviral treatment when the CD4 count is below 200 cells/μl, in the absence of other AIDS-defining illnesses.The patient was treated with a course of ifosfamide and anthracyline based chemotherapy. Disease progression was noted on chemotherapy and she was subsequently palliated with a course of radiotherapy. She had a satisfactory response with an improvement in local symptoms. She is currently receiving symptomatic care.South Africa is at the epicenter of the HIV epidemic. Consequently, the management of patients in the field of oncology in our clinical practice is often burdened with malignancies manifesting with an atypical disease presentation and clinical course. Angiosarcoma of the head and neck is an uncommon aggressive cancer of the skin and soft tissues . These mWe report an atypical presentation of a case of angiosarcoma of the scalp occurring in association with HIV in a 22 year old black female.Linkage studies of population-based registries involving people with HIV/AIDS and cancer have shown a statistically significant increase in the incidence and relative risk of many Non-AIDS Defining Cancers (NADCs). Tumours displaying unusual features and aggressive behaviour patterns in young individuals should alert physicians to the possibility of underlying HIV infection .HIV-related defects in the cell mediated immunity lead to an increased risk of malignancy due to decreased tumour surveillance and suppression of oncogenic viruses. However, the role of immunosuppression in the pathogenesis of NADC is controversial, based on studies demonstrating that the increased risk of NADC is not associated with low CD4 T-lymphocyte cell counts or the onset of AIDS [The impact of HIV on the South African healthcare system has been profound. The total number of persons living with HIV in South Africa increased from an estimated 4.1 million in 2001 to 5.2 million by 2009. For 2009, an estimated 10.6% of the total population is HIV positive [The scourge of HIV in sub-Saharan Africa has contributed to the unusual clinical presentation and natural history of malignant disease.A young black female presented with a 6 month history of a mass over the posterior aspect of her scalp which was initially thought to be an abscess and subsequently ulcerated. There was no history of previous radiotherapy or trauma to the scalp.Clinically, she had a 12 cm by 10 cm diameter, ulcerative lesion over the occiput with surrounding ulceration and satellite lesions. Examination of the neck revealed bilateral level II-V lymphadenopathy measuring approximately 1 cm in diameter  scan revealed a large heterogeneously enhancing soft tissue mass over the scalp in the occipital region with erosion of the underlying bone. Extensive subcentimetre enhancing lymphadenopathy was noted in the anterior and posterior triangles of the neck with the largest noted in the right parotid region measuring 1.1 cm. No distant metastases were noted Figure .2 I.V d1-3, Ifosfamide 1.5 g/m2 I.V d1-3, q 3/52). Clinical disease progression was noted after 3 cycles of chemotherapy  over 2 weeks. She had a satisfactory response to treatment with an improvement in local symptoms.She was subsequently managed symptomatically.Sarcomas are uncommon in the head and neck region constituting only 1% of all malignant neoplasms in this region. Angiosarcomas make up less than 1 percent of all sarcomas. Their most frequent site of origin is the head and neck, especially the scalp of elderly men. These highly aggressive mesenchymal tumours may arise in association with some recognised clinical condition (chronic lymphoedema or previous irradiation) or as a de novo occurrence .Cutaneous angiosarcoma of the head and neck is a distinct subtype which most commonly presents as an enlarging purple bruise-like lesion that develops over several months. There may be intermittent bleeding, oedema or ulceration. A delay in diagnosis is common in the early stages of disease due to confusion with infection, or traumatic bruises .These malignancies spread radially within the dermis of the scalp and face and are associated with ecchymotic patches extending beyond the obvious lesion, making it difficult to achieve excision with clear margins. It is often multifocal and associated with a high incidence (10-15%) of lymph node metastasis .There is currently no accepted staging system for this disease .There is also no standard treatment schedule for these malignancies on account of its rarity. Surgery is considered the mainstay of treatment with wide local excision and frozen