Urachal cyst is one of a spectrum of urachal abnormalities most commonly found in children. They are very rarely seen in adults because the urachus is normally obliterated in early infancy.We describe a case of a 32 year old male Caucasian who presented with a tender, midline, infraumbilical mass and purulent umbilical discharge. Diagnosis of an infected urachal cyst was confirmed on magnetic resonance scan. He was treated initially with broad spectrum antibiotics in order to allow sepsis to resolve prior to surgical excision of the cyst and fibrous tract. Cystoscopy was performed intraoperatively to exclude sinus communication with the bladder. Histology of the excised specimen showed chronic inflammation with no evidence of malignancy. Postoperative recovery was uneventful.Urachal abnormalities are rare in adults. Clinical presentation is non-specific; therefore, a high index of suspicion is required in order to make the diagnosis. When diagnosed, surgical excision is advised because of the risk of malignant transformation. Urachal abnormalities result from incomplete regression of the foetal urachus. They are more common in children than in adults, due to urachal obliteration in early infancy .In adults, urachal cyst (UC) is the commonest variety, with infection being the usual mode of presentation . DiagnosWe describe a case of urachal cyst presenting with a tender infraumbilical mass, purulent umbilical discharge and sepsis, in a previously fit and well man.A 32 year old male Caucasian was referred after presenting to the accident an emergency department with a 10 day history of persistent purulent umbilical discharge associated with constant lower abdominal pain, chills and rigors. He gave no history of nausea, vomiting or change in bowel habit. Systemic review revealed no abnormality. He had completed a course of antibiotics prescribed by his General Practitioner with little relief.On examination, he was apyrexial and haemodynamically stable. Abdominal examination, however, revealed purulent umbilical discharge with surrounding erythema and a tender infraumbilical mass. A working differential diagnosis of omphalitis or pilonidal disease of the umbilicus, or patent urachus was made.Bacteroides sp.Haematology showed a raised C-reactive protein of 112 mg/l, but normal white cell count. Microbiology culture of the pus grew Abdominal ultrasound scan showed a 3.8 cm echogenic collection in a cavity within the anterior abdominal wall in the midline. An MRI scan, confirmed the diagnosis of UC communicating proximally with the umbilicus . The iniThe patient was initially treated with intravenous antibiotics followed by a 2 week course of oral antibiotics with surgery planned at the end of this period. Cystoscopy and excision of the infected urachal cyst were performed simultaneously. Cystoscopy confirmed no evidence of a sinus into the bladder. A midline incision was used to excise the cyst, together with the omentum adherent to it posteriorly. The sac of the cyst contained white cheesy material . Histopath month of development into the foetal pelvis pulls the urachus with it resulting in the formation of the urachal canal. The lumen of this canal progressively obliterates during foetal life, with eventual formation of a fibrous tract in early adult life.The urachus, developmentally is the upper part of the bladder, both of which arise from the ventral part of the cloaca . DescentAt the end of development, the urachus lies between the transversalis fascia anteriorly and the peritoneum posteriorly (space of Retzius), surrounded by loose areolar tissue and attaches the umbilicus to the dome of the bladder. Histologically, it is composed of 3 layers; an innermost layer of modified transitional epithelium similar to the urothelium, the middle layer of fibroconnective tissue and outermost layer of smooth muscle continuous with the detrusor ,3.There are five types of urachal abnormalities: 1) patent urachus, in which the entire tubular structure fails to close; 2) urachal cyst, in which both ends of the canal close leaving an open central portion; 3) urachal sinus, which drains proximally into the umbilicus; 4) vesicourachal diverticulum, where the distal communication to the bladder persists; and 5) alternating sinus, which can drain to either bladder or umbilicus.Escherichia coli, Enterococcus faecium, Klebsiella pneumonia, Proteus, Streptococcus viridans and Fusobacterium [Bacteroides sp was cultured.The incidence of UC in adults is unknown but it is rare. It is more common in men than women ,4. In a acterium . In our The clinical signs and symptoms are non-specific, as UC are largely asymptomatic until they become infected. However, the presence of the triad of symptoms including a tender midline infraumbilical mass, umbilical discharge and sepsis should arouse suspicion of UC. If left untreated, UC slowly enlarges and may drain through the umbilicus as was seen in our patient, or drain into the bladder or both, resulting in alternating sinus.Ultrasound scan can help to make diagnosis in 77% of patients . In our UC can be complicated by rupture into the peritoneal cavity leading to peritonitis. Other reported complications include uracho-colonic fistula, stone formation and neoplastic transformation -9. The rAlthough histologically, the innermost layer of the urachus is mainly transitional cell, adenocarcinoma is the predominant histological type and most are mucinous. This is probably due to metaplasia arising from chronic inflammation.The treatment of choice for urachal cyst is by complete primary excision. An earlier report suggests a single stage procedure backed with appropriate antibiotic therapy for the treatment of infected UC . HoweverUrachal anomalies are rare in adults. Presentation is atypical; therefore, a high index of suspicion is required in order to achieve a diagnosis. A triad of lower midline mass, umbilical discharge and sepsis is suggestive, although MRI confirms the diagnosis and defines the surrounding anatomical relationship. Complete surgical excision is the treatment of choice due to the risk of malignant transformation. We recommend a 2 stage treatment with a combination of broad spectrum antibiotics or incision and drainage, followed by interval excision after resolution of sepsis.


Extradural spinal arachnoid cysts are rare lesions, which may become symptomatic due to mass effect. Multiple cysts are even rarer of which few are reported to date. A 17-year-old male with acute onset urinary retention and progressive paraparesis is presented. Magnetic resonance imaging of spine revealed multiple spinal extradural arachnoid cysts located dorsal to the spinal cord, causing mass effect. The patient underwent surgery for excision of the cyst and closure of dural defects. He gained urinary continence and near normal muscle strength of lower extremities over a period of two weeks following operation. Up to date, there have been only sixteen reported cases of multiple spinal extradural arachnoid cysts in the literature and the present case appears to be the second most extensive one reported so far. Appreciation of the rarity of such lesions as well as the importance of surgical planning  is highlighted. The term “spinal arachnoid cyst” is a relatively rare entity among spinal lesions. According to some authors, extra-dural cysts are less common than intra-dural ones , but thiA 17-year-old Caucasian male of Iranian nationality was visited at our institution complaining of forced urination and hesitancy since last two weeks which evolved to frank urinary retention on admission day 2. One month before presentation, he developed progressive weakness of both lower limbs. Neurological examination revealed the following: spastic paraparesis ; sensory level with hypesthesia to pinprick, light touch, and loss of vibration sense up to T2 level. A T4 to L5 laminectomy (sparing the T5 lamina) was performed disclosing multiple extra-dural cystic lesions. They were filled with CSF-like fluid. The cysts communicated with the subarachnoid spaces along the dural sleeves of the following nerve roots: (1) right T4, (2) left T6, (3) left T10, (4) left T11, (5) left L2. The cysts were excised and their ostia were closed. Closure was enforced in some points with muscle flaps.Microscopic examination of the specimens revealed fibrocollagenous tissue with an inner layer of arachnoid. The early postoperative course passed uneventfully. The patient regained urinary continence and was ambulatory with 4+/5 lower extremity forces over a period of two weeks following operation. The patient was symptom free during 12-month period of follow-up. Yet, kyphosis, a complication of the procedure, evolved and progressed during the follow up period which was controlled by brace and its progression ceased.Spinal arachnoid cysts were first described by Spiller in 1903 , althougth decade of life. This may be due to relatively smaller diameter of spinal canal in thoracic region. Lower thoracic, higher thoracic, and thoraco-lumbar regions are involved in order of reducing frequency [Regarding the reported case so far, multiple extra-dural spinal arachnoid cysts seem to occur with higher frequency in females . The pearequency ,4-8. FeaWith the advent of new diagnostic modalities and using state-of-the-art technologies, diagnosis of these complex lesions is not a dilemma at present. Formerly, plain X-ray studies and myelography led to clues for detection of the cysts. Plain radiographs have not been useful in the diagnosis of spinal cysts except for implicit signs such as enlarged spinal canal, bony erosions of the spine including eroded pedicles and posterior elements, slender pedicles, widened foramina, increased inter-peduncular space, and posterior vertebral body and laminar scalloping, and kypho-scoliosis  3-4,10--10-11.MRI is the technique of choice for both diagnosis and delineation of the cyst dimensions. Identification of a sharp interface between the cyst and subarachnoid space related to the cyst wall and the surrounding dura mater, particularly on T2-weighted MR images, allows easy diagnosis of an extra-dural arachnoid cyst . MRI canOne important aspect of preoperative evaluation is to determine the size and location of the dural defect, since it facilitates its repair via minimal laminectomy . HoweverTotal cyst excision, obliteration of the communicating pedicle, and repair of the dural flaw is the treatment of choice of the symptomatic lesions. Postoperative kyphosis may be prevented by performing laminoplasty rather than laminectomy . NeverthMultiple extra-dural arachnoid cysts are rare lesions. To the best of our knowledge, only sixteen extra-dural variants of these lesions are reported previously in the literature. The present case is the second most extensive case of multiple cysts reported to date. Reasonable addressing such pathology requires correct and comprehensive preoperative evaluation, along with a logical operative approach chosen. Since laminectomy and laminoplasty (in multiple levels) might lead to post-operative spinal deformity, it seems that the best approach would be exact determination of the communication site between the cyst and the subarachnoid space, which is currently suggested to be done through Myelography with and without CT . Althoug


