The general consensus is that appendicitis is basically provoked by fecaliths or lymphoid hyperplasic obstruction. Several studies based on histological diagnosis have not confirmed this hypothesis. On the contrary, obstruction has been proved in only a minority of cases. Diverse infections by parasites, bacteria, fungus, and noninfective agents have been associated with appendicitis in the medical literature. We describe a firefighter, who ingested a small quantity of leachate from decomposing corpses while working and developed enteritis a few hours later, which lasted several days and evolved to appendicitis. This case raises the possibility that the high quantity of bacteria concentration present in the leachate could have provoked enteritis and the subsequent appendicitis due to a direct effect of the bacteria on the appendix. Common sense leads us to believe that appendicitis is mainly provoked by obstruction of the appendix lumen due to fecaliths or lymphoid hyperplasia, followed by secondary bacterial invasion of the wall. Numerous studies and reports based on histologic diagnosis have not confirmed this hypothesis, and obstruction of the appendix lumen as cause of appendicitis has been proven in only a minority of cases . In factThe patient was a 27-year-old, athletic, Caucasian male, with no previous health problems and a firefighter in the city of Guarulhos, within the metropolitan area of São Paulo, Brazil. In March 2004, his mission was to explore a 20 m deep clandestine cesspool that was used to hide corpses of the victims of a local criminal gang. There were at least five advanced decomposed corpses at the bottom of the cesspool. He descended into the cesspool to retrieve the corpses, wearing special clothing and a mask to protect him from biological contamination from the decomposing corpuses. He spent about 30 minutes working inside the cesspool. Strong rain occurred while performing the task and his mask and his special cloths were damaged, and his body and his mask were covered in contaminated leachate from the decomposing corpuses. This situation led him accidentally swallow a small quantity of this leachate mixed with rain water while he was doing his job. Upon completion of the mission, he was dispensed from duty. About four hours later, he started to feel very sick and presented vomiting, diffuse abdominal pain, important diarrhea, and prostration. After 36 hours, the vomiting and the diarrhea had ceased, but abdominal distension manifested in association with constipation, nausea, loss of appetite, and diffuse abdominal pain. Sometimes, the level of abdominal pain increased to the point where he was obliged to seek medical assistance at the emergency unit for administration of analgesics and antiemetics. He never presented fever. This scenario lasted about 13 days when the diarrhea returned associated with fecaloid vomiting. Two days later the diarrhea ceased, the constipation restarted, flatus ceased, and the diffuse abdominal pain evolved into mesogastric pain. The vomiting became uncontrollable. He was hospitalized and submitted to an exploratory laparotomy, during which he was diagnosed with retrocecal appendicitis associated with intra-abdominal abscess and adhesions and was submitted to surgery to remove the same. The anatomopathological exam verified suppurative acute appendicitis and periappendicitis, with no evidence of fecaliths. Microscopic examination revealed neutrophilic infiltration of the mucosa, submucosa, and muscularis associated with areas of ulceration of the mucosa and abscess in the appendix wall presenting necrosis. He was drained and treated with antibiotics . After surgery, he began to feel progressive abdominal pain again, distension, constipation, and no fever. These symptoms got worse, and after 14 days he underwent a second surgery during which an intra-abdominal abscess and peritonitis were observed. One month later, he presented intestinal obstruction and abdominal pain and was submitted to a third operation due to another intra-abdominal abscess and adhesion. One year later, he was again submitted to surgery due to intestinal occlusion and incisional hernia. Six months after that, he underwent a fifth surgery for abdominal adhesion and intestinal occlusion. After the first surgery, he began to suffer chronic constipation that worsened after each successive surgery. Nowadays, he is only able to defecate after enteroclysis, to which he must be submitted every five days. Salmonella , Yersinia [Bacteriodes [Campylobacter [Shigella [Clostridium difficile with appendicitis [Salmonella infection could cause appendicitis by direct invasion of the appendix without the initial obstructive component. This bacterium colonizes the appendix mucosa and submucosa, provoking abscess and phlegmon [Yersinia characteristically causes granulomatous appendicitis, which may or not be associated with enterocolitis and mesenteric adenitis [Campylobacter jejuni is limited to the mucosa and submucosa, lacking transmural suppuration. Campbell evaluated 50 archival cases of acute appendicitis using molecular detection of Campylobacter jejuni and detected Campylobacter jejuni DNA in a significant percentage (22%) of acute appendicitis cases [Clostridium difficile infection is very rare, and the pathological findings are similar to pseudomembranous colitis [Actinomyces is a gram-positive, anaerobic, gut commensal that causes infection and predominantly neutrophilic inflammation of the appendix [Clostridium perfringens, enterobacteriaceae, streptococci, micrococcaceae, staphylococci, Corynebacterium, Bacterioides, Pseudomonas, Alcaligenes, Achromobacter, and occasionally bacilli [What is most remarkable in this case is the fact that only a few hours after the patient had ingested a contaminated leachate solution, an illness suggestive of bacterial gastroenteritis initiated that lasted 13 days and evolved to appendicitis. This could be considered a coincidence, but the fact that a coherent nexus exists linking the moment of contamination, the gastroenteritis, and the appendicitis is undeniable. Another interesting aspect is no fecaliths were detected in the appendix, and there is no proof that his appendicitis was provoked by fecalith obstruction. This report leads us to an intriguing possibility that ingestion of a high concentration of bacteria could provoke appendicitis. Specific bacterial infection may cause appendicitis, with or without involvement of the surrounding bowel . The medYersinia , Bacteriteriodes , Campylolobacter , 6, ShigShigella , and Clondicitis , 9. Somephlegmon . Yersiniadenitis . Acute iis cases . Appendircentage 2% of acu colitis , 9. Acti bacilli . Many ofAnother possible explanation for the patient's appendicitis is related to enteric lymphoid proliferation. Intestinal mucus is a fundamental and complex immunological organ. The entire intestine and appendix mucus presents an important lymphoid tissue that constantly reacts to antigens on the intestinal lumen. Some researchers believe the appendix has an important immunological role . BacteriMoreover, Yildiz and Bulut reported a possible lymphoid hyperplasia appendicitis triggered by a barium enema performed 15 days prior to its manifestation [After the surgery, the recurrence of intra-abdominal abscesses and adherences may reflect the high virulence intensity of the infection and the inflammatory process that the patient suffered. The sequelae developed due to multiple surgeries, included an important chronic constipation, probably the result of adherences, and dysfunction of intestinal peristalsis.Another interesting aspect of this case is the fact that the patient's appendicitis was provoked by accidental ingestion of a substance with a high bacterial concentration while working; a situation that could be characterized as an occupational disease. We believe that this could be the first case report of appendicitis related to an occupational cause.