section control. Prognosis correlates well with the ability to attain clear surgical resection margins although the tendency of scalp angiosarcoma to demonstrate a diffuse pattern of clinically undetectable spread makes resection challenging .The role of radiotherapy is less well-defined. The reported outcomes of radiotherapy alone have largely been unsatisfactory. Several authors have thus recommended a treatment approach comprising both surgery and radiotherapy. Wide field radiotherapy appears to be a rational therapeutic choice for scalp angiosarcoma because the involved dermis as well as a sufficient area of surrounding skin can be treated, while sparing the brain and other normal tissue .Chemotherapy has been suggested for unresectable cases, but has generally not proven beneficial . AnthracThe overall prognosis for angiosarcomas is poor when compared to other head and neck sarcomas with the 5 year survival rate ranging from 4 to 20% . Local rSouth Africa is at the epicenter of the HIV epidemic. Consequently, the management of patients with malignant neoplasms in our context is often compounded by atypical disease presentation and clinical course. This case of angiosarcoma of the scalp co-existing with HIV is indicative of the challenges faced by the oncology fraternity in our country.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 author declares that they have no competing interests.PSG participated in the treatment of the patient, collection of case details, literature search and drafted the manuscript. The author has read and approved the final manuscript.


A 60-year-old woman presented with a history of pain in the back of the chest, with occasional cough, for the last 3 months. She had a history of a radical mastectomy for carcinoma of the right breast 17 years ago. The routine blood counts were within normal limits.The patient underwent a contrast-enhanced CT scan of the chest Figure  and B anth rib is seen. The adjacent neural foramen is not widened. The lungs and abdomen were normal on the other images.The contrast-enhanced CT of the chest  shows a The provisional diagnosis of a calcified paravertebral hydatid cyst was offered, with alternative diagnoses of a calcified neurogenic tumour or a calcifying fibrous pseudotumor (CFPT) of the pleura. To rule out a malignant process an FNAC was performed, which confirmed the diagnosis of a hydatid cyst by demonstrating scolicial hooklets and laminated hydatid wall membranes.et al. has reported a similar case of a calcified paravertebral hydatid cyst.[Mediastinal involvement by echinococcosis is rare and only around 100 cases have been described. Benzartitid cyst.et al. found mediastinal hydatid cysts in 8 of 1619 cases of intrathoracic hydatid cysts (0.5%).[Thameur s (0.5%). The mosts (0.5%).4Rakower and Milwidsky recordedWhen calcification is present in a hydatid cyst, it can be curvilinear, lamellated, or diffuse, the curvilinear form being more common. In the pHydatids are diagnosed on the basis of the clinical, radiological, and laboratory findings, though FNAC or surgical pathology is often required for confirmation. FNAC has generally not been recommended as a diagnostic procedure since it has been believed that it could cause an anaphylactic reaction and possible dissemination of infection. However Primary treatment of mediastinal hydatids is complete surgical resection. Preopera


Giant cell tumor of talus is a rare entity. In contrast to GCT of long bones, most cases occur in a younger age group and tend to be multicentric. The authors report a case of GCT in a 19 year old boy which had led to extensive destruction of the talus. In view of the extensive involvement, total talectomy along with tibio – calcaneal arthrodesis was performed. At 6 months of followup, the patient had a painless and well arthrodesed ankle. There was no evidence of recurrence at 18 months of followup. Talus is a rare site for involvement by Giant Cell Tumor. The authors report a Giant Cell Tumor which had led to destruction of the entire talus in a 19 year old boy. In view of the extensive involvement, total talectomy along with tibio – calcaneal arthrodesis was performed with the aim of achieving a stiff but painless joint.A 19 year old boy presented with chief complaints of insidious onset pain in the right ankle since the last two years, swelling in the right ankle since the last six months and inability to bear weight on right side since the last six months. There was no history of fever, loss of appetite, loss of weight, similar