A seventy-year-old male presented with severe myasthenia gravis and an episode of obscure bleeding. There was a history of gastric ulcer leading to Billroth II surgery twenty-five years ago. Upper endoscopy revealed no pathology. Colonoscopy showed a few solitary diverticula and traces of old blood in the terminal ileum. Capsule endoscopy pictured red smear in the upper jejunum. Diverticula were seen as well. Suspecting bleeding jejunal diverticulosis double balloon enteroscopy was performed. The complete jejunal ascending loop and about 100 cm of the jejunum through the descending jejunal loop could be inspected. Large diverticula with fecoliths were found in both loops. Bleeding had ceased. The patient was discharged to neurology for optimizing therapy for myasthenia gravis. Diverticulosis of the big bowelis a common disorder in elderly patients with a high percentage of clinicalsymptoms and complications. Contrary to this, diverticulosis of the small bowelis considered a rare clinically silent disease.We present a case with extensive diverticulosis of the small bowel anddiscuss it in the light of recent literature.A seventy-year-old male presented with an episode ofobscure bleeding. There was a history of gastric ulcer leading to Billroth IIsurgery twenty-five years ago. No medication affecting blood coagulation wastaken. The patient suffered from a severe myasthenia gravis that was treatedwith pyridostigmine 360 mg. Extensive examination had been done to exclude neoplasticdisease. Family history was negative for any neuromuscular or gastrointestinaldisorders, for example, inflammatorybowel disease or diverticulosis. No medication affecting blood coagulation wastaken. Upper endoscopy revealed no pathology. Colonoscopy showed a few solitarydiverticula and traces of old blood in the terminal ileum. Random biopsies didnot show any pathology; especially no histological signs of inflammatory boweldisease were present.Capsule endoscopy was indicated. The patient requiredmechanical ventilation and could not swallow the Pillcam capsule. So a flexibletube constructed for the Endocinch system was inserted in the oesophagus . The PilLittle is known about diverticulosis of the smallbowel. The prevalence of the disorder on autopsy ranges 0.06–1.3%. Thedisorder seems to be mostly acquired, but two families with extensive jejunaldiverticulosis have been published in 1988 and 2007 [Diverticula can occur anywhere in the small intestine,but they are the most common in the jejunum. Jejunal diverticulosis isassociated with many diseases, for example, scleroderma, celiac disease, Fabrydisease, and Cronkhite-Canada syndrome. An association with myasthenia gravishas not been described so far. Myasthenia gravis has been associated with inflammatorybowel disease , 4 and wUsually the disorder is clinically silent.Complications requiring intervention—perforation, bleeding diverticulitis, orintestinal obstruction—occur in 8–30% of patients . Until 1Bleeding from diverticula of the small bowel may bedifficult to localize. The available diagnostic tools are intraoperativeenteroscopy, angiography, CT-angiography, multidetector-row CT enteroclysis,wireless capsule enteroscopy, and double balloon enteroscopy.Visceral angiography is commonly used for identifyingthe active gastrointestinal bleeding site. A bleeding rate of 0.5 mL/min. isrequired for diagnosis . CompariWireless capsule enteroscopy is the instrument ofchoice not only for the overt but also for the obscure gastrointestinalbleeding with a detection rate of the source of bleeding of an average 67% , 15. ComDiverticulosis of the small bowel is a rare diseasewith a potential for the development of bleeding. Optimal diagnostic tools forthis situation should be able to show not only the mucosa but also the vesselsand the surrounding structures. CT angiography would fulfil these criteriathough it is less sensitive as wireless capsule enteroscopy.


Echinococcosis, or hydatid disease, is endemic in some regions of the world, and has been a common pathology of surgical wards in Kosovo. Primary hydatid cyst of the gallbladder is an unusual and very rare localization of hydatid disease. So far, only five cases that fulfill the criteria of primary gallbladder hydatidosis have been published in the English medical literature.We report a case of a 39-year-old Kosovan Albanian woman referred to the Abdominal Surgery Division of the University Clinical Center of Kosovo for "a calcified hydatid cyst of the liver with gallbladder involvement". Her history was significant for chronic right upper quadrant pain, characterized as intermittently colicky pain, accompanied by nausea. The patient underwent right subcostal laparotomy. Intra-operatively, a calcified primary hydatid cyst of the gallbladder was found. Its pericyst was tightly attached to the liver. Complete pericystectomy with cholecystectomy followed. The histopathology confirmed the presence of calcified hydatid cyst of the gallbladder, and that the cyst had developed entirely extra-mucosally. Five year follow-up showed no recurrence of disease.Primary hydatid cyst of the gallbladder is a very rare clinical entity. Accurate preoperative diagnostic localization is not always easy, particularly in centers with limited diagnostic tools. E granulosus is the most common presentation and probably accounts for more than 95% of the estimated 2-3 million annual worldwide cases [Hydatid disease is a zoonotic infection caused by larval stages of dog tapeworms belonging to the genus Echinococcus (family taeniidae) and is also referred to as echinococcosis . Three bde cases . Hydatidde cases . The livde cases  and the et al. (2004), Rigaset al. (1979); Waniet al. (2005), and Razaet al. (2003) [Primary hydatid cyst of the gallbladder is an extremely rare entity -8. Theret al. 200, and Raz. (2003) ,11. The A 39 year old Kosovan Albanian woman was referred to the Division of Abdominal Surgery at the University Clinical Center of Kosovo for an 18-month history of chronic (intermittently colicky) pain in the right upper quadrant, often accompanied by nausea. There was no history of jaundice. She had been treated by her primary care physician with antispasmodics, H2 receptor blockers and antibiotics, without resolution. On admission, physical examination showed no abnormal abdominal findings except mild tenderness in the right upper quadrant. Routine blood tests such as CBC, renal and liver panel proved unremarkable. Chest x-ray showed no signs of cardio-respiratory disease. Plain radiograph of the abdomen showed a calcified opacity at the level L2-L3 vertebrae on the right. The diagnosis, supported by ultrasound and computed tomography, was a calcified hydatid cyst of the liver with involvement and deformation of the gallbladder; the architecture and the size of biliary ducts were normal. The patient underwent right subcostal laparotomy. Intra-operatively, a calcified primary hydatid cyst of the fundus and body of the gallbladder was found with its pericyst attached to the liver Figure . The patEchinococcus granulosus and alveolar hydatidosis caused by Echinococcus multilocularis. The main species pathogenic for humans in Mediterranean and Southern European countries is Echinococcus granulosus [Hydatid disease is a common clinical pathology in many parts of the world. There are two clinical forms of this disease: cystic hydatidosis caused by anulosus . There hanulosus . Infectianulosus -7,12.et al. (1994) [et al. (2003) [Unusual locations of hydatid cysts support the hypothesis that beside portal circulation, the echinococcus embryos can spread via other routes, such as the lymphatic system ,8,12, th. (1994)  and Raza. 2003)  found cy. (2003)  reported. (2003) -8,13. Ma  found cPain, midabdominal discomfort and dyspepsia were the main symptoms in all reported cases of primary hydatid cyst of the gallbladder. Neither jaundice nor anaphylactic reaction were noted in any cases -7. ChronSurgery is the preferred treatment for hydatid disease. The goal is the eradication of the parasite without spillage of the cyst content. In liver hydatididosis, complete pericystectomy is not always possible, and therefore partial perycystectomy is the most frequent surgical approach ,4. SuccePrimary hydatid cyst of the gallbladder is a very rare clinical entity. Accurate site diagnosis was not made pre but intra-operatively. In our experience of 241 patients treated surgically for liver hydatididosis, only one patient (0.4%) was found to have primary gallbladder hydatid cyst. Careful pericystectomy with cholecystectomy is the procedure of choice for radical excision of primary hydatid cysts. Compare to liver hydatidosis, the gallbladder primary hydatid cyst has different spread routs of parasite embryos and a better prognosis due to earlier manifestation of symptoms leading to earlier treatment.CBC: Complete blood count; US: Ultrasound; CT: Computerized tomography.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.AK and DL performed the surgery and paper writing. LGL performed the histological examination of the specimen. GS conducted anesthesia and searched the literature. ID helped with conservative therapy and paper writing.All authors have read and approved the final manuscript.