Oncocytic carcinoma is a rare proliferation of cytomorphologically malignant oncocytes mainly found in glandular tissue, accounting for 0.5% of all epithelial salivary gland malignancies and 0.18% of all epithelial salivary gland tumors.We report a case of oncocytic carcinoma arising in the parotid gland of a 65-year-old Caucasian man. Our patient initially underwent left superficial parotidectomy, including the removal of the mass. A close follow-up was made, and four years after first surgery cervical lymph node metastases were confirmed. Therefore, a complete parotidectomy and radical neck dissections were performed. There were no complications and no sign of recurrence after six months of follow-up.Oncocytic carcinoma is an extremely rare malignancy in the salivary glands. Prophylactic neck dissection may be indicated for tumors larger than 2 cm in diameter (our patient's tumor was 2.5 cm at its greatest diameter). The clinical course of our patient, with the appearance of cervical lymph node metastases after four years of follow-up, supports this approach. Further investigation of the prognosis and correct treatment of patients with oncocytic carcinoma are required as more cases are reported. Oncocytic carcinoma (OC) is an unusual proliferation of cytomorphologically malignant oncocytes and adenocarcinomatous architecture phenotypes mainly found in glandular tissue . The terThis tumor represents 5% of all oncocytic salivary gland neoplasms and less than 1% of all salivary gland tumors . OC may In this case report we describe a case of OC that arose in our patient's parotid gland and presented regional lymph node metastases four years after first surgery.A 65-year-old Caucasian man presented with a one-year history of a painless mass in the left parotid gland. Physical examination revealed an elastic, movable and non-tender mass. There was no facial palsy or regional lymphadenopathy. A computed tomography (CT) scan revealed a 20×25 mm mass in the anterior portion of the gland, near the parotid duct, resembling a mixed tumor Figure . The cerA month later, our patient underwent left superficial parotidectomy, including the removal of the mass. Because of clear infiltration of the tumor, the buccal branch of the facial nerve was resected. Macroscopically, the tumor was a well-circumscribed, firm, grey-brown, ovoid nodule measuring 2.5 cm in diameter. Microscopic examination revealed that the neoplasm had replaced a wide area of the parotid gland and peri-neural invasion was found Figure . The neoWe preferred a close follow-up to an elective neck dissection, and reserved the neck dissection for a recurrence, as there was no lymph node involvement either clinically or radiologically. Our patient was followed up accordingly, and four years after surgery started complaining of swelling and pain in the left cervical area. Physical examination revealed an elastically hard and poorly defined left cervical mass. There were no signs of local recurrence. FNAC showed oncocytes and atypical cells. A CT scan demonstrated several enlarged lymph nodes in the left parotid gland and on the left side of the neck Figure . TherefoOC is an extremely rare malignancy in salivary glands, accounting for just 0.5% of all epithelial salivary gland malignancies and 0.18% of all epithelial salivary gland tumors . This tuApproximately one-third of patients with oncocytic carcinoma of the parotid develop a painful mass or experience facial paralysis. The skin overlying the gland is occasionally discolored or wrinkled . In our As with other high-grade salivary gland neoplasms, aggressive surgical intervention is indicated. Therapy for OCs consists of surgical intervention, and total parotidectomy with preservation of the facial nerve whenever possible. The efficacy of radiotherapy is unclear. Radiation does not appear to favorably alter the biological behavior of this tumor. In the case reported, intra-operative frozen sections of the tumor would have been indicated for pathological diagnosis and would have altered the surgical decision. FNAC alone did not determine a correct diagnosis and the first surgical management was inadequate.et al. published a review of 28 cases of oncocytic carcinoma of the parotid gland [A number of studies have reported multiple recurrences of this tumor and regional or distant metastases ,6,8-10. Prophylactic neck dissection may be indicated for tumors larger than 2 cm in diameter  (our patThe prognosis of oncocytic carcinomas is not well known because of their low incidence. Patients with malignant oncocytoma appear to have good short-term survival, but poor long-term survival . The aveOC is an extremely rare malignancy in salivary glands, and standard treatment and prognosis is unclear. Clinicians should perform a careful follow-up, as distant metastasis appeared to be the most important prognostic feature.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.LG was a major contributor to the writing the manuscript. LG-C was the main surgeon, reviewed our patient's notes, collected the data and prepared the radiology slides. ED performed the histological diagnosis and photographs. NC and LJ reviewed and corrected the manuscript. All authors read and approved the final manuscript.


Endometrial stromal tumor is a rare mesenchymal uterine tumor. We report the case of a patient with endometrial stromal sarcoma and concomitant bilateral endometrioid adenocarcinoma of the ovary in the context of pelvic endometriosis. The patient underwent a complete cytoreduction including total hysterectomy and bilateral adnexectomy, pelvic lymphadenectomy, appendicectomy, infracolic omentectomy, and pelvic peritonectomy. This is the first report to our knowledge that describes a synchronous endometrial stromal sarcoma and bilateral endometrioid adenocarcinoma of the ovary. Endometrial stromal sarcoma (ESS) accounts for 0.2% of all malignant tumors of the female genital tract . The meaEarly on, endometrial stromal sarcoma (ESS) was subdivided into low grade and high grade categories mainly according to the mitotic count , 3.In cuVarious hypothetical risk factors observed for ESS are pelvic irradiation and long-term tamoxifen therapy . SporadiEndometriosis is a common benign, estrogen dependent, chronic gynecological disorder associated with pelvic pain and infertility. It is characterized by the presence and growth of endometrial glands and stromal outside the uterine cavity –10. Prior studies have found an increased overall cancer incidence in women with endometriosis , 12 and Despite being benign, the endometrium in endometriosis behaves like a tumoral tissue expression of growth factors involved in tumor proliferation, invasiveness, angiogenesis, lack of response to the mechanisms of normal regulation, susceptibility of relapse after complete excision, and ability to invade adjacent tissue and distant locations all being documented in endometriosis , 14, 15.Endometriosis has mixed traits of benignity and malignancy. The pathogenesis involves loss of control of cell proliferation and it is associated with local and distant spread. Although endometriosis cannot be termed a premalignant condition, epidemiologic, histopathologic, and molecular data suggest that endometriosis has a malignant potential .Unlike typical ovarian cancers, those that arise from endometriosis have discrete features: they are more commonly constituted by clear cell and endometrioid subtypes, tend to be found in earlier stages, and have a favorable prognosis. Due to these unique characteristics, ovarian cancers derived from the malignant transformation of endometriosis have been specifically referred to as endometriosis-associated ovarian cancer (EAOC) –18.We present a rare case involving a patient with endometrial stromal sarcoma and concomitant bilateral endometrioid adenocarcinoma of the ovary.A 43-year-old patient was sent from another institution presenting an adnexal mass and elevated tumor markers: cancer antigen 125 (CA 125)-5232 IU/mL; CA 19.9-468 IU/mL; CA 15.3-910 IU/mL IU/mL, CEA-6.9 ng/mL; alpha-fetoprotein 1.9 ng/mL. Her relevant medical history included epilepsy and two recent previous episodes of deep venous thromboembolism. She was a gravid 3 para 1, with two spontaneous first trimester miscarriages. No previous surgeries were known.The abdominal exam showed a hypogastric pain and a hard-to-define mass. Gynecological exam revealed a hard-to-delimitate uterus, a painful posterior vaginal wall, and enlarged adnexal regions. Vaginal ultrasound was performed and revealed a slight uterine enlargement with an image of a nodular interstitial formation measuring 32 × 31 mm, an enlarged right ovary (84 × 62 mm) containing a cystic image with heterogeneous filling, and an enlarged left ovary also with central cavitation. The chest scans showed a large pleural effusion .The patient was submitted to thoracentesis (with drainage of 1500 cc of fluid) and the cytological