complaints in other joints or history of similar complaints in the past. The family, occupational, recreational and drug histories were not significant. The general physical and systemic examinations were within normal limits.On local examination, the attitude of the limb was neutral. There was a 5 × 4 cm swelling over dorsum of right foot and anterior aspect of ankle joint. There were no visible veins, sinus or discharge from the swelling. The local temperature was increased and the swelling was tender. All movements at the ankle joint were painfully restricted.Serum biochemistry studies were within normal limits. AP and lateral radiographs of the ankle showed a radiolucent lesion occupying the whole of talus with expansion and thinning of the cortex Figure . CT scanThe condition, its prognosis and various treatment modalities were discussed at length with the patient. In view of the extensive involvement of talus, total talectomy with tibio – calcaneal arthrodesis was planned. The patient was a manual laborer and therefore chose the option of a stiff but painless joint.Total talectomy was performed via the standard anterior approach. Fusion was achieved by autologous iliac crest graft and stabilization with a Steinmann pin Figure . HistopaGiant cell tumour, also known as osteoclastoma, is a fairly common bone tumour accounting for 5% of all the primary bone tumours. It is a benign tumour with a tendency for local aggressiveness and high chances of recurrence. The most common sites are distal end of femur, upper end of tibia and lower end of radius . The fooThe clinical picture is that of insidious onset pain, which in many cases may be mismanaged as ankle sprain. A history of preceding trivial trauma may be present. Other features are non specific.Radiologically, the tumour appears as an eccentric lytic lesion with cortical thinning and expansion. There is absence of reactive new bone formation. The tumour may erode the cortex and invade the joint. Pathological fracture may also be seen . CT scanMany authors have reported satisfactory results with intralesional curettage and bone grafting . HoweverPartial or total talectomy may be contemplated in cases where there is extensive involvement of the talus. Arthrodesis may or may not be done, but it is said that arthrodesis is essential after resection of all tarsal bones except calcaneum . Fresh fAP: Antero Posterior; CT Scan: Computed Tomographic; Scan GCT: Giant Cell Tumor.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.IHW analyzed and interpreted the patient data regarding the disease. NM and NG discussed the case with radiology and pathology experts and formulated the treatment plan. AQS, SS, NM and NG performed the operative procedure. SS was responsible for followup and prepared the manuscript. All authors read and approved the final manuscript.


A 3-year-old boy presented with iso-sexual precocious puberty for 18 months. Radiological images revealed left suprarenal mass and hormonal profile showed markedly increased testosterone and dehydroepiandrosterone-sulfate. The child underwent open adrenalectomy and histopathology revealed adrenocortical carcinoma. At 3 months follow-up, the child is doing well. Adrenocortical neoplasm has bimodal age distribution, with its peak in the first and fifth decades. Adrenocortical neoplasms in children are uncommon, comprising 6% of all adrenal neoplasms and 0.3% of all pediatric neoplasms ViriliziA 3-year-old boy presented with enlargement of the penis and pubic hair for 18 months and deepening of voice and axillary hair for 6 months. On examination, the child was otherwise healthy and normotensive Pubic hairs, axillary hairs and hairs in the beard area and upper lip were present. Stretched penile length was 9.8 cm. Bilateral testes were normally descended and normal in size. There were no skin spots and no evidence of features of Cushing syndrome. No lump was palpable on abdominal examination. Spine and digital rectal examinations were unremarkable.His hemoglobin was 14.0 g% and blood biochemistry reports were normal, except serum alkaline phosphatase which was 939 U/l. Serum electrolytes were Na: 137 meq/l and K: 4.2 meq/l. Chest X-ray was normal. Hormone profile of the patient is summarized in Ultrasonography of the abdomen revealed left adrenal solid mass with few speculated calcifications. Contrast enhanced computed tomography (CT)-whole abdomen  showed aThe child underwent left open adrenalectomy via subcostal transperitoneal approach. The postoperative period remained