Primary ovarian angiosarcoma is a very rare gynaecological sarcoma, with poor prognosis. These tumors are though to arise from carcinosarcomas, teratomas, or the ovarian vasculature and occur at any age. There are only a few cases reported in the international literature, most commonly associated to surface epithelial-stromal or germ cell tumours. Herein, the authors report the clinicopathologic features of an angiosarcoma arising in an ovarian fibroma. A 65-year-old patient was admitted with a palpable mass in the hypogastrium. Grossly, the removed ovary was completely replaced by a solid tumor mass. On histological analysis, the lesion revealed the typical histological features of angiosarcoma with sinusoidal patterns and anaplastic cells, admixed with spindle-shaped cells arranged in fascicles or in a storiform pattern, compatible with a fibroma. The vascular component was strongly immunopositive for CD31 and CD34. The patient was submitted to chemotherapy, and she was alive for two months after surgical proceedings. Mesenchymal neoplasms of the ovary include all primary ovarian neoplasms of connective tissue origin, and not of teratomatous or surface epithelial-stromal origin. This mode of origin cannot be excluded in a number of cases in view of the possibility of one-sided differentiation of a teratoma or of a carcinosarcoma of the ovary. Mesenchymal neoplasms of the ovary can be benign or malignant and are classified on the basis of their line of differentiation. This uncommon group of neoplasms is not specific to the ovary, determining difficult problems in diagnosis, histogenesis, behavior, and therapy .Angiosarcoma is a very rare malignant mesenchymal ovarian neoplasm. The tumor is usually unilateral, with uncertain histogenesis. It may originate from the vascular tissue present in the ovary or from a teratoma in which the vascular component has overgrown the other parts of the tumor. Patients usually have symptoms related to the presence of a lower abdominal mass, which may be associated with torsion of the tumor and hemorrhage –5. Fibroma is the most common ovarian neoplasms of connective tissue origin and constitutes 3%–5% of ovarian neoplasms. The histogenesis of ovarian fibroma is controversial. The neoplasm most likely arises from mesenchymal cells of the ovarian stroma, which differentiate in the fibroblastic direction. Ovarian fibroma is bilateral in 4%–8% of patients and multiple in 10% of cases. Patients with ovarian fibroma frequently are asymptomatic, or they manifest with abdominal pain, urinary symptoms, and ascite. Fibroma of the ovary is a benign neoplasm, with excellent prognosis, and the treatment of choice is excision of the affected ovary , 7. The authors report a case of angiosarcoma arising in an ovarian fibroma, describing morphologic and immunohistochemical findings and diagnostic criteria of these two distinct neoplasms.A 65-year-old female black patient was admitted with heaviness sensation in the hypogastrium for a few months. She had not any other complaint. Physical examination revealed a palpable abdominal mass in the lower abdomen. Abdominal computed tomography revealed a massive intraperitoneal lesion, extending from the pelvis to the level of L2, determining compression on small bowel and inferior vena cava and measuring 23.0 × 22.0 × 12.0 cm. With the hypothesis of ovarian neoplasm, an exploratory laparotomy was performed. A massive ascites was found (about five liters) and a brownish ovoid tumor in the topography of the right ovary, with loose adhesions in the omental and small bowel segments.The surgical specimen consisted of uterus, ovaries, tubes, greater omental, and peritoneal biopsies of the diaphragm and parietocolic gutter, previously fixed in formalin. The right ovary weighed 2825.0 g and measured 23.0 × 18.0 × 9.0 cm. On a cut section, the parenchyma was replaced by a yellowish-gray, frosted, swirled and firm tumor, with reddened areas in the middle . At micr The morphology of ovarian lesions associated with the findings of the immunohistochemical study was consistent with angiosarcoma associated with ovarian fibroma. In the remainder of the surgical specimen, intramural, subserosal and submucosal uterine body leiomyomas, endometrial polyp, and squamous cervical intraepithelial neoplasia grade 3 (CIN 3) were identified. The surface of the albuginea was compromised by the neoplasm. Ectocervical margin was free of dysplasia. After two months of clinical followup, the patient had no evidence of disease progression, being evaluated by the oncology team and underwent chemotherapy regimen.Pure soft tissue sarcomas of somatic type rarely occur as primary tumors of the ovary. They tipically present as a rapidly enlarging adnexal mass. Their histological appearance is similar to soft tissue tumors in other locations. In some cases, sarcomas arise as a component of a complex ovarian tumor such as carcinosarcoma, adenosarcoma, immature teratoma, or dermoid cyst or from heterologous elements in a Sertoli-Leydig cell tumor. Rare cases of primary ovarian angiosarcoma have been reported. Ovarian sarcomas may be associated with surface epithelial-stromal tumors, particularly serous and mucinous tumors, the so-called mural nodules. These tumors must be distinguished from metastatic sarcoma to the ovary –5, 8. InAngiosarcoma is a very rare ovarian neoplasm that recapitulates many of the functional and morphologic features of normal endothelium. Although the histogenesis of the tumor is uncertain, it may originate from the vascular tissue present in the ovary. It may vary from highly differentiated tumors that resemble hemangiomas to those in which anaplasia makes them difficult to distinguish from carcinomas. In general, angiosarcoma is a highly aggressive cutaneous tumors sometimes associated with lymphedema. Less than one quarter presents as a deep soft tissue mass , 9, 10. Microscopically, angiosarcomas are composed of vascular spaces of varying size and appearance, lined by endothelial cells that are usually large, showing atypical appearance, bizarre nuclei, and mitotic activity. In some areas, the tumor may contain a considerable amount of connective tissue interspersed between the vascular spaces. Some tumors exhibit a solid pattern. The tumor invades locally and metastasizes via the bloodstream. Angiosarcoma arising in the ovary must be distinguished from immature teratomatous neoplasms with a prominent vascular component. The presence of other neoplastic germ cell elements distinguishes teratoma from primary angiosarcoma. Ovarian angiosarcoma may to be distinguished too from lymphangiosarcoma, yolk salk tumor, and leiomyosarcoma on the basis of histological appearance and immunohistochemistry. Prognosis is poor, especially in patients who have metastases at the time of presentation. The tumor has not responded to combination chemotherapy regimens. Angiosarcomas usually express the usual vascular antigens including von Willebrand factor, CD31, and CD34. Cytokeratin is expressed in about one third of soft tissue angiosarcomas. Immunopositivity to actin and laminin can be found too , 13–17. Fibromas account for 4% of all ovarian tumors, and it is by far the most common sex cord-stromal tumor. They are most common in middle age and are rare in children, except for those in patients with the basal cell nevus syndrome, in whom the tumors are almost always bilateral, multinodular, and calcified. Clinically, patients with ovarian fibroma frequently are asymptomatic, mainly because of the tumor's small size. When symptoms do occur, they manifest with abdominal enlargement, urinary symptoms, and abdominal pain. Fibromas average 6 cm in diameter and are tipically uniformly solid, firm, white neoplasms. Hemorrhage, necrosis, calcification, and cyst formation may be seen. Fibromas over 10 cm in diameter are associated with ascites in up 40% of cases and Meig's syndrome  in about 1% of cases , 18.The ovarian fibroma is composed of spindle-shaped cells with uniform, bland nuclei and scant cytoplasm that may contain small amounts of lipid or eosinophilic droplets. The cells are arranged in fascicles or in a storiform pattern. Mitoses are absent or rare. The tumor is sparsely to moderately cellular with abundant intercellular collagen, hyalinized plaques, and variable degrees of edema. In some cases, fibromas can be cellular and mitotically active. Small, irregular nests or tubules of sex cord cells are occasionally found in the stroma. The sex cord cells are polygonal and have uniform nuclei and small amounts of cytoplasm. They resemble indifferent sex cord cells or granulose cells. The absence of fat differentiates a fibroma from a thecoma, but it cannot distinguish between a fibroma and a completely fibrosed thecoma. Massive edema of the ovary and fibromatosis may resemble an edematous fibroma. Fibromas express vimentin and may be immunoreactive for alpha-inhibin, and it is adequately treated by surgical excision , 18–20.The authors emphasize the rarity of primary malignant mesenchymal neoplasms of the ovary, especially the cases of angiosarcoma, which most often are associated with teratomas or epithelial/surface tumors of the ovary. To the best of our knowledge, the reported case is peculiar morphological finding that has not been previously reported yet.


Cutaneous horn is characterized by the accumulation of abnormal keratinized material and may occur in association with a variety of benign, premalignant, and malignant cutaneous lesions. Cutaneous horn occurs very rarely in association with soft-tissue neoplasias. A cutaneous horn located on the toe was completely removed by excision in a 78-year-old male patient. Macroscopic examination revealed a hemorrhagic nodular lesion, 0.5 cm in diameter, located on the dermis underlying the cutaneous horn with a height of 1 cm. Histopathological examination revealed a neoplastic lesion consisting of fusiform cells and extravasated erythrocytes underlying the compact keratin mass. The immunohistochemical analysis showed immunoexpression of endothelial markers and HHV8 in fusiform cells. The case was evaluated as “cutaneous horn developed in a nodular stage Kaposi's sarcoma.” Our case is the second case of cutaneous horn related to Kaposi's sarcoma reported in the English literature and is presented in this case report with its clinical and histopathological features. Cutaneous horns (cornu cutaneum) are chronic, dense hyperkeratotic cutaneous protrusions that often resemble the horn of an animal. Cutaneous horns are found in the upper parts of the body such as face, neck, and shoulders and are frequently related to actinic damages . Even thCutaneous horn occurs very rarely in association with soft-tissue neoplasias . In thisA 78-year-old male patient with a diagnosis of KS who has been followed for three years presented with a rapidly growing, brown, horn-like lesion between the second and third toes of his right foot that appeared 2 months prior. The hyperkeratotic lesion that was 0,5 cm in diameter and 1 cm in length had been totally removed by excision with a clinical prediagnosis of cornu cutaneum. Three years before the current diagnosis, the patient underwent excision of pink-to-purple, slightly protuberant lesions on the plantar surface of the left foot and on the pretibial area. After the histopathological examination a KS diagnosis in patch and nodule stage was given. HIV test (enzyme-linked immunosorbent assay and Western blot) was negative. The patient's family history remained insignificant; however the personal past medical history revealed that the patient had been treated for hypertension and insulin-dependant diabetes mellitus. The patient did not receive radiotherapy or chemotherapy during the period he had been followed for KS and his routine biochemical examination was unremarkable. He had got only cutaneous involvement by KS; mucosal and systemic disease had not been detected.  Histopathological examination revealed a nodular tumour tissue under the hyperkeratotic epidermis, separated from surrounding tissue with irregular borders, consisting of fusiform cells, extravasated erythrocytes, and irregular vascular structures. Rare atypical mitosis and cytoplasmic hyaline globules were noted in the tumor. A cutaneous horn structure composed of compact keratin was observed on the epidermis at the tumour surface Figures . The immCutaneous horn is a morphologic designation referring to cohesive keratinized material protuberant above the surface of the skin but not a true pathologic entity. The pathogenesis of this abnormal formation of keratinized material has not been fully elucidated. It may be of clinical importance because the underlying condition may be a malignant lesion. It is difficult to define the underlying lesion, especially in superficial biopsies. Therefore, deep (punch) biopsies or total excision of small lesions are recommended to be certain, and a specimen from the lesion under the keratin layer is obtained –3. CutanTo the best of our knowledge, there is only one previous report of cutaneous horn related to Kaposi's sarcoma in the literature . Histolo Histological features including acanthosis and papillomatosis typical for hyperkeratotic KS are not present in the epidermis, and the horn structure is clinically visible at the surface of the lesion. Another feature of our case is that the cutaneous horn is located on the toe, which is an unexpected area for these types of lesions. This suggests that trauma might be a predisposing factor for the development of cutaneous horn in areas that are not exposed to actinic damage. In addition, the advanced age and gender of our case support the role of the age and sex in the aetiology of cutaneous horn development, as reported in the literature. In conclusion, although it is rare, cutaneous horn may be associated with soft-tissue neoplasms. Adequate and appropriate surgical sampling is important for the determination of the underlying lesion.