analysis revealed inflammatory exudates without neoplastic cells. Esophagoduodenoscopy and colonoscopy revealed no abnormalities. Pap smear, mammography, and breast ultrasound were normal. Abdominopelvic computed tomography (CT)  showed aThe patient underwent total hysterectomy and bilateral adnexectomy, pelvic lymphadenectomy, appendicectomy, infra-colic omentectomy, and pelvic peritonectomy. During the laparotomy, the patient was transfused with two units of packed red blood cells. Both ovaries were transformed to solid tumor masses 10 cm long, there were peritoneal implants less than 1 cm scattered throughout the pelvic excavation and epiplon, and the uterus was enlarged and hard. The postoperative period was uneventful, and the patient was discharged on the fifth day without complications.The pathology report stated that the left ovary was transformed to a cystic formation; it weighed 350 g and measured 12 × 10 × 10 cm. The external surface was lobulated and predominantly bright pink. There was a tumoral-looking area of infiltration of the capsule extending along 1 cm. Upon sectioning, there were areas of friable edematous aspect. The right ovary was transformed into a cystic formation with no content coated with a smooth surface. The internal surface showed multiple vegetations of yellow softened tissue measuring 0.2 to 2 cm.Microscopically the left ovary exhibited clumps of cells, architecturally of cribiform endometrioid type. Tumoral cells had hyperchromatic nuclei with moderate atypia, with solid areas where there was a more pronounced pleomorphism. The tumor infiltrated adjacent ovarian parenchyma with ovarian capsule disruption. Microscopically, the right ovary presented endometrioid glandular structures with moderate pleomorphism, hyperchromatic nuclei, and an increased nucleus/cytoplasm ratio. On the surface, there were solid areas with cells featuring marked nuclear pleomorphism. In the transition to the nontumoral epithelial tissue, there was an endometrial-type epithelium with little underlying stromal and reactive modifications.The uterus weighed 200 g and measured 9 cm in height, presented a fasciculate myometrium with a thickness ranging from 1 to 3 cm: in its posterior wall, a 4-cm hard nodular structure with ill-defined limits was identified. Microscopically the nodular formation exhibited a high cellular density and a solid pattern. The large cells presented a high nucleus/cytoplasm ratio. The nucleus showed slight atypia. The pattern of growth was mainly expansive, infiltrating the adjacent myometrium with vascular embolization. The immunohistochemical features found in the histology specimen of the uterus included reactivity to antibodies specific for vimentin and negativity for epithelial markers cytokeratin (CK7) and pankeratin MNF116, smooth muscle actin, desmin, HMB45, CD68, and calponin. The presence of vascular embolization was confirmed through positive immunoreactivity to factor VIII and CD34. Estrogen, progesterone receptors, and Bcl2 were positive. Immunoreactivity to CD10 was not found, and external advice was sought which confirmed the negative reaction to CD10. The index of cell proliferation (Ki67) was 10%.The pathologic study revealed bilateral endometrioid adenocarcinoma of the ovary, moderately differentiated , showingThe patient subsequently underwent chemotherapy with six cycles of paclitaxel and carboplatin. A clinical suspicion of a vaginal fistula prevented treatment with vaginal brachytherapy (28 Gy/4F). A clinical follow-up at six months and a CT scan 1 year after surgery revealed no adenopathy or peritoneal effusion; the value of CA 125 was 19 IU/mL. Currently, the patient is under hormonal therapy with tamoxifen.To the best of our knowledge, this is the first report that describes a synchronous ESS and bilateral endometrioid adenocarcinoma of the ovary. Several studies have documented synchronous bilateral ovary and endometrial cancer but until now none reported a synchronous ESS.The frequency of malignant transformation of endometriosis is unknown, but it is estimated that up to 1% of women with endometriosis will develop an endometriosis-associated neoplasm , 19–21. Several laboratory and clinical data suggest that ESS are hormonally sensitive , 21, 22.Although CD10 immunoreactivity is a well-known positive predictive marker of ESS, there are exceptional cases when CD10 is negative, namely, in fibrous variants .Surgery has always been described as the most effective treatment for uterine sarcomas. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is considered to be the standard treatment for ESS , 25, 26.The options of adjuvant therapy following surgery include radiotherapy, chemotherapy, and hormonal therapy , 30. AdjProlonged survival and even cure are common after surgical resection of recurrent or metastatic lesions .These tumors have usually an indolent clinical course with 80–100% 5-year survival, but about 37–60% of patients eventually recur after a very long time and 15–25% die of the disease , 35. ProPatients with a uterine sarcoma should be treated within well-designed, randomized clinical trials, that are difficult to be conducted because of the rarity of such malignancies . It is p


IgH gene was detected by molecular analysis. Evaluating a splenectomy sample in cases like this may present a diagnostic challenge to pathologists. Therefore, correlation with B cell clonality studies , clinical findings and peripheral blood morphology searching for characteristic binucleated lymphocytes is essential to avoid misdiagnosing this benign process as B-cell lymphoma. We also present here a literature review on pathogenesis of PPBL.Persistent polyclonal B-cell lymphocytosis (PPBL) is rare and intriguing hematological disorder predominantly reported in young to middle- aged smoking women. It is characterized by persistent moderate polyclonal B-cell lymphocytosis with circulating hallmark binucleated lymphocytes and elevated polyclonal serum IgM. Most patients have benign clinical course on long-term follow-up. Some pathologic features of PPBL may resemble malignant lymphoma, including morphology as well as frequent cytogenetic and molecular abnormalities. Significant symptomatic splenomegaly requiring splenectomy is very unusual for this disorder; therefore there is a lack of descriptions of the morphologic features of the spleen in the literature. We present here one of the first detailed descriptions of the morphologic and immunohistochemical features of the spleen from a young female with PPBL who developed massive splenomegaly during 6-year follow up. Splenectomy was performed for symptomatic relief and suspicion of malignant process. The morphological and immunohistochemical features of the spleen closely mimicked involvement by B-cell lymphoma, however there was no monotypic surface light chain restriction seen by flow cytometry and no clonal rearrangement of http://www.diagnosticpathology.diagnomx.eu/vs/5329558967545656The virtual slide(s) for this article can be found here:  Very recently, Italian investigators Del Giudice et al. described similar histopathological findings of the spleen in three patients with PPBL and progressive splenomegaly who underwent splenectomy [BCL2/IG gene rearrangements as seen in some lymphomas [Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare clinically benign lymphoproliferative disorder first described by Gordon et al. in 1982 . Most paymphomas . To avoi9/L with peripheral blood smears showing few atypical binucleated lymphocytes. Flow cytometry of her peripheral blood revealed an increase in polytypic CD19+/ CD20+/ CD5-/ CD10- B cells. Serum protein electrophoresis showed elevated polyclonal IgM at 9.6 g/L. The patient was diagnosed with PPBL and was closely monitored by her family physician and local oncologist. Over the following 6 years, she received no medical intervention. Several months prior to her splenectomy, she started complaining of increasing abdominal discomfort. Her spleen slowly increased in size and reached over 8 cm below left costal margin. She had no hepatomegaly or lymphadenopathy on CT imaging of the neck, chest, abdomen and pelvis. Her CBC remained unchanged with persistent peripheral lymphocytosis varying between 10 to 13 x 109/L and showing variable numbers of characteristic binucleated lymphocytes. Repeated flow cytometry of peripheral blood just prior to splenectomy showed persistent increase in polytypic B cells. She subsequently underwent splenectomy for symptomatic relief as well as for suspicion of “malignant transformation”. Patient’s postoperative course was unremarkable requiring no further therapy. The patient continued heavy smoking throughout her years of follow-up.The 38-year-old woman with a 20 years history of heavy smoking of 1 ½ packages of cigarettes per day was referred to QEII Health Science Centre, Halifax, Nova Scotia about 6 years ago for investigation of fatigue, frequent colds