uneventful.Histopathology revealed a well-circumscribed, encapsulated nodular tumor (6.7 cm) in its greatest axis, weighing 125g. The cut-section showed a variegated pattern, with soft and friable intratumoral nodules with areas of hemorrhage and necrosis . MicroscAdrenocortical carcinomas with mixed virilizing and Cushing’s syndrome are typical, but pure virilizing adrenocortical carcinomas are uncommon, comprising 5–10% of the cases in most large series. ViriliziOur patient had markedly increased serum testosterone and serum DHEA-S. High concentrations of adrenal androgens, especially DHEA-S, are highly suggestive of a virilizing tumor and contrast-enhanced CT-abdomen localizes the adrenal mass.Prognosis for benign adrenal tumors is excellent. On the other hand, usual prognosis for adrenocortical carcinoma is generally poor, with an overall 5-year survival of 20–25%. Factors associated with poor prognosis, specifically for virilizing tumors, include incomplete resection, weight >80 g, volume >200 ccm, age >3.5 years at diagnosis, preoperative symptom duration >6 months and marked increase in urinary 17-ketosteroid and 17-hydroxysteroids. Pure virilizing carcinomas in general appear to have a better prognosis than other adrenal carcinomas. In our cet al. utilized the modified criteria of Weiss to analyze pediatric adrenocortical tumors.[Bugg l tumors. This claRegarding staging, in our case, the tumor was completely resected with a negative margin, weighed 125 g and there was no evidence of metastasis. The abnormal hormone levels returned to normal after surgery and thus our case was labelled as stage 1, high-grade adrenocortical carcinoma.Hence, it is concluded that a child with virilizing symptoms should be promptly investigated for biochemical profile and radiological imaging. Surgical resection is the treatment of choice. Surgery is the only definitive therapeutic modality in children, which can be curative only if adrenocortical carcinoma is diagnosed early and treated promptly.Adjuvant chemotherapy is used for metastatic or recurrent disease, but paucity of cases makes its evaluation difficult. The role


Peliosis is a rare benign disorder that is characterized by the presence of diffuse blood-filled cystic spaces and can occur in the liver, spleen, bone-marrow, and lungs. We present a 10-year-old boy with Fanconi anemia who presented with peliosis hepatis due to androgen treatment. Magnetic resonance (MR) imaging revealed multiple non-enhancing masses. Some of the lesions revealed fluid-fluid levels and extrahepatic extension on MR images. Diffusion-weighted (DW) imaging showed restricted diffusion. Fluid-fluid levels and extrahepatic extensions are unusual findings for hepatic peliotic lesions. In addition, DW imaging findings of peliosis hepatis have not been reported previously. Peliosis hepatis is a rare benign entity, which is characterized by the presence of multiple blood-filled lacunar spaces within the liver. This disorder can mimic other hepatic masses, such as hemangioma, hepatocellular carcinoma, abscess, metastasis, adenoma, and focal nodular hyperplasia . Imaging2 during free breathing. An apparent diffusion coefficient (ADC) map was automatically calculated. The lesions were slightly to markedly hyperintense both on T2- and T1-weighted images with no contrast enhancement. Two of the lesions also showed fluid-fluid level on T2-weighted images. In addition, one right lobe mass was extending outside the liver contours (-3 mm2/s), and normal-appearing liver had a mean ADC value of 1.21 × 10-3 mm2/s. Because of the clinical history of the androgen usage, the diagnosis of peliosis hepatis was reached, and the androgen therapy was discontinued.A 10-year-old boy was admitted to our hospital with Fanconi anemia. After the diagnosis, the patient started treatment with oxymethalone. Eighteen months after the initiation of therapy, liver enlargement and abnormal liver function tests were noted. US revealed multiple solid-appearing hepatic lesions, predominantly hypoechoic, and intralesional small cystic areas. To further characterize the liver lesions, MR imaging was performed by using a 1.5-T MR unit. A routine MR imaging sequence, which included un-enhanced and gadolinium-enhanced axial and coronal T1-weighted fast low angle shot (FLASH) sequences, and axial and coronal T2-weighted turbo-spin echo sequences, were obtained prior to DW imaging. A DW single-shot spin-echo sequence was performed with b-values of 0, 400, and 800 s/mmcontours . On DW scontours . ADC valBartonella