A pancreatic sarcoma of nerve sheath origin is reported in a 28-year-old female patient, who presentedwith melaena. Preoperative imaging showed an 8.5 cm diameter mass in the head of pancreas. Therewas bleeding from the papilla of Vater at endoscopy and a highly vascular lesion on arteriography. Thepatient was submitted to proximal pancreatoduodenectomy and remains symptom-free at 1 year follow-up.


A 3-year-old girl presented with a hemorrhagic conjunctival lesion in the right eye. The medical history revealed premature cessation of breast feeding, intolerance to the ingestion of baby foods, anorexia, and abdominal distention. Prior to her referral, endoscopic small intestinal biopsy had been carried out under general anesthesia with a possible diagnosis of Celiac Disease (CD). Her parents did not want their child to undergo general anesthesia for the second time for the excisional biopsy. We decided to follow the patient until all systemic investigations were concluded. In evaluation, the case was diagnosed with CD and the conjunctival tumor showed complete regression during gluten-free dietary treatment. The clinical fleshy appearance of the lesion with spider-like vascular extensions and subconjunctival hemorrhagic spots, possible association with an acquired immune system dysfunction due to CD, and spontaneous regression by a gluten-free diet led us to make a presumed diagnosis of conjunctival Kaposi sarcoma. Celiac disease (CD) is an autoimmune gluten-induced enteropathy that typically causes intestinal malabsorption syndrome in childhood. The clinical course may be complicated by extra-intestinal immunological disorders such as dermatitis herpetiformis, cutaneous vasculitis, polyarteritis, glomerulonephritis, chronic liver disease, polymyositis, and myocarditis. AlthoughA 3-year-old girl presented with a complaint of hemorrhagic tear episodes of 3 months duration in the right eye. Her parents stated that the reddish conjunctival lesion was detected elsewhere one month ago and was unresponsive to topical steroids, and hence, excisional biopsy was suggested. The patient was referred to our clinic to get a second opinion.Her past medical history revealed premature cessation of breast feeding, intolerance to the ingestion of baby foods, anorexia, and abdominal distention since 2 months. Her weight and height percentiles were subnormal compared to her age group. From 26 months of age, she had recurrent serous otitis media treated with systemic antibiotics. However, the primary etiology could not be determined by her pediatrician in the first 3 years of life.Our initial visit showed that the visual acuities were 20/20 in both eyes. Slit-lamp examination of the right eye revealed a reddish, elevated, and highly vascular spider-like lesion on the superior bulbar conjunctiva, measuring 12×4×2 mm . Our preThe blood test for HIV antibody was negative. Serology showed high anti-gliadin and anti-endomysial immunoglobulin A antibody levels. Endoscopic intestinal biopsy demonstrated partial villous atrophy, intraepithelial lymphocytosis, and crypt hyperplasia consistent with CD. Genetic testing of the family members revealed high maternal autoantibody titers for CD.After the diagnosis of CD, gluten-free diet was instituted. The conjunctival lesion gradually regressed  and disaCD is an autoimmune small bowel enteropathy due to an immune-mediated hypersensitivity to gluten. It has b4Ocular involvement in CD has been reported in a very few number of case reports.–4 To ourIn our case, the differential diagnoses of such a conjunctival lesion includes KS, subconjunctival hemorrhage, malignant melanoma, squamous cell carcinoma, pyogenic granuloma, cavernous hemangioma, lymphoma, caroticocavernous fistula, foreign body granuloma, and lymphangioma. We did nAcquired defects of the immune system have been found in patients with CD and KS. Thus, concurrent presence of both disorders may be observed. There is only one case report that presented histopathology-proven visceral KS in a 54-year-old man with CD.In conclusion, we present a very unusual conjunctival tumor in a patient with CD that showed complete regression by a gluten-free diet. The precise pathological nature of this conjunctival lesion remains unknown due to the lack of histopathological confirmation. However, prompt regression of the conjunctival lesion during gluten-free diet suggests a possible relationship to CD and an autoimmune process.


Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a rare variant of uterine sarcomas, associated with postoperative recurrence, metastases and a fatal outcome. The mean age at diagnosis is 54.5 years. A 37-year-old nullipara presented with irregular vaginal bleeding, a normal pelvic examination, and an initially negative ultrasound. Repeat ultrasound one month later revealed an 11-cm heterogeneous pelvic mass. She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Pathology confirmed uterine MASO. Computed tomography 2 weeks postoperatively showed a huge mass compatible with recurrence. Patient died 2 weeks later. MASO is rarely diagnosed in women in their 4th decade. This case stresses that these aggressive tumors should be considered in the differential of patients with vaginal bleeding and pelvic masses irrespective of their age. Uterine sarcomas generally account for less than 4% of all uterine malignancies with the subtype adenosarcoma comprising only 8% of cases, reflecting the rarity of this entity . MulleriA 37-year-old nullipara, previously healthy, presented with a 3-month history of irregular heavy menstrual bleeding. Ultrasound of the pelvis showed normal pelvic structures and the absence of any masses. Shortly afterwards, she started to report dull left lower quadrant abdominal pain, increased abdominal girth, and malodorous greenish vaginal discharge. A repeat ultrasound performed a month later showed a huge amount of abdominal ascites and a heterogeneous formation measuring ~11 cm in the pelvis. Liver, pancreas, kidney, spleen and bladder were normal. Abdominal tap was done and bloody fluid was aspirated. Cytology of the fluid showed numerous malignant cells. A computed tomography (CT) of the abdomen and pelvis revealed a big heterogeneous tumor in the pelvis with thickening and irregularity of the peritoneum.The patient underwent a total abdominal hysterectomy with bilateral salpingooophorectomy and omentectomy. A 3-kg tumor floating in the peritoneum was evacuated. The tumor revealed abundant surface ulceration associated with necrosis.Histology of the polypoid mass revealed a biphasic tumor composed of benign epithelial elements and a sarcomatous stroma Figure . Most ofA panel of immunohistochemical stains, including smooth muscle actin (SMA), muscle specific actin (MSA), desmin, S-100, CD10, multiCK, epithelial membrane antigen (EMA), and vimentin were performed and cells were positive for MSA and vimentin. The findings were consistent with MASO. The sarcoma was of the undifferentiated endometrial stromal type with rhabdomyosarcomatous differentiation. Two weeks later, the patient's abdomen increased in size again. Ultrasound of the abdomen showed a huge mass, and tumor recurrence was considered. Spiral CT revealed extensive peritoneal carcinomatosis. Patient died after 2 weeks of disease recurrence before adjuvant therapy could be administered.Mullerian adenosarcomas of the uterus rarely present in premenopausal women. It is even more unusual to diagnose the rare variant MASO in this age group with a reported mean age at diagnosis of 54.5 years . The mosThese tumors can present as a pelvic mass 37%), uterine polyp (22%), or an enlarged uterus (22%) . Pain, f%, uterinA review by Krivak et al revealed 37 cases of MASO described in the literature up to 2001 . It may ASSO are considered high grade, have a high mitotic rate, and are DNA aneuploid with an S-phase fraction >10% . The facCornerstone of therapy of uterine sarcomas remains surgery. Total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) can be curative if disease was confined to the uterus with five-year survival approaching 50% . AdjuvanThe rarity of such tumors in young age groups makes the clinical suspicion very low, resulting in delay in making the diagnosis, and with highly proliferative tumors, rate of complications is expected to increase as seen in our patient who presented with extensive peritoneal metastasis. Whether a repeat ultrasound performed at a shorter interval would have detected the tumor at an earlier stage before it grows and spreads is to be considered. In addition, due to the rarity of adenosarcomas there is no consensus regarding therapy. A study dealing with the time of initiation of postoperative adjuvant therapy in relation to the interval to tumor recurrence is further needed to outline recommendations regarding promptness of adjuvant therapy and its effect on survival, tumor progression, and disease free period.In conclusion, gynecologists, pathologists, and oncologists should be aware of the consequences associated with a delay in the diagnosis and/or initiation of therapy for Mullerian adenosarcoma with sarcomatous overgrowth. Although rare, this entity should be kept in the differential of young women presenting with abnormal menstrual bleeding in the presence of a pelvic mass.The author(s) declare that they have no competing interests.MF: data collection, literature review, paper writing.EH: literature review, paper writing.GM: pathology reading and revision, paper writing.AN: literature review, paper editing.All authors revised and approved the final draft of the manuscript.


Most patients with brain metastases from malignant melanoma are diagnosed after treatment for known extracranial metastases and have a poor outcome despite various local and systemic therapeutic approaches. Here we discuss an unusual case where a 45-year old patient presented with a brain metastasis as the first symptom of disease and where the presumed primary lesion later was found in the gastro-intestinal tract. Treatment consisted of sequential surgical removal of a total of 4 tumor sites , whole-brain radiotherapy and two radiosurgery procedures within 13 months. Following her last treatment, the patient has now been in remission for 20 months. This case illustrates that some patients with multi-organ melanoma manifestations may benefit from the repeated use of effective local therapeutic approaches and may experience a quite favourable prognosis. Malignant melanoma is next to lung cancer, the most frequent cause of brain metastasis . These mThe patient is a 45-year old caucasian female without serious previous disease or family history of cancer. In October 2004, she had noted a few days of hypesthesia in her left leg, followed by slight hemiparesis and a seizure resulting in hospitalisation. A magnetic resonance imaging (MRI) scan of the brain revealed a tumor in the right parietal lobe, presumably representing a glioma Figure . In NoveStaging including examinations of the eyes, head and neck mucosa and total skin, gynecological evaluation, bone scintigraphy and computed tomography (CT) scans showed an enlarged left adrenal gland as the only pathological finding Figure . All rouAfter a symptom-free interval, routine MR evaluation in November 2005 disclosed tumor progression of the temporal lesion and a second gamma-knife procedure was performed. Since then, the patient returned to repeated follow-up examinations including MR and CT scans. The last one was performed in July 2007. As shown in Figure Neurological symptoms as the first sign of malignant melanoma are relatively uncommon, as is the inability to identify the primary tumor in patients with brain metastases from this disease . The patRadiotherapy plays an important role in palliative treatment in this setting. Patients with a single brain metastasis managed with surgical resection plus WBRT have a 2-year survival rate of 20–25% . The preThroughout the melanoma literature, long-term survival after complete resection of metastatic disease has been reported repeatedly ,10. DespThis case illustrates that patients with multi-organ melanoma manifestations may benefit from the repeated use of effective local therapeutic approaches and may experience a quite favourable prognosis.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(s) declare that they have no competing interests.AD and AP treated the patient and collected the data. CN and JN drafted the manuscript. All authors read and approved the final manuscript.


Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma.Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy.Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery.This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms. Malignant transformation of mature cystic teratoma (MCT) is a rare complication occurring in approximately 1–3% of patients who have mature cystic teratoma . AlthougThe patient was a 50-year-old female who presented with a 3-months history of progressive chronic low back and right hip pain. She had no symptoms of carcinoid syndrome. She had no previous history of malignancy, chemotherapy or radiotherapy. General physical exam was unremarkable. Chest radiographs and electrocardiogram were within normal limits. Her routine hemogram, urine and blood biochemical analyses were within normal ranges. A lumbar spine X-ray, done to workup her complaint of low back pain, accidentally found a large 1.9 cm) calcification overlying the lower pole of the right kidney  , calretinin , carbonic anhydrase IX , CD10 , CD56 , CD99 , CDX2 , chromogranin , cytokeratin 7 , cytokeratin 20 , pancytokeratin cocktail , smooth muscle actin , synaptophysin , and thyroid transcription factor-1 . Appropriate tissue sections that had been shown to be positive or negative for each marker were used as controls. Slides stained omitting the primary antibody were also used as negative controls.We analyzed the intensity and immunoreactivity of the immunostained sections. The intensity was graded qualitatively as weak, moderate, or strong on the basis of the brown color produced by the 3, 3'-diaminobenzidine chromogen. Diffuse and intense brown staining of the cytoplasmic or nuclear surfaces, as appropriate for each stain, was interpreted as strong staining intensity. Moderate intensity staining was characterized as non-diffuse but intense staining pattern, whereas weak intensity had a non-diffuse and non-intense staining pattern. Immunoreactivity was quantitatively estimated by the percentage of positive cells per representative section. Immunoreactivity was graded as 1+, 2+, and 3+, corresponding to less than 25%, between 25% and 50%, and greater than 50% of neoplastic cells showing positive staining per representative section, respectively.Grossly, the partial nephrectomy specimen revealed an encapsulated multiloculated gray-tan tumor (9.7 × 7.4 × 7.8 cm). The interface between tumor and uninvolved kidney was sharp. Sectioning of the tumor revealed circumscribed complex solid and multiloculated cystic lesion with large area of calcification (1.7 cm). The largest cyst measured up to 1.4 cm, and the cysts were filled with yellow-tan gelatinous material. The wall of the cysts showed focal thickened firm and hard calcified areas. The tumor was confined to the kidney and was at least 0.5 cm from the nearest stapled resection margin. There was no gross evidence of extension of the tumor into the renal pelvis, renal vein, renal artery, ureter, renal sinus, renal capsule, or perinephric adipose tissue. The entire tumor was submitted for histological examination.The histological examination revealed three components. The first component was multilocular cystic spaces Figure  lined byAlpha-methylacyl-coenzyme A-racemase (AMACR/P504S), calretinin, CD10 and thyroid transcription factor-1 (TTF-1) did not stain any of the three components of the tumor. The teratomatous cysts were lined by ciliated epithelium (strong and diffuse cytoplasmic labeling for cytokeratin 7 and pancytokeratin) and mucinous columnar enteric-type or colonic-like epithelium , beta-subunit of human chorionic gonadotropin (β-hCG), carbohydrate antigen (CA) 19-9, CA 72-4, CA 125, carcinoembryonic antigen (CEA), chromogranin and serotonin continued to remain within normal ranges. Her postsurgery urinary 5-HIAA level also continued to remain within normal range. Additionally, postsurgery whole body somatostatin receptor scintigraphy with octreotide performed was negative, hence confirming the carcinoid tumor as originating primarily in the resected renal tumor (rather than elsewhere with metastasis to the kidney) and the absence of metastases from the resected renal carcinoid tumor. Hence, the partial nephrectomy with complete resection of the tumor was considered adequate conservative clinical management. She received no adjuvant therapy. Two follow-up CT and MRI scans of the brain, abdomen, chest and pelvis, and PET scans performed at 3-month intervals after surgery revealed no lesions. The patient herein presented is alive with no evidence of local recurrence or metastatic disease, six months postoperatively, and regular periodic follow-up with interval CT, MRI and PET imaging studies, and octreotide scintigraphy is planned.Mature cystic teratomas may consist of a wide variety of ectodermal, mesodermal, and endodermal tissues, and hence the possibility of additional neoplasms occurring in teratomas, though rare, can at least be anticipated. It is not surprising that squamous cell carcinoma leads the list of secondary neoplasms arising in teratomas, because many mature cystic teratomas contain large amounts of squamous epithelium -3. In seCarcinoid tumors are characteristically low grade malignant tumors with neuroendocrine differentiation that have been described in several locations, including the gastrointestinal, respiratory, hepatobiliary, and genitourinary systems. Carcinoid tumors most commonly occur in the gastrointestinal tract 74%) and bronchial system of the lungs 25%) [% [5,7,184% and brPrimary carcinoid tumors of the kidney are rare -40, and We were not able to find any reported case of simultaneous occurrence of mature cystic teratoma, carcinoid tumor and adenocarcinoma in the same kidney, in our literature search. We have herein described a case of coexistent mature cystic teratoma, carcinoid tumor and adenocarcinoma in a horseshoe kidney in a 50-year-old female. The age of the case herein presented is close to the mean and/or median age of both previously reported patients with carcinoid tumor arising within normal kidneys (49–52 years) ,18 and cCarcinoid tumors are thought to arise from enterochromaffin cells or amine precursor uptake and decarboxylation (APUD) cells, and are widely distributed throughout the body. In the urogenital tract, APUD cells have been described in the urinary bladder , the urethra, the prostate, and the renal pelvis, but not in the renal parenchyma ,43,46,47in utero, which coalesce to form the isthmus of horseshoe kidneys [The less popular hypothesis states that the coexistence of carcinoid tumors  with congenital and acquired renal abnormalities (such as horseshoe kidney and mature cystic teratoma) are due to hyperplasia of interspersed neuroendocrine cells within metaplastic or teratomatous epithelium in horseshoe kidneys; or nests of misplaced progenitor cells developing into teratomatous intestinal or respiratory epithelia in renal teratomas, might serve as a nidus for renal carcinoid tumors and adenocarcinoma. Cases of renal carcinoid tumor arising in association with renal teratoma and/or horseshoe kidneys lend weight to this hypothesis ,24,34,37 kidneys . Additio kidneys ,15-17,20 kidneys ,20,21,38 kidneys ,43,46,47Synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature teratoma of horseshoe kidney is a unique occurrence. This is the first reported case to our knowledge of mature cystic teratoma coexisting with carcinoid tumor and adenocarinoma in the same kidney. This unique case report emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas since multiple additional histogenetically distinct small neoplasms occurring in a mature cystic teratoma could easily be missed through incomplete sectioning and histologic evaluation, potentially compromising patient care. Although longterm follow-up was lacking in the case herein presented, this unique occurrence of three neoplasms in horseshoe kidney was not associated with local recurrence and metastasis, was surgically curable, and had a rather favorable prognosis.Consent was received from the patient before publication.The authors declare that they have no competing interests.HBA participated in the histopathological evaluation, performed the literature review, acquired photomicrographs, and drafted the manuscript. AMP and AVP conceived and designed the study, gave and reviewed the final histopathological diagnosis, and revised the manuscript for important intellectual content. All authors read and approved the final manuscript.


This is a report on the use of transperineal intraoperative ultrasound imaging in a case of Currarino’s triad for the first time in the literature. The syndrome consisting of anorectal malformation, sacral bone abnormality and presacral mass is known as Currarino’s triad. Chronic constipation is the most common presenting feature of this syndrome. The sacrAt surgery, precise dissection of the presacral mass is required in view of its deep location, adherence to the rectal wall, proximity to the pelvic nerves and, often, extension into the intrathecal space. Transperineal intraoperative ultrasound (IOUS) imaging was used in a boy with Currarino’s triad to aid precise definition of the extent of the pelvic mass and its intraspinal extension. Use of IOUS for Currarino’s triad has not been reported before.A 3-year-old boy presented with abdominal distension and history of constipation, the latter since early infancy. The abdomen was distended, with palpable fecal masses, and the rectal examination revealed a stenosed anus. The abdominal radiograph showed dilated bowel loops with deformed sacrum. The patient was initially treated with stool softeners and rectal washouts. Rectal biopsy showed normal ganglion cells. Pelvic MRI showed hypoplasia of body of the fourth sacral vertebra with absence of its posterior elements and absence of whole of the fifth sacral vertebra. A presacral mass measuring about 3 cm × 3 cm × 3 cm and consisting mainly of fat with locules of fluid was seen distal to the fourth sacral vertebra with intraspinal extension .Divided transverse colostomy was performed initially and 3 months later and definitive surgery was performed through a posterior sagittal perineal approach. Transperineal IOUS imaging with a 7.5 MHz linear transducer placed within the gluteal cleft was used to define the anatomy of the pelvic mass during excision . The intHistological examination of the pelvic mass revealed a mature teratoma with clear surgical margins. MRI performed 4 months later showed no residual tumor . The colIn surgery for tumors of the liver, pancreas, biliary tree and pelvis, IOUS imaging has been used extensively for the diagnosis of impalpable lesions, to locate multiple lesions, for precise delineation of adjacent structures such as vessels and nerves and also to confirm completeness of excision. However,Transperineal IOUS guidance is a useful tool in the precise surgical management of Currarino’s triad and is easy to organize with the help of a radiologist or with some training by the surgeon concerned. Transrectal IOUS  is not feasible in most children with Currarino’s triad because of the anorectal stenosis and the relatively large size of transrectal probes currently available.