and persistent lymphocytosis for over 6 months. Her past medical history was unremarkable. The only positive finding on her physical examination was mild splenomegaly with spleen palpable 4 cm below left costal margin. Her initial complete blood cell count (CBC) showed isolated lymphocytosis of 10.2 x 10Formalin-fixed, paraffin-embedded tissue sections were stained with hematoxylin and eosin (H&E) and Periodic Acid-Schiff (PAS). Immunohistochemistry was performed on 4-μm-thick sections prepared from formalin-fixed, paraffin-embedded tissue using an automated immunostainer . The antigenic determinants and probes tested are listed in table Mononuclear cells from fresh peripheral blood were separated by density-gradient centrifugation and were characterized using four-color immunostaining by a FACSCalibur flow cytometer  and Cell Quest software . The following monoclonal antibodies were used: CD45-allophycocyanin (APC), CD5-phycoerythrin (PE), CD19-peridinin cholorophyll protein (PerCP), CD20-fluorescin isothiocyanate (FITC), CD22-FITC, CD23-FITC, CD38-PE, CD11c-PE, CD10-PE, FMC7-FITC, anti-kappa chain-FITC, anti-lambda chain-PE. CD23 and FMC7 were obtained from Beckman Coulter , and the rest of the monoclonal antibodies were purchased from Becton, Dickinson and Company.BCL2/IG gene rearrangement targeting the usual breakpoint regions of the t.DNA from paraffin-embedded spleen sections was extracted using the commercially available DNA Blood Mini Kit . B cell clonality was assessed using nested PCR with consensus primers for the variable and joining regions as described by Reed et al. . This apGrossly, the spleen was enlarged, weighing 519 grams and measuring 16 x 11 x 6.5 cm. The splenic capsule was well preserved. Histologically, the spleen architecture was altered with expansion of the white pulp nodules by small mature lymphocytes with no prominent germinal center formations identified  is the most common chromosomal abnormality and is present in 71% of cases when using the most sensitive fluorescence in situ hybridization (FISH) method . Other lechnique ,28.Our report adds to the extremely limited literature about the histopathologic features of PPBL; to our knowledge this is one of the first detailed descriptions of spleen pathology in a patient with PPBL. Both histological and immunohistochemical findings were misleading and mimicked B cell lymphoma. To avoid misdiagnosing this process as B-cell lymphoma, which may lead to unnecessary treatment, it is important to recognize the misleading histology and somewhat unusual phenotype, assess clonality and to be aware of the cytogenetic and molecular abnormalities that may be associated with this intriguing but benign entity.This case report was based on the existing data, and the patients’ identification was kept confidential in this study. This case report does not meet definition of human or animal subject research by University and Medical Center Institutional Review Board of East Carolina University and University of Manitoba, and no ethical approval was necessary for this study.Both authors declare that they have no competing interests.PS performed literature review, patient records review and drafted the manuscript. RJ conceived of the study, participated in its design and coordination, helped to draft and edited the manuscript and created the figures. PS and RJ were directly involved in the diagnosis and care of the patient. All authors read and approved the final manuscript.


Varicose veins are a common entity presenting a worldwide distribution. Although they are usually benign, sometimes are proved to be a threatening condition. Massive hemorrhage is an unusual complication of this common venous pathology that demands immediate medical intervention.We present a case of a 66-year-old woman found dead in her house surrounded by a large quantity of blood. Autopsy revealed a 7 mm ulcer on the internal surface of the left lower leg communicating with a varicose vein, signs of exsanguinations and liver cirrhosis. Toxicological analysis was negative.Massive hemorrhage from a ruptured varicosity is a severe medical emergency. Awareness of the risk of massive hemorrhage may provoke preventive treatment to be undertaken so as terminal loss of consciousness and a subsequent unattended death to be averted. Varicose veins are a chronic clinical condition well known since antiquity and with a wide distribution. AccordiWe present an autopsy case of a 66-year-old woman who died due to fatal varicose vein rupture in the context of liver cirrhosis.A 66-year-old woman was found dead by a neighbor at her home, lying in a large quantity of blood. Multiple blood-soaked tissues were found around her body Figure. The exaDuring autopsy, sparely developed livers were observed on the body. The skin of her left lower leg showed significant edema, brownish-red discoloration and induration, compatible with chronic venous insufficiency. On the internal surface of left lower leg a 7 mm cutaneous ulcer was situated FigureA. On disRemarkable findings on internal examination included significant pallor of all the organs, subendocardial hemorrhages, a small yellowish, nodular and firm liver and an enlarged and congested spleen.Histology revealed pulmonary edema and liver cirrhosis. Additionally, a defect in the epidermis with signs of continuity with a superficial vein was observed. The ulcer margin was covered by epidermis that abutted above, within or under the few fibrinous exudates. The underlying dermis contained numerous small and medium-sized blood vessels, filled with red blood and surrounded by thick organized fibrous cuffs. The remainder dermis presented fibrosis and abundant macrophages, as well as focally extravasated red blood cells and perivascular deposits of hemosiderin, a sign of repeated past dermal micro-hemorrhages FigureB.Toxicological analysis was negative.Death was attributed to massive hemorrhage due to rupture of a varicose vein of the left lower leg.Death from fatal hemorrhage due to varicose vein rupture is a well described phenomenon with a recognized representation in the literature-15. NeveTwo types of ulceration related to fatal hemorrhage have been described: the acute perforative type, which is a small lesion (<5 mm) with almost no skin involvement; and the chronic ulceration, being a large lesion (10-100 mm) associated with skin pigmentation and induration and erosion into a superficial or deep vein of the leg. In the Characteristic features of deaths due to massive bleeding from a ruptured varicose vein have been mentioned. They include old age, social isolation, possible underlying medical conditions , hemorrhage related to minor trauma, a rapid outcome and some associated features such as alcohol consumption or anticoagulant medications. AnotherIn the cases of death due to varicosity rupture it is worth mentioning the victims’ failure to give themselves the adequate immediate medical care, e.g. direct pressure application and elevation of the bleeding limb. The victim in the present case tried to give herself a first aid, as bloodstained tissues were found around her at the place of death, but the coexisting liver pathology (cirrhosis) seems to have exaggerated hemorrhage, something that quickly resulted in unconsciousness and hastened death. Such a hemorrhage is definitely a medical emergency and requires prompt treatment. However, the possible lack of training and obtaining the right information relevant to this pathology, as well as the false appreciation of the importance of bleeding due to mental disease or alcohol ingestion, are the main factors that no first aids are given.Although varicose veins are a benign clinical pathology, they conceal the danger of rupture, especially if left untreated. The subsequent venous bleeding can be severely intensive and can quickly lead to a loss of consciousness and death. This case is remarkable from the forensic pathology point of view but also contains a preventive imperative. Awareness of the risk of massive hemorrhage may provoke preventive treatment to be undertaken. Therefore, therapists and general practitioners should bear in mind that whenever they treat a patient for varicose veins or any other pathology that might deteriorate varicosities, they should always consider the possibility of adverse interaction of the morbid entities leading to fatal bleeding of a varicose vein.Written informed consent was obtained from the patient daughter (next of kin since the patient is dead) 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.KF wrote the manuscript, performed the literature search and contributed to autopsy. AM performed the histological examination and provided the micro-photograph. VB performed the toxicological analysis. GS and GTV contributed to autopsy and have been involved in drafting the manuscript. TV performed the autopsy and revised the manuscript. All authors read and approved the final manuscript.