infection) have been reported in the etiology  and several disease entities (such as malignancies, solid organ transplantations, chronic infections, and etiology ,6.Peliosis hepatis is characterized by the presence of blood-filled cavities in the liver . The lesThe clinical course may range from asymptomatic to a progressive case with liver failure or fatal intra-abdominal bleeding ,3,6. If Imaging findings are variable and depend on the pathologic presentation and the various stages of the blood component of the lesion. The signal intensity of the lesion on MR imaging largely depends on the age and the status of the blood component. T1-weighted imaging can demonstrate hypo, iso- or hyperintense foci. On T2-weighted images the lesions were reported as hyperintense ,5. The lIn addition, only one case of fluid-fluid levels was described on CT images previously, by Hiorns et al. . Both ofWith the advent of ultrafast sequences, DW-imaging is now feasible for abdominal studies, and several researchers have used this technique in differential diagnosis of benign and malignant lesions. DW-imaging findings of peliosis hepatis have not been described previously. Although peliosis hepatis is a benign condition, ADC values were lower for than normal-appearing liver, probably due to its content including thrombus and hemorrhage. We speculate that both fluid-fluid levels and low ADC values on MR images were related to old and new blood products in the lesions.In conclusion, peliosis hepatis must be added in the differential diagnosis of an atypical liver lesion with fluid-fluid levels and restricted diffusion. The use of MR imaging and DW imaging may be useful in the diagnosis of this challenging but rare disease entity. Extension outside the liver contours, fluid-fluid level inside the lesion, and restricted diffusion on MR images may help to differentiate peliosis hepatis from malignant lesions and prevent unnecessary and dangerous interventions.


Gastrointestinal tract duplications are rare congenital malformations that may occur anywhere in the alimentary tract from the mouth to the anus, and vary greatly in presentation, size, location, and especially in symptoms. We present a case of an infected spherical colonic duplication, in a 20-day-old baby, located at the splenic flexure of the colon. The prominent symptom was acute abdomen, accompanied by bilious vomiting, intestinal obstruction, and high fever. We present this case, due to atypical clinical presentation and the inability of the imaging modality to establish the diagnosis preoperatively. Gastrointestinal tract duplication (GTD) is defined as a structure which shares a common muscular wall and blood supply with the adjacent portion of the gut. The epithelial lining of the duplication resembles a part of the gastrointestinal or respiratory tract . They ar3, erythrocyte sedimentation rate 35 mm/h, whereas all other findings were normal. An upright plain abdominal radiograph revealed intestinal obstruction with air-fluid levels and massively distended and oedematous intestinal loops in the upper abdomen . This mass was full of pus and adherent to the antimesenteric border of the splenic flexure of the colon and shared a common muscle wall with it (2 × 2 cm in dimension). The spherical mass was isolated from the colon and removed easily. Examination of the colon and the mass did not reveal any communication between them. The muscle coat of the colon was simply sutured. The histopathological examination of the excised specimen showed histological patterns resembling the colon, resulting in the final diagnosis of colonic obstruction due to an inflamed and purulent non-communicating spherical duplication in the splenic flexure of the colon.th postoperative day.The postoperative course was uneventful and the baby was discharged on the 6The first report of a GTD was published by Galder in 1733, and Ladd was the first that introduced the term "duplication of the alimentary tract" in 1937. Gross, in 1950s, classified the duplications into four variations according to the shape: tubular, double-barrelled, cystic and spherical. Tubular structures branch out of the intestine and extents for some distance between the mesenteric leaves. The double-barrelled duplications communicate with the intestinal lumen at one or both ends. The cystic structures are attached by a thick mesenteric stalk only and are lying free in the peritoneal cavity. The spherical duplications, most common of all, are contiguous with some part of the bowel, particularly along the