Small supernumerary marker chromosomes are still a problem in cytogenetic diagnostic and genetic counseling. This holds especially true for the rare cases with multiple small supernumerary marker chromosomes. Most such cases are reported to be clinically severely affected due to the chromosomal imbalances induced by the presence of small supernumerary marker chromosomes. Here we report the first case of a patient having four different small supernumerary marker chromosomes which, apart from slight developmental retardation in youth and non-malignant hyperpigmentation, presented no other clinical signs.Our patient was a 30-year-old Caucasian man, delivered by caesarean section because of macrosomy. At birth he presented with bilateral cryptorchidism but no other birth defects. At age of around two years he showed psychomotor delay and a bilateral convergent strabismus. Later he had slight learning difficulties, with normal social behavior and now lives an independent life as an adult. Apart from hypogenitalism, he has multiple hyperpigmented nevi all over his body, short feet with pes cavus and claw toes. At age of 30 years, cytogenetic and molecular cytogenetic analysis revealed a karyotype of 50,XY,+min(6)(:p11.1-> q11.1:),+min(8)(:p11.1->q11.1:),+min(11)(:p11.11->q11:),+min(12)(:p11.2~12->q10:), leading overall to a small partial trisomy in 12p11.1~12.1.Including this case, four single case reports are available in the literature with a karyotype 50,XN,+4mar. For prenatally detected multiple small supernumerary marker chromosomes in particular we learn from this case that such a cytogenetic condition may be correlated with a positive clinical outcome. Multiple small supernumerary marker chromosomes (sSMC) with diverse sSMC derived from different chromosomal origin are rarely reported. According to Liehr , up to nPatients with multiple sSMC constitute a sub-group of patients with sSMC ,3. LittlHere we report the first case with four sSMC derived from chromosomes 6, 8, 11 and 12, with almost no clinical signs.Our patient was a 30-year-old Spanish Caucasian man; the third child from healthy and non-consanguineous parents. The first child was a healthy boy and the second child was also a boy who died after two days due to hyaline membrane disease and prematurity. Our patient was delivered by caesarean section after 39 gestational weeks because of macrosomy, with a weight of 4250 g and an Apgar score of three, thus, intensive reanimation was required. Within five hours of life he suffered apnea. He was also hypoglycemic and hypocalcemic, but responded well to treatment without suffering a recurrence. Clinical examination showed bilateral cryptorchidism. During her pregnancy our patient's mother was treated with diazepam towards the end of the pregnancy.When our patient was 19 months old, his weight and length were two standard deviations below normal. During further development, he showed psychomotor delay and a bilateral convergent strabismus; also he started walking when he was 22 months old. At the age of 10 years, his testes were surgically descended. And at the age of 13 years the strabismus was corrected. At school he had slight learning difficulties, with normal social behavior. He later left studying to become a painter.de novo because his parents had normal karyotypes. Now, at the age of 30 years a new blood sample for cytogenetic analysis was requested. Surprisingly, the high resolution G-band karyotype attained from this sample showed the presence of a relatively big SMC, together with the presence of three additional tiny SMCs in most cells. This cytogenetic analysis revealed a karyotype of 50,XY,+mar1,+mar2,+mar3,+mar4.When he was 22 years old, he had no facial dysmorphism, he weighed 89 kg, his height was 165 cm and he had a corporal index mass of 32.7. He had hypogenitalism, with a short thick penis (6 cm), and testes of 8 and 10 cc. He has multiple hyperpigmented nevi all over his body, showing no sign of malignancy after biopsy Figure . He alsoin situ hybridization (cenM-FISH [To further characterize the sSMC centromere-specific multicolor fluorescence enM-FISH ) was carenM-FISH ,7) (FiguHere we report the fourth unusual case with four different sSMC and the 34th case with multiple sSMC. It is the eighth case with no or only minor clinical signs due to the sSMC presence. The only detectable sSMC-related chromosomal imbalance is a small partial trisomy 12p11.2~12.1. According to Liehr  there arMoreover, it is interesting that the multiple sSMC derive in the present case from chromosomes 6, 8, 11 and 12. Chromosomes 6 and 12 are over-represented in multiple sSMC cases reported to date compared to their contribution to single sSMC . This miThe present case confirms that multiple sSMC may be correlated with an almost normal clinical outcome. This is especially important for the correct genetic counseling of similar pre-natal cases. Furthermore, a small partial trisomy12p11.2~12.1 seems to correlate largely to no clinical effects. Finally, involvement of chromosome 6 in sSMC formation seems to be correlated with the tendency of multiple sSMC formation.The authors declare that they have no competing interests.LR performed the cytogenetic studies in the present case. JFT and AP collected the data relative to this case report and provided genetic counseling to the parents. MLMF supervised the cytogenetic analysis as Director of the ECEMC. EE, ABH, MZ and TL did the molecular cytogenetic analysis and interpretation. TL drafted the paper and all authors contributed to the finalizing of the 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.


We report a patient who underwent pancreaticoduodenectomy for a cystic lesion in the region of the pancreatic head and duodenum. Preoperatively, we had suspected a malignant lesion; however, it turned out to be ectopic pancreatic tissue in the duodenal wall, with the changes of chronic pancreatitis and pseudocyst formation. With this report we seek to highlight the rarity of this particular pathologic combination and the difficulties in its correct preoperative diagnosis and management. Ectopic pancreas is an uncommon condition. It is usually found in the gastrointestinal tract, with 24–38% being seen in the stomach, 9–36% in the duodenum, and 0.5–27% in the jejunum.–3 It is A 50-year-old male patient, a chronic alcoholic as well as a chronic smoker, presented with 1½ months' history of moderately severe nonradiating pain localized to the upper abdomen. He had no history of acute pancreatitis, jaundice, anorexia, weight loss, or other such illness. There was no history of any similar illness in the family. The pulse rate was 86/min, blood pressure 126/80 mm Hg, and respiratory rate 16/min. His temperature was normal, he weighed 58 kg, and his height was 165 cm. General and abdominal examination revealed no abnormalities. Standard laboratory investigations, including hemoglobin, blood counts, blood sugar, and renal and hepatic function tests, were within normal limits. Serum amylase was 32 IU/l .Abdominal ultrasonography (USG) revealed two hypoechoic lesions (25 mm × 12 mm and 39 mm × 55 mm in size) in relation to the pancreatic head region. Endoscopic USG (EUS) confirmed these findings. Abdominal contrast-enhanced computed tomography (CECT) scan of abdomen showed a mass in relation to the duodenum and the head of the pancreas. The mass had central cystic areas and there was periportal and precaval lymphadenopathy Figure  and b. Tth postoperative day; he has been under follow-up for 6 months now.Based on the CECT and FNAC findings, the patient was provisionally diagnosed as having a malignant cystic tumor of the head of the pancreas and posted for surgery. Diagnostic laparoscopy revealed no evidence of metastases. On exploration, a hard mass in the pancreatic head region was noted, with multiple lymph nodes in the vicinity. The rest of the pancreas was firm. In view of the imaging and operative findings, as well as the inability to rule out malignancy, Whipple pancreaticoduodenectomy was carried out. The patient recovered well and was discharged on the 12Histopathological examination (HPE) showed unexpected and uncommon findings Figure –c. GrossMost patients with ectopic pancreas are asymptomatic or have nonspecific symptoms, such as abdominal pain or discomfort, nausea and vomiting, gastrointestinal bleeding, etc. Symptoms may be due to the mass effect of the ectopic tissue or because of its involvement in a pathologic process that may also involve the normally placed pancreas  scan of abdomen showed a mass in relation to the duodenum and the head of the pancreas.The previously reported patient also presented with abdominal pain and was diagnosed as malignancy  on preoperative imaging; that patient too underwent pancreaticoduodenectomy. Another Cysts can form in ectopic pancreas due to retention of secretions because of the absence of communication between the glandular tissue and the bowel lumen. However, true pseudocyst formation in ectopic pancreas is rare. Cyst amyAlthough chronic pancreatitis was present in both ectopic as well as native pancreas, it was minimal in the latter. This preferential involvement of the ectopic pancreas might have been due to the absence of a draining duct, causing chronic pancreatitis by way of mechanisms similar to those occurring in obstructive chronic pancreatitis; this obstructive element was absent in the native pancreas.Duodenal wall cystic lesions can also arise from enterogenous duplication, retention cysts in Brunner glands, and cystadenomas, and thesThere are three histologic subtypes of ectopic pancreas: Type I ; type II ; and type III . The presThe management of ectopic pancreas remains controversial. It has been reported that in 61% of cases the symptoms are attributable to the ectopic pancreas itself. However,To summarize, in this article we describe a rare case of an ectopic pancreas in the duodenum, with chronic pancreatitisand pseudocyst formation. We highlight the difficulty in making a preoperative diagnosis even with the use of advanced diagnostic modalities and the limitation of FNAC in this setting.