Glioblastoma multiforme (GBM) is the most aggressive form of primary brain tumours known collectively as gliomas. Gliomas are graded by their microscopic appearance. As a rule, their behaviour can be predicted from histology: Grade I (pilocytic astrocytomas) and Grade II (benign astrocytomas) tumours are of low grade and grow slowly over many years. Grade IV tumours (GBM) are the most aggressive and, unfortunately, also the most common in humans, growing rapidly, invading and altering brain function. These tumours arise from the supporting glial cells of the brain during childhood and in adulthood.These growths do not spread throughout the body like other forms of cancer, but cause symptoms by invading the brain. Untreated GBMs are rapidly lethal. Most patients with GBM die of their disease in less than a year and none have long term survival.Extracranial metastases from GBM are extremely rare, with a reported frequency of only 0.44% because of the absence of lymphatics in the brain and the difficulty of tumours to penetrate blood vessels. A case of glioblastoma multiforme with the rare features of extensive liver and bone metastases is presented in this paper. Distinct genetic alterations in GBM cases have been identified but as a rule there is no familial history. The peak age of presentation is in late adult life. Reported series from the USA give an incidence of approximately 20,000 cases of GBM per year.Gliomas are malignant brain tumours of glial cells growing along white matter tracts. Glioblastoma multiforme (GBM) is the most aggressive form of these primary brain tumours, which usually arise GBM is usually located in the cerebral hemispheres, with a predilection for the white matter of the centrum semiovale and corpus callosum. There is relative sparing of the basal ganglia and grey matter. Posterior fossa and brain stem gliomas are seen in a younger age group. Multifocal tumours occur in 2-5% . ContrasThe diagnosis of GBM requires tissue obtained by surgical methods. Surgery may vary from stereotactic needle biopsy of deep tumours to open surgical excision of surface tumours using frameless stereotaxis. As the name implies, GBM is multilobulated in appearance on gross pathology, demonstrating quite extensive vasogenic edema. It is a deeply infiltrating tumour with areas of haemorrhage and necrosis. Histologically, it is highly cellular, often bizarrely pleomorphic, undifferentiated multipolar astrocytes with prominent vascular endothelial proliferation and no capsule is demonstrable .A 45-year-old woman had complained of progressive headache over a period of one year with associated disturbances in speech and blurring of vision over the last three weeks. A contrast-enhanced MRI of the brain was performed in February 2004, showing a heterogeneously enhancing lesion in the left temporal lobe with an associated cystic component . She theShe then presented 9 months post-surgery with complaints of back pain. An MRI of the lumbosacral spine revealed features suggestive of bone metastases. A radionuclide bone scan performed two weeks later demonstrated extensive skeletal metastases. A subsequent MRI of the thoraco-lumbar spine demonstrated multiple areas of metastases with evidence of spinal cord compression at the T7 level .The patient by this time had also complained of vague abdominal pains, and a CT scan of the abdomen and pelvis showed multiple massive hypodense liver lesions of varying sizes . An ultrBased on the laboratory histopathology results and imaging findings, a diagnosis was made of glioblastoma multiforme (GBM) of the brain, with extensive liver and bone metastases.et al. found metastases occurred in regional lymph nodes (51%) and lungs and pleura (60%) and occasionally in the bone (31%) and liver (22%) [Extracranial metastasis of GBM is extremely rare, with a reported frequency of only 0.44%. Pasquier ph nodes 1% and luExtra-cranial metastases have been described following stereotactic biopsy and few cases have been reported to occur in the absence of previous craniotomy . DistantIn the present case, the patient was initially thought to have another malignancy giving metastases to the liver. However, histopathologic confirmation obtained via a liver biopsy revealed highly cellular pleomorphic cells similar to that seen in the primary cerebral glioblastoma multiforme. Therefore, the extensive liver lesions were accepted as extra-neural metastases of GBM and the patient was treated accordingly.Much evidence now exists to support the notion that the removal of a maximum volume of tumour in the brain improves function and prolongs survival in the patients with GBM. Chemotherapy is a controversial treatment as many studies have failed to show prolonged median survival in treated patients, although the proportion of long term survivors may be somewhat greater. With distant metastases, chemotherapy is the only appropriate treatment but in these patients the prognosis is very poor.A case of glioblastoma multiforme of the brain with the rare occurrence of extensive extra-cranial metastases to the liver and bone is reported.