ileum . CurrentApproximately 75-85% of duplications are located within the abdominal cavity, and the remaining are intrathoracic (20%) or thoracoabdominal (5%) . MultiplAlimentary duplication should be considered in any pediatric patient suffering from unexplained and non-specific gastrointestinal symptoms with atypical clinical presentation, which makes a correct diagnosis difficult, and in many cases delayed for a long time. Approximately two thirds of all intestinal duplications are discovered within the first two years of life, with one third identified in the newborn period. However in some series the percentage of symptomatic patients in the neonatal period is higher 69%) . Symptom% . SymptUltrasonography is the imaging modality of choice for the detection and diagnosis of duplication cysts, as it will identify a three-layered image, unless complications such as haemorrhage and infection have supervened ,9. ApproAlthough GTDs considered benign lesions, if they are not treated properly they can have lethal consequences. Most of the authors believe that once the diagnosis is made, an elective surgical procedure should be performed to avoid complications and to perform the procedure in an optimal state of the patient, but few think that only symptomatic duplications should be surgically treated . If inciWritten informed consent was received from the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CL performed the exploration and wrote the paper. CS helped in the exploration and in the writing of the paper. MAK helped in the exploration and reviewed the literature. CT reviewed the literature. OT, helped in the review of the literature and the writing. All authors read and approved the final manuscript.


Distant metastases of the parotid gland are uncommon. They arise from primary tumors located in the head and neck, mainly melanoma and epidermoid carcinoma. Other histological types of metastasis are very rare. We report an exceptional case of parotid metastasis of myxoid liposarcoma in a 42-year-old man and insist on the worse prognosis of this entity. Parotid metastases are uncommon; they arise from cancers located in head and neck . MetastA 42-year-old man presented with a painless mass in the left preauricular area which had beenrapidly enlarging over a period of 1 month. There was no evidence of tumorspread beyond the parotid gland. The patient has a history of liposarcoma ofthe thigh soft tissues 5 years before, treated by surgery and radiotherapy. At the time, no metastatic disease wasidentified at the medical examination including X-ray of the chest and abdominal ultrasound. No other past medical or surgicalhistory is noticed.Computed tomographic scan revealed a 6 cm heterogeneous mass of the parotid with areas ofadipose density see . The patThe original resection consisted of a parotidectomy specimen measuring 7 × 4, 5 × 4 cmand weighing 65 g. The tumor was a relatively well-demarcated tan/yellow,measuring 5, 5 × 3 × 2, 5 cm, with focal myxoid areas, surrounded by scant glandularparenchyma.Microscopically, the tumorinfiltrated extensively the parotid gland see . It had A postoperative radiotherapy was performed but the patient developed pulmonaryand cerebral metastasis two months after the diagnosis, and he died of diseasetwo weeks after.Secondary malignant tumors of the parotid are rare. Head and neck cutaneous tumorsespecially squamous cell carcinomas and melanomas are the most common primarytumors . ParotidMore rarely Merkel cell carcinoma and rhabdomyosarcoma can metastasize to the parotid.However, metastatic liposarcoma to the parotid was exceptional. To our knowledge, onlyone case has been reported in the English literature by Alemán López et al. ; it was Distinction between primary liposarcoma and metastatic liposarcoma to the parotid gland is ofparticular importance for therapy and prognosis; it can be difficult especiallyif the primary site is unknown. Surgical excision with wide free surgical margins appears to be the besttreatment for primary liposarcoma. In the head and neck region, the close proximity of vital neurovascularstructures tampers the extent of excision which may result in severe morbidity;nonsurgical treatment modalities have limited value . The appIn our case, a total parotidectomy and a sacrifice of facial nerve were preformed with adjuvant radiotherapybecause surgical margins were positive.In our case and one more case before , metastaIn our case, patient died of disease from pulmonary and cerebral metastases, ten weeksafter the diagnosis.