Liposarcomas are among the most common sarcomas of adult life. Pleomorphic liposarcoma, characterized by pleomorphic lipoblasts, is the rarest subtype. To our knowledge only three cases of pleomorphic liposarcoma of the foot or ankle have been reported so far.A 71-year-old female presented with a large growing mass on the dorsum of her right foot. Computed tomography showed invasive tumorous mass. Excision biopsy revealed the mass to be a pleomorphic liposarcoma, and below the knee amputation was performed.Although the incidence of pleomorphic liposarcoma in the foot is very low, it is essential to perform thorough histological analysis of all soft tissue masses, regardless of their benign appearance, because only prompt radical surgery can result in a good prognosis for the patient. Liposarcomas are malignant tumors and among the most common soft tissue sarcomas of adult life . They caWe present a case of a pleomorphic liposarcoma of the foot, stressing necessity of a proper histological analysis, followed by radical surgery leading supposedly to a better prognosis for the patient.A 71-year-old female of normal weight presented with a large mass on the dorsum of her right foot which has been gradually increasing in size over 4 months prior to admission to the hospital. Two years before she had a soft tissue injury at the same location, but without any complication and sequels. Otherwise she was without serious illness. Her family history revealed no lipomatous disorders. On physical examination a firm, palpable tumor mass, 6–7 cm in diameter, poorly-defined from surrounding tissue and attached to underlying structures and skin, was detected. The mass was painless, unless examined by deep palpation. Skin above it was of normal color and structure. Laboratory findings, including blood count, were completely normal. On plain x-rays only an ill defined mass on the dorsum of the foot, with no clear correlation to the bone, was observed. Computed tomography revealed invasive tumorous mass. Scintigraphy with Tc99 was positive. Magnetic resonance imaging showed expansive infiltrative process of the dorsum of the foot with penetration between metatarsal bones to the plantar side, and partial destruction of the fourth metatarsal bone. Histologically, in biopsy sample, areas with lipogenic characteristics were surrounded by nonlipogenic areas Figure . In lipoAfter histological diagnosis below the knee amputation was indicated. No further therapy was indicated, and patient is still alive, 6.5 years after the diagnosis was made.l biopsies. This is especially true for dedifferentiated liposarcoma where quality of sampling represents the key parameter in avoiding underdiagnosis. Only adequate sampling will identify both well/and poorly differentiated areas of a given tumor. The distinction from nonlipogenic tumors is based on demonstration of multivacuolated lipoblasts and occasional positivity for S-100 protein in the spindle or poorly differentiated areas. The most complicated task is the differential diagnosis of tumors with highly pleomorphic (MFH-like) features. The differential diagnosis in the groups with round or pleomorphic cells, including epitheloid appearance, largely depends on specific immunohistochemistry for the given tumors. In distinction from poorly differentiated epithelial neoplasms the positivity of occasional cells for epithelial markers should be kept in mind [Pleomorphic liposarcoma is a rare neoplasm, and until know there have been only 3 reported cases of pleomorphic liposarcoma in the foot and ankle. It is known that this neoplasm is usually aggressive, occurring in adulthood, and usually in the limbs. Pleomorphic liposarcoma can occur in mediastinum, liver, orbit, paratesticular region, and also as a purely dermal tumor ,7-11 and in mind . In all Molecular studies so far yielded little useful results: in one third of the patients MDM2 amplification was detected, and TP53 alterations were present in four out of nine patients  .We presented a case of a 71-year old female, with tumorous mass on the dorsum of her right foot, which, according to pathohistological analysis, turned out to be a pleomorphic liposarcoma, showing areas of different histology. Our case demonstrates the importance of pathohistological analysis of all soft tissue tumorous masses in the foot/ankle region, which may look like common benign soft tissue lesions, but if not recognized can become life-threatening.It is important to bear in mind that the differential diagnosis of pleomorphic lyposarcoma is fairly broad and that thorough sampling and searching for lipoblasts may be rewarding in tumors firstly presenting histologically as one of the mimics .The authors declare that they have no competing interests.LB is primarily responsible for drafting, literature search and submission of the manuscript. AJ and LBV vere involved in literature search and preparing the material, DO suplyed relevant clinical information about the patient and was involved in manuscript revision. SS outlined the general concept of the manuscript, has been involved in drafting and revising it critically. All authors have read and approved the final manuscript.


Chronic expanding haematoma (CEH) is a very infrequent event with imprecise developmental mechanism and is rarely reported in literature.We present a case of enormous and spontaneous chronic haematoma of the back, expanded from the lower thoracic area to the sacral area, in a young patient without any history of trauma or chronic coagulopathy.The MRI scan is very useful in preoperative diagnosis, however only the histopathological examination is able to perform the differential diagnosis with soft tissue tumors. Careful surgical treatment is important to minimize the haematoma recurrence. Chronic expanding haematoma is a rare condition and constitutes a challenge for the physicians because of the difficulty of early and correct diagnosis. The incidence and the true mechanism of this lesion are unknown. They are supposed to be structurally identical with subdural haematomas.Many case reports have reported CEH in various anatomical regions such as: head, chest, abdominal cavity and extremities with variable sizes. Both MRI scan and histopathological examination are very useful in differential diagnosis with the soft tissue sarcomas. We present herein, a case of impressive spontaneous, chronic expanding haematoma, in a young man without history of trauma or any disease involving the coagulate system function.A 24-year-old man was presented in our department with a large, painless swelling in the lumbar region with a moderate extension to the lower thoracic and to the upper sacral area. In fact, the parents observed the last three months a protuberant mass in this region enlarging gradually. The patient initially was negative to a medical examination. The recent history was negative. The patient was an amateur soccer player, playing with a frequency of 1-2 matches per week. The recent history was unremarkable for trauma or any disease involving the liver and the coagulation tests. The recent pharmacological history for administration of anticoagulant therapy resulted negative.The inspection revealed a large mass 28 cmx10 cm) from the sacral spine to the lower thoracic region without skin changes. The lesion was soft, fluctuant and painless in palpation , sarcoma, actinomycosis and inflammatory pseudotumor ,2,5. In The final diagnosis is made by the histopathological examination. Microscopically the capsule is consisted in hyalinized fibrous tissue and a granulation tissue with new capillaries and inflammatory cells.The correct treatment for the CEH is only surgical. Aspiration of the liquid, drainage and curettage could result in serious bleeding and have a higher possibility for recurrence. Complete resection of the capsula and a meticulous suture of the subcutaneous tissue, with the underlying fascia in order to eliminate the dead space are highly recommended ,6,9.We conclude, that a chronic expanding haematoma could occur spontaneously or may be caused by a minor or not appreciable trauma. MRI scan is very important in the preoperative diagnosis and the histopathological examination is crucial in the differential diagnosis with the soft tissue sarcomas. A careful preoperative plan and a meticulous surgical treatment could limit considerably the possibility for recurrence.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.All authors read and approved the final manuscript.DP, AB and PK initiated and co-wrote the paper and performed the surgical treatment of the haematoma. KA performed the proof editing. EL examined the specimen and prepared the histological illustrations.