Choriocarcinomas generally develop in females. Non-gestational choriocarcinoma in males is extremely rare. The present study describes two cases of young males who were diagnosed with intracranial choriocarcinoma. One case was of an aggressive choriocarcinoma with multiple metastases to the brain, but with an unidentified origin. The patient was admitted in the terminal stage of the cancer. Although a tumor resection was performed, the condition of the patient rapidly deteriorated and chemotherapy was not recommended. The patient succumbed nine days after the surgery. The second case was of a primary ventricular choriocarcinoma. The patient was hospitalized for acute hydrocephalus caused by a mass that was located in the ventricle. Following a tumor resection, the patient underwent a course of whole-brain and spinal radiotherapy. The patient was followed up for more than half a year and remained in a good condition. The present study describes the two cases and a comprehensive review of the literature that was performed to identify similar studies that document choriocarcinomas in males. A choriocarcinoma is a highly malignant tumor arising from the embryonal chorion. Generally, the term refers to gestational choriocarcinoma, which most commonly occurs with a hydatidiform mole, spontaneous abortion, ectopic pregnancy or normal delivery . ChoriocA 21-year-old male with a two-month history of dizziness and fever and a one-week history of vomiting and numbness of the right limbs was admitted to the Renji Hospital . The patient developed symptoms of dizziness and fever two months prior to being admitted. At that time, the patient was treated in the local clinic with a course of anti-inflammatory medication. The symptoms improved, but a mild fever remained. The chest X-ray indicated nothing unusual at that time. One week prior to admission, the patient complained of coughing and sputum with occasional blood streaks, left upper limb pain and numbness of the right limbs. The patient also vomited frequently. The vomitus contained gastric contents. A chest X-ray was performed again at the outpatient department and revealed multiple nodules in the lungs. The brain magnetic resonance imaging (MRI) scans demonstrated multiple mass lesions occupying the bilateral parietal and occipital lobes . The pasIntracranial surgery for the resection of the multiple lesions was performed one day after admission. The tumor appeared dark red with a tough texture and rich blood supply. The boundary with the normal brain tissue was clear. An intra-operative biopsy of the mass was sent for frozen section analysis and the result was that of a metastatic poorly-differentiated cancer. Following the surgery, the patient gradually developed intracranial hypertension symptoms, including headaches and projectile vomiting. The symptoms progressively worsened. On post-operative day seven, a head computed tomography (CT) scan indicated that new hemorrhagic metastases had emerged in the frontal and parietal lobes. The parents of the patient rejected a decompressive craniectomy and chose a conservative treatment. The pathology report revealed that the brain tumor was a metastatic choriocarcinoma and the immunohistochemistry is shown in A 20-year-old male was admitted to the Renji Hospital due to a sudden onset of unconsciousness, with a six-month history of nausea and vomiting. The neurological findings were unremarkable, with the exception of a low level of consciousness. A head CT scan demonstrated acute hydrocephalus and a mass lesion occupying the third ventricle.Emergency surgery for external ventricular drainage was performed upon admission. Following the surgery, the consciousness level improved. A head MRI examination was then performed and revealed a mass lesion in the ventricle . The cheChoriocarcinomas are aggressive malignancies that are grouped into two categories, gestational choriocarcinoma, which is derived from any form of previously normal or abnormal pregnancy, including a hydatidiform mole, spontaneous abortion or ectopic pregnancy, and non-gestational choriocarcinoma, which typically arises from gonadal organs, but also occurs rarely in extragonadal primary sites. The present study described two cases of non-gestational choriocarcinoma. One case was of an aggressive choriocarcinoma with multiple metastases to the brain, but with an unidentified origin, and the other case was of a primary intracranial choriocarcinoma.Non-gestational choriocarcinoma typically arises from the gonadal organs. When the primary origin is extragonadal, choriocarcinoma occurs preferentially at the midline section, including the pineal body, mediastinum and retroperitoneum . In the Several theories explaining the pathogenesis of these extragonadal choriocarcinomas have been proposed, but no conclusions have yet been reached. There are presently three hypotheses: i) The tumor is a metastasis from a testicular choriocarcinoma that regressed spontaneously ; ii) theA diagnosis is possible if the site is near the body surface, and a partial or total biopsy may be performed relatively safely. However, a biopsy may not be performed at a number of sites, including the mediastinum, pineal body, lung and retroperitoneum, making a pre-operative diagnosis extremely difficult. When a diagnosis of non-gestational choriocarcinoma is suspected, it is necessary to fully examine the patient’s testes. Generally, the physical examination of testicular tumors includes palpation for testis enlargement and occasionally, an assessment of trigger points for pain.et al(Furthermore, since non-gestational choriocarcinoma has a trophoblastic element, the tumor secretes β-hCG. Therefore, the tumor is associated with a markedly raised serum β-hCG concentration, which is significant in the diagnosis and monitoring of the clinical progress. Yokoi et al reportedThe clinical manifestation of non-gestational choriocarcinoma is varied. The cancer from the primary site spreads via the blood and lymphatics, with early hematogenous dissemination to the lungs, liver, brain and other visceral sites . The usuIt is evident that an early diagnosis was not established in the first case, and the condition of the patient deteriorated prior to the appropriate therapy being instituted. Subsequent to the pathology results confirming the diagnosis, a physical examination and ultrasound of the testis was performed, and nothing abnormal was identified. Since an autopsy was not allowed, the primary site was not identified. According to the ‘burned out tumor’ theory, as the primary site in the testis may be quite small or even totally regressed, a clinical examination of the testis may appear normal . Therefoet al(A total or subtotal resection of an intracranial choriocarcinoma is useful for the prognosis of the patient. Advances in neuroimaging, microsurgical techniques, surgical instruments and surgery-supporting systems allow a subtotal or greater resection of an intracranial choriocarcinoma to be feasible and safe. However, the total removal of the tumor remains difficult. Radiotherapy is an indispensable therapeutic modality for intracranial choriocarcinoma. Kohyama et al reportedet al. In Japaet al.Choriocarcinoma is one of the most aggressive and malignant germ cell tumors and the clinical outcome is poor. An investigation based on 97 cases revealed that the mean survival time was 7.7 months and that the one-month mortality rate was 23.8% . As the


Hepatic solitary fibrous tumor (SFT) is a rare tumor originating from the mesenchyme. Here we report a new case of SFT in the liver and review the clinical presentation, radiological and operative findings, diagnosis, treatment, and outcome. The patient was a 59-year-old man who presented with progressive fatigue for 3 months and an abdominal mass for 3 days. On laboratory tests, no abnormality was detected except that abdominal ultrasonography revealed a 9.0 × 6.2 cm hypoechogenic mass in the left lobe of the liver. A computed tomographic scan confirmed a hypodense lesion in the left lobe of the liver. The patient underwent left hepatectomy. SFT was diagnosed on the basis of histopathological findings. The patient was free from all symptoms and had no signs of local recurrence after 24 months' follow up. Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm of mesenchymal origin, first described by Klemperer and Robin in the visceral pleura in 1931 , the skiA 59-year-old man was admitted to our hospital because of progressive fatigue for 3 months and an abdominal mass for 3 days. The patient had no history of viral hepatitis. Laboratory tests, including routine biochemistry, liver function, and tumor markers, were normal. A plain chest X-ray was normal. Abdominal ultrasonography revealed a 9.0 × 6.2 cm hypoechogenic mass in the left lobe of the liver. Computed tomography (CT) demonstrated a large heterogeneous circumscribed mass in the left hepatic lobe and contrast enhancement in the arterial and portal phases Figure . Left heGrossly, the tumor was a large, gray-white, lobulated, well-circumscribed partially encapsulated mass, measuring 9 × 7 × 6 cm. In the peripheral liver parenchyma, the vascular structure and bile duct were compressed, and no cirrhosis or fibrosis was observed Figure . HistoloImmunohistochemically, the tumor cells were strongly positive for CD34 Figure , CD99 F, Bcl-2, Primary solitary fibrous tumor of the liver is an extremely rare neoplasm. Individual case reports are infrequent. The first description of this type of tumor involving the liver may be that provided by Nevius and Friedman in 1959 ,8,10. ThThe radiological findings may suggest the diagnosis of SFT, but benign or malignant hepatic tumors such as hepatocellular carcinoma, sarcoma, leiomyoma, and inflammatory pseudotumor may have similar features . Thus, tThe pathologic features of the SFT of the liver described here resemble those described for solitary fibrous tumors of other locations . TypicalThe differential diagnosis includes leiomyoma  , inflammAlthough most cases have benign clinical behavior, there is no strict correlation between histological findings and biological behavior. Some may have malignant histological features and recur locally or metastasize . TherefoWe observed a rare case of SFT of the liver. Correct interpretation of unique pathological findings and CD34 immunoreactivity plays a significant role in differentiating SFT from other spindle-cell neoplasms of the liver. A complete surgical resection is the treatment of choice and is curative in most cases, and follow-up surveillance is necessary. The outcome of SFTs is mostly related to resectability rather than pathologic grade or tumor size. Given the limited number of cases reported in the literature, it is still difficult to establish the long-term prognosis of this disease.The authors declare that they have no competing interests.KS, JFW, and XDT conceived the concept, participated in drafting the manuscript, and conducted critical review. JJL took part in the care of the patient, assembled data, and participated in writing the manuscript. LXY carried out the histopathological evaluation and reviewed pathology. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor in Chief of this journal.