Gastrointestinal stromal tumors are CD117 (C Kit) positive mesenchymal neoplasms, that may arise anywhere in the gastrointestinal tract. Their current therapy is imatinib mesylate before or after surgery.We describe a case of 17-year-old female with metastasis to the cervix uteri of a primary mesenteric gastrointestinal tumor.Surgery remains the mainstay of known curative treatment. The manifestations of GIST are not restricted to the typical locations within the bowel; may have very unusual metastatic sites or infiltrations per continuitatem. Gastrointestinal stromal tumor (GIST) is a rare mesenchymal tumor of the gastrointestinal tract with an incidence of 10–20 cases per million populations of which almost one third are deemed malignant [A 17 year old unmarried female presented with history of menorrhagia and passing tissue per vagina since 3 months, low grade fever since 1 month and feeling of mass per abdomen since 10 days. On examination, she had a mass corresponding to 24 weeks size gravid uterus arising from pelvis, which was tender on palpation. There was anemia. Four units of blood transfusion were given in a private clinic. Her routine liver and renal function tests and coagulation profile were normal. Ultrasound followed by contrast enhanced computerized tomography (CT) showed bilateral adnexal masses with a large soft tissue heterogeneous mass suggestive of sarcoma of the uterus and presence of enlarged retroperitoneal lymph nodes. Examination under anesthesia revealed the same mass with side to side restricted mobility, and a fleshy, friable and vascular growth protruding into vagina. Biopsy from the growth was initially misdiagnosed as a leiomyosarcoma. Exploratory laparotomy was carried out as the patient was continuously bleeding and staging was performed. Intraoperatively, there was a large 30 × 30 × 12 cm friable mass with necrosis and hemorrhagic areas arising from the mesentery of the transverse colon, with tumor deposits were present all over the pelvic peritoneum. A solid right adnexal mass, 10 × 4 cm was also present. Resection of tumor with wide margin along with panhysterectomy and infracolic omentectomy was done. Cut section of uterus, with bilateral fallopian tubes and ovaries showed serosal deposits. Endocervix revealed a 4.5 × 4 × 1 cm cauliflower like, exophytic, polypoidal vascular friable growth lying at the lower end. Vagina was grossly looking normal. Histopathological examination revealed a primary gastrointestinal tumor of mesentery with >5 mitoses per 50 high power field and atypical spindle cells arranged in fascicles. There were metastasis in the cervix, serosa and omentum. Immunohistochemical stains showed positivity for vimentin, smooth muscle actin, desmin and CD 117 , small intestine 15%), colon and rectum (5%), other abdominal organs including mesentery and omentum (5%) . Usual a%, colon This is usually followed by relapse in more than 50% of patients, which is mostly intraabdominal. Targeted adjuvant therapy against KIT tyrosine kinase using imatinib mesylate ST1571) 400 mg has revolutionized the treatment of GISTs . GISTs h1 400 mg Surgery remains the mainstay of known curative treatment. Recently, the diagnosis and management of GIST has undergone a revolution with the emergence of CD117 staining and the transforming oncogene (KIT mutation). Its specific targeted inhibition is greatly effective in treating GIST. The manifestations of GIST are not restricted to the typical locations within the bowel; may have very unusual metastatic sites or infiltrations per continuitatem. Thus, a multidisciplinary approach including a gynecologist and a medical oncologist can improve the prognosis of patients with GIST.The author(s) declare that they have no competing interests.NG, literature search prepared the draft manuscript, SM, NL, RM helped in preparation of manuscript, LK, helped in preparation of oncology part of the manuscript, SB contributed the pathological aspect of case. All authors read and approved the final manuscript.