Primary tumors of seminal vesicles are rare and only a few cases have been reported. Diagnosis is difficult due to the absence of early clinical signs. Prognosis is generally poor.We present the case of a 70-year-old Caucasian man with a seminal vesicle mass and concomitant lymph node metastasis detected by computed tomography and body positron emission tomography/low-dose computed tomography scan carried out for evaluation of Lambert Eaton syndrome. Transrectal ultrasound-guided biopsy showed a poorly differented neuroendocrine carcinoma with an immunhistochemical profile similar to small cell lung cancer. Following chemotherapy the disease was stable and active surveillance was initiated.Lambert Eaton syndrome may be the initial symptom of a seminal vesicle mass. Diagnosis needs to be obtained by transrectal biopsy and chemotherapy may delay progression of the tumor. Primary tumors of seminal vesicles are extremely rare. A total of 51 documented cases of seminal vesicle carcinoma in men between the ages of 19 and 90 years old have been reported in the literature . EpithelSeminal vesicle neoplasms are often difficult to diagnose, generally presenting as a retrovesical mass that can be detected by digital rectal examination and transrectal ultrasound. However, in roughly 30% of patients no abnormalities on digital rectal examination are detected due to concomitant benign prostatic hyperplasia obscuring the seminal vesicle tumor, or to location of the tumor in the seminal vesicles are found . In addiet al. state that seminal vesicle carcinoma can run a rapidly fatal course or possess potential for cure [Prognosis of patients with a seminal vesicle tumor is generally poor. Early diagnosis may result in long-term palliation or even cure. To date, no histopathological prognostic factor could be identified and the estimate of prognosis is challenging. Smith for cure . SurgicaA 70-year-old Caucasian man was transferred to our department from the Department of Neurology where he was an inpatient due to Lambert Eaton myasthenic syndrome (LEMS).LEMS is an autoimmune disease of the neuromuscular junction, characterized by proximal muscle weakness, areflexia and autonomic dysfunction. Antibodies are found, directed against P/Q-type voltage gated calcium channels in the pre-synaptic nerve terminal . LEMS isLambert, Eaton, and Rooke in 1956 described a myasthenic syndrome in association with lung cancer . There aIn our case muscle weakness, an atactic gait, diplopic images, dysphagia and ptosis were present. In retrospective view of our patient, the first symptoms of LEMS were already present two and a half years earlier. At that time our patient did not undergo medical examination and no further diagnostic workup was initiated.After presenting at the Department of Neurology other possible causes of LEMS were ruled out; internal investigation was performed with findings of a hypercholesterolemia. The medical history of our patient contained a transurethral resection of the prostate gland three years prior to treatment in our institution with unsuspicious histological findings showing a chronic prostatitis and benign hyperplasia. Additionally, our patient was a smoker of 60 pack-years. There was no evidence of autoimmune disease in the medical history. Currently, no lower urinary tract symptoms and no loss of weight or loss of appetite were described. The clinical and ultrasound examinations were normal.Subsequent radiologic evaluation by CT scan of the cranium, thorax and abdomen did not show any sign of malignant disease.One year previously our patient was first sent to our department for urogenital tumor search. No suspicious findings were detected on digital rectal examination, like the whole clinical and ultrasound examination of the urogenital tract. PSA level was 0.8 ng/mL. To complete the diagnosis a 10 core biopsy of the prostate gland was performed that showed chronic prostatitis and benign hyperplasia.Whole body positron emission tomography (PET)/low-dose CT scan Figure  revealedFollowing the imaging results another transrectal ultrasound-guided 10 core biopsy of the suspicious right seminal vesicle and the right prostate gland was performed.The histological findings of the biopsy of the right seminal vesicle showed a poorly differentiated neuroendocrine carcinoma with immunohistochemistry similar to SCLC. The immunohistochemistry was negative for PSA, positive for chromogranin A, synaptophysin and CD99. Transcription termination factor TTF-1 was positive in about 60% of the tumor cells. A biopsy of the right prostate gland showed chronic prostatitis and a benign hyperplasia.Having discussed all treatment options, our patient underwent six cycles of chemotherapy with carboplatin (520 mg) and etoposide (210 mg) at the Department of Hemato-oncology. Additionally pyridostigmine bromide 60 mg twice daily and 90 mg during the night for the myasthenic symptoms were initiated. With this medication the pronounced weakness that he initially presented with declined.Following six cycles of chemotherapy, our patient presented again to our department for re-evaluation. Staging was completed by a PET-CT scan Figure , a CT scThe suspicious, enlarged para-aortal and para-iliacal lymph nodes were stable. There was no evidence of new metastases. A PET-CT scan showed a declining accumulation in projection to the described para-aortal and para-iliacal lymph nodes on both sides. Also a declining accumulation in projection to the seminal vesicle was described. The transrectal ultrasound of the prostate and the seminal vesicles detected a hypoechoic 2.0 × 1.7 cm tumor in the right seminal vesicle. The biological marker neuron-specific enolase declined from 72 μg/L to 22 μg/L.In cooperation with the Department of Hemato-oncology, we decided a strategy of observation. Our patient will re-present for re-evaluation for a two month follow-up. In case of impairment of the clinical symptoms, monotherapy with etoposide would be initiated.Primary tumors of seminal vesicles are rare. In the present case a poorly differented neuroendocrine carcinoma, a very rare entity, was confirmed by histopathology. While this entity is known for sporadic primary tumors of the prostate gland, cases of seminal vesicle involvement are reported. Our patient presented with symptoms of LEMS therefore a complete neurological, internal and radiological investigation was carried out. Autoimmune diseases could be excluded along with other neoplasms. CT and PET-CT scan showed a tumor of the right seminal vesicle as an irregular circumscribed mass. Numerous lymph nodes suspicious for metastasis were detected. Taking imaging results of the CT scans and the PET-CT scans with the results of a mass in the right seminal vesicle and ultrasound and subsequent biopsy findings into consideration, a primary neuroendocrine carcinoma of the seminal vesicle was diagnosed.The aim of treatment of primary seminal vesicles is curative radical surgery prior to any infiltration of neighboring organs or even a metastatic disease. However, hormonal manipulation and radiotherapy seem to be effective as adjuvant treatment modalities . Due to While neuroendocrine carcinomas of the lung have been described in association with LEMS, the present case is the first description of this entity deriving from seminal vesicles. Therapy of poorly differented neuroendocrine carcinoma is equivalent to therapy of SCLC. Chemotherapy with carboplatin and etoposide is an accepted treatment of choice for patients with advanced SCLC . AlternaSeminal vesicle tumors are rare. To the best of our knowledge, we present the first case of a patient with poorly differentiated neuroendocrine carcinoma of the seminal vesicle diagnosed due to his presentation with LEMS. The prognosis of a neuroendocrine carcinoma with lymph node metastases is poor. In our case chemotherapy with carboplatin and etoposide led to stable disease, which was followed up by active surveillance.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.BK and WO drafted the case report, SD, RG, HMF and MB helped to draft the report. WFW supervised drafting the report.


We report a 12-year-old-boy with gastric lipoma. Upper gastrointestinal (GI) endoscopy with biopsy and abdominal computed tomogram (CT) scan revealed the diagnosis. Open surgical excision of the mass with stomach preservation was done. The clinical presentation and management are discussed and the literature reviewed here. This is the sixth pediatric case reported in the English literature. Benign gastric lipomas (GL) are rare in children; even in adult population, it represents only 2% of all gastric tumors. They areA 12-year-old boy presented with the complaints of active hematemesis and malena of 1 week duration. He had a history of similar episode 6 months back during which he was treated with multiple blood transfusions. He also complained of a vague lump in the upper abdomen since 6 months. On examination, he was extremely pale with a firm mobile mass palpable in the upper abdomen. Laboratory investigations revealed hemoglobin of 3.6 g%. The coagulation profile was within the normal range. An ultrasound of the abdomen showed a large lobulated intraluminal mass in the stomach, following which a contrast-enhanced computed tomogram (CECT) scan of abdomen was done. CT showed a large ovoid intraluminal mass of fat attenuation with strands of soft tissue component involving the body and antral region of the stomach . The posGastrointestinal (GI) lipomas are tumors of mature adipose tissue, surrounded by a fibrous capsule. GL constReview of literature for GL reveled only five such cases in children. Stefan reportedThe radiological investigations and the endoscopic findings complement each other in the diagnosis of GL. CT is specific for lipomas located anywhere in the body; it shows a homogenous structure with a negative HU value, regular shape, no infiltrative growth, and sometimes a fibrous capsule. It permits a specific diagnosis on the basis of fat density of the tumor. Magnetic13Surgical resection is the mainstay of management of symptomatic large tumors. Endoscopic polypectomy has gained consensus on safety and efficacy for submucosal lesions smaller than 3 cm. Larger s


Primary peritoneal cystadenocarcinoma is a rare tumor of similar histogenic origin as primary ovarian carcinoma. We present a case of primary peritoneal serous cystadenocarcinoma mimicking advanced colorectal cancer in a 68 yr-old African American female. Radiology, endoscopy and cytology yielded only inconclusive findings. Immunohistochemical analysis of percutaneously obtained ascitic fluid provided a correct diagnosis of primary peritoneal cystadenocarcinoma. The discovery of serous ascites at the time of laparotomy confirmed a diagnosis of primary peritoneal serous cystadenocarcinoma. Final surgical pathology reconfirmed the diagnosis of primary peritoneal cystadenocarcinoma. This case demonstrates the utility of immunohistochemistry for accurately diagnosing patients with inconclusive findings in the setting of peritoneal carcinomatosis and primary peritoneal cystadenocarcinoma. Primary peritoneal cystadenocarcinoma is a rare tumor . OriginaBoth primary ovarian carcinoma and primary peritoneal cystadenocarcinoma can present with carcinomatosis. Clinical presentation results from local tumor effects involving multiple organs . Common Management of primary peritoneal cystadenocarcinoma has followed treatment of primary ovarian carcinoma with surgical debulking and adjuvant platinum-containing chemotherapy . SurvivaPrimary peritoneal cystadenocarcinoma and primary ovarian carcinoma both stain positive for estrogen receptor (ER), cytokeratin 7 (CK7), Wilm's tumor suppressor gene (WT1), and cancer antigen 125 (CA 125). Neither entity possesses cytokeratin 20 (CK 20), progesterone receptor (PR), Calretinin, carcinoembryonic antigen (CEA), gross cystic disease fluid protein (BRST-2), and thyroid transcription factor 1 (TTF1). Cytochemical overlap can occur between different celltypes but the constellation of positive and negative antigenicity produces a unique immunohistochemical "fingerprint" that identifies cellular origin.The patient was a 68 yr-old African American female who presented to the emergency department of Henry Ford Hospital with complaints of shortness of breath and abdominal distension. Her past medical history was significant for asthma, Type II diabetes mellitus, hypertension, gastroesophageal reflux, adenomatous colonic polyps and breast cancer. Abdominal ascites and a right pleural effusion were present. Ultrasound imaging (US) of the abdomen and pelvis was normal except for the known ascites. Computed tomography (CT) of the abdomen and pelvis demonstrated moderate ascites, a right pleural effusion and omental thickening. US and CT imaging failed to demonstrate any masses. Magnetic resonance imaging of the abdomen and pelvis revealed pelvic peritoneal masses suspicious for metastatic implants and a 4.1 centimeters mass in the appendix. Colonoscopy was performed to the cecum. At 50 centimeters proximal to the anal verge, a 4 centimeters subserosal non-circumferential partially occluding mass was discovered. The mass was biopsied. The remainder of the exam was normal. Consideration was given to an endoscopically concealed appendiceal cystadenocarcinoma or primary appendiceal colonic adenocarcinoma. Pathology reported the endoscopic tissue biopsy as displaying a moderately well differentiated adenocarcinoma with a papillary growth pattern  declare that they have no competing interests.WV (Chief Resident/HOV) directly assembled patient information, directed further in depth analysis of the known literature, performed the operative interventions and managed the recovery of the patient, reviewed and directed several drafts of the manuscript, and was responsible for the final construction of the manuscript for submission to the senior staff/primary investigator.SJ (MSIV) acquired patient information, researched the known literature, participated in the patient's surgery and recovery, and constructed the manuscript under guidance. In this capacity she directly contributed to the conceptualization, design and production of this manuscript. Her final manuscript was then submitted to senior members for final approval.DK (HOIII) acquired patient information, instructed the junior investigator (SJ) in conceptualization and construction of the manuscript.ZZ (Pathologist) processed, evaluated and diagnosed the submitted surgical specimens. She also constructed the pathology narrative for the final manuscript.VV (Senior Staff) served as the senior staff reviewer/primary investigator on this case report. He initially conceived the possibility of alternative diagnoses, arranged patient testing, scheduled and immediately directed the surgical interventions, supervised the patient's recovery and follow-up. He was responsible for final approval of the submitted manuscript following directed revision.Written informed consent was obtained from the patient for publication of the study.