Leiomyosarcoma of the gallbladder is a rare entity, constituting about 1.4 per 1000 gallbladder malignancies. Literature review shows female preponderance in sixth decade of life, due to unknown reasons. We report one such rare case of a 50-year-old female admitted with pain in right upper abdomen. On examination, mass was felt in right hypochondrium. The ultrasound abdomen showed mass with loss of interface with liver and cholelithiasis. CECT abdomen showed polypoidal gallbladder malignancy with ill-defined interface with liver. She was operated upon with diagnosis of carcinoma gallbladder; extended cholecystectomy was done. Histopathological examination revealed spindle-cell proliferation and possibility of malignant tumor of mesenchymal origin was kept. This was later confirmed on immunohistochemistry. Primary leiomyosarcoma of the gallbladder is rarely encountered tumor out of adenocarcinoma and other sarcomatous tumors of gallbladder. These cases constitute about 1.4 per 1000 gallbladder malignancies . Second The index patient was a 50-year-old female who presented with history of vague right upper abdominal pain and was found to have a firm palpable mass in right hypochondrium. With a provisional diagnosis of gallbladder cancer, she was subjected to routine investigations including radiological investigations. She had normal haemogram and liver function tests. Ultrasound of the abdomen revealed 9 × 8 cm mass in gallbladder fossa with interface with liver not delineated and cholelithiasis. Biphasic CT abdomen showed gallbladder grossly distended: soft tissue mass in gallbladder with multiple large intraluminal projections and features suggestive of cholelithasis with polypoidal gallbladder malignancy . A proviLeiomyosarcoma is a malignant mesenchymal tumor composed of cells showing smooth muscle differentiation. This tumor usually occurs in middle-aged or older individuals. It constitutes significant percentages of retroperitoneal, vascular, extremity, and uterine sarcomas. However, their existence as a mesenchymal neoplasm of gallbladder is still a rare entity except for isolated case reports. Most of the reported cases were females in their sixth decade of life who had vague clinical presentation suggestive of cholecystitis , 4. In t


Chronic hyperplastic pulpitis (pulp polyps) usually occurs in molar teeth of children and young adults and is characterized by an overgrowth of granulomatous tissue into the carious cavity. Here, we report a rare type of pulp polyp in lower third molar of a 27-year-old woman that not only grow into carious cavity but also extruded in very large size that interfered with occluding of the teeth. Hyperplastic pulpitis is a type of irreversible chronic pulpitis that occurs usually in young teeth where the pulp has been exposed by caries or trauma . MechaniA 27-year-old woman referred for treatment of left side lesion of the oral cavity. She gave history of six months for its presence that enlarged gradually and interfered with eating and occluding the teeth, so that made patient worried about it , Figure.rd molar with no specific lesion in bone and adjacent tissues (In review of systems (ROS) she gave history of idiopathic thrombocytopenic purpura and splenectomy of 12 years ago and caesarian section three years ago, but both of them had no relation to the present lesion. Laboratory examinations including CBC differential, WBC, platelet count, PT, and PTT were within normal limits, and in orthopanthogram radiography we observed left carious mesioangular semi impacted 3 tissues .Intraoral examination showed a large polypoid lesion with about 3.5 cm height, 1.5 cm width and a 5 mm stalk diameter protruded from the carious cavity of partially exposed crown of the tooth, the lesion overlaid on lingual side of this tooth and obviously presented itself in left oropharyngeal area.So with considering the radiographic and clinical data these differential diagnoses were possible:1. A large pulp polyp2. Peripheral giant cell granuloma3. Papillomard molar tooth performed under local anesthesia .The surgical procedure for excisional biopsy of the lesion and surgical removal of 3rd molar with base of the lesion in the carious cavity and the bulk of the lesion to the pathologist for histopathologic examination  is the most visually dramatic of all pulp response, rising out of the carious shell of the crown and is a "mushroom" of living pulp tissue that is often firm and insensitive to touch .It is a response of the pulp to acute inflammation occurs in young teeth but never in teeth of old patients, may this be indicative of a good pulpal response. Presumably the young pulp does not become necrotic following exposure, because its natural defenses and rich supply of blood allow it to resist bacterial infection. Transudates and exudates which are inflammatory response products in open chronic pulpitis, drain into the oral cavity and do not accumulate.Thus intrapulpal pressure, which may consequently cause tissue damage and destruction of the microcirculation, does not develop .According to chronologic time for development of the lower 3rd molar teeth between 4.4-22.1 years old , this paThe large size of the lesion in this patient may be due to:1. No severe pain because this type of lesions usually has no pain and subside within seconds after the stimulus is removed 4. Neglected treatment by the patient for about six months5. Good blood supply to the lesion6. Continuous trauma from occluding the teeth.In treatment planning for Oral and maxillofacial lesions, we must consider the clinical finding, and dental history of the patient and finally the histopathologic report for management of such lesions in spite of unusual size of them.


A case of adrenal cystic neuroblastoma detected at 37 weeks of gestation is reported. Postnatal ultrasonographic examination showed slightly increased in size demonstrating marked septations within the cyst. After the tumor was resected, histopathological examinations confirmed the diagnosis. The patient is developing normally at 1 year of age. Neuroblastoma is the most common solid tumor in children under 1 year of age . This tuPrenatal diagnosis of neuroblastoma was first reported by Fénart et al. . NeuroblWe have reported a case of cystic adrenal neuroblastoma that was growing rapidly postnatally with a brief review of the literature.A 38-year-old woman, gravida 2 para 1, was referred to our center for assessment of cystic mass in fetal abdomen at 37 weeks of gestation. Her medical history was unremarkable. The detailed ultrasonographic evaluation revealed suprarenal cystic mass on the left side with a diameter of 45∗45 mm . The cysImprovements in prenatal imaging and widespread use of fetal ultrasonography have led to an increased rate of prenatal diagnosis of fetal neuroblastoma. The features of neuroblastoma on the antenatal ultrasound are variable and range from cystic, mixed solid and cystic, and completely solid with or without calcification. Patients with cystic neuroblastoma had a better outcome than those noncystic tumors . The maiReported prevalence of neuroblastoma cases in prenatally detected suprarenal masses is 81–85 % in the literature , 11. TheRelationship between neuroblastoma and congenital heart defects was emphasized in a report with a recommendation of echocardiography for congenital cardiovascular malformation screening in patients with newly diagnosed neuroblastoma . Fetal h¹²³I methyliodobenzylguanidine (MIBG) scintigraphy that is most sensitive for identifying metastatese in general [Urinary catecholamines are helpful in confirming the diagnosis of neuroblastoma; however, they will be normal in two-thirds of patients with prenatally diagnosed tumors, thus this test will not serve as a reliable diagnostic aid in every case . Evaluat general . Classif general . The liv general , 23.The treatment options for congenital neuroblastoma include surgery, chemotherapy followed by surgery or expectant management with ultrasonographic followup . Most paClose sonographic monitoring of cystic adrenal masses during the first months life is helpful to detect early complications, and surgical excision could be performed safely during the neonatal period.


Serous cystadenoma is a common benign neoplasm that can be managed without surgery in asymptomatic patients provided that the diagnosis is certain. We describe a patient, whose pancreatic cyst exhibited a radiological appearance distinct from that of typical serous cystadenoma, resulting in diagnostic difficulties. CT and MRI showed a 10 cm-polycystic tumor with upstream dilatation of the main pancreatic duct (MPD), suggestive of intraductal papillary mucinous tumor (IPMT). Ultrasonographic aspect and EUS-guided fine-needle aspiration gave arguments for serous cystadenoma. ERCP showed a communication between cysts and the dilated MPD, compatible with IPMT. The patient underwent left pancreatectomy with splenectomy. Pathological examination concluded in a serous cystadenoma, with only a ductal obstruction causing proximal dilatation. Typical macroscopic characteristics of serous cystadenoma of the pancreas consist of microcystic mass with a sponge-like honeycomb aspect or central scar or both. The risk of malignant transformation seems to be low even in the long-term course . TherefoIn January 2009, a 66-year-old woman with a medical history of thyroidectomy was admitted to Lille University Hospital for further evaluation of a large cystic mass in the distal pancreas. She had complained of transient epigastric pain one month before. Blood biochemical parameters showed no pancreatitis and the tumor markers, mainly CEA and CA 19-9, were all within the normal limits. On admission, the patient was asymptomatic and the pancreatic mass was not palpable in the upper abdomen. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) showed a huge, well-defined, multiloculated, cystic mass, of 10 cm in greatest dimension, replacing the entire body and tail of the pancreas, and displaying multiple calcifications in its isthmic part Figures . Both MRSerous cystadenoma has 2 main morphologic patterns: typical one, seen in 70% of cases, displays microcystic pattern that is characterized by radiologically visible multiple cysts measuring 2 cm or smaller, without communication with the MPD –7. The cHowever, there are various features of serous cystadenomas found on imaging studies which may lead to diagnostic difficulties. Main atypical manifestations include cystic tumor without microcystic pattern, cystadenoma with interval growth, cystadenoma communicating with the MPD, and giant tumors with ductal dilatation as seen in our patient , 7. SeroIn most cases, the diagnosis of cystic pancreatic lesions relies on CT and MRI. Nevertheless, when combined, their diagnostic accuracy was less than 50% of cases in a recent series of 70 cystic pancreatic lesions independently reviewed by two blinded radiologists . ERCP prPatients bearing a cystic pancreatic lesion are advised to undergo resection based on defined criteria: the presence of symptoms, abnormal cyst aspiration fluid, and radiologic criteria suspicious for a mucinous neoplasm  . By contIn conclusion, our case highlights an extremely rare case of serous cystadenoma with compression of the main pancreatic duct. Despite the availability of high-quality imaging techniques, uncertain diagnosis led us to perform left pancreatectomy, although EUS with FNA were suggestive of serous cystadenoma. Algorithm is then proposed to manage cystic pancreatic lesions: (i) symptomatic lesions must be removed and (ii) EUS with FNA is the key investigation for asymptomatic lesions of uncertain imaging diagnosis.


Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of late adult life and may also arise as a primary tumor in bone. It is a rare condition that constitutes less than 1% of the malignant tumors of bone and commonly occurs in the mathaphysis of long bones of extremities such as the femur and tibia. The occurrence in the head and neck region is very rare. MFH of the jaws is a highly malignant tumor that recurs, metastasizes, and usually causes death despite aggressive surgical therapy. We present a case of MFH of maxilla with 8 years follow-up. The clinical, pathologic and radi-ographic features as well as the treatment of this case are discussed. The mandibulomaxillary area is the most common site for MFH in the skull.5 MFH of the maxilla and mandible is a highly malignant tumor that recurs, metastasizes, and commonly results in death despite aggressive surgical therapy.6 The etiology of this tumor is unknown but seems to be multifactorial. Genetic background, environmental factors such as trauma, radiotherapy and malignant transformation from benign lesions can be involved in the etiology of this malignant tumor.9 In this paper, we present a case of MFH of maxilla with eight-year follow-up. The clinical, pathologic and radiographic features as well as the treatment of this case are discussed.Malignant fibrous histiocytoma (MFH) is the most common sarcoma of soft tissue in the late adulthood. It may also arise as a primary neoplasm in bone, a rare lesion that constitutes less than 1% of the malignant tumors of bone, commonly occuring in the mathaphysis of long bones of extremities such as the femur and tibia.A 36-year old man was referred to the Department of Oral and Maxillofacial Surgery, Taleghani Hospital, Tehran, Iran in September 26, 2001 with a mild and painful swelling for two months mostly in the left cheek and palate. The patCT scan of the upper jaw showed an osteolytic mass with extensions to surrounding soft tissues. The margins were ill-defined .An incisional biopsy was performed under local anesthesia. Histological evaluations showed the tumor mainly consisted of spindle cells and atypical multinucleated giant cells. Spindle cells were arranged in a storiform pattern. No other specific features of differentiation were observed in the specimens.1, and cytokeratin. The tumor was diagnosed as high-grade MFH, storiform-pleomorphic type, of the maxilla.Immuno-histochemical staining revealed positive reactivity for vimentin and α-1 antitrypsin. There was no immuno-reactivity for smooth muscle actin, S100 protein, desmin, MyoDOn October 4, 2001, patient underwent surgery. The Weber Ferguson incision and also lip split were performed. Hemi-maxillectomy with orbital floor extension was performed to allow for safety margins.Frozen sections were performed in almost twenty regions including soft and hard palate, pterygoid muscles, cheek tissue, upper lip, lateral nasal and orbital floor. Evaluation of the sections revealed all areas were free of tumor. A dermal graft from medial thigh region was used to prevent histocontraction. Subsequently, the wound was dressed.The patient was discharged three days after operation and the sutures were removed eight days later. After surgical treatment, the patient received post operative radiation with 55 Gy during two months utilizing the combination regimen of Adriamycin, dacarbazine and cyclophosphamide. A maxillofacial prosthesis (obturator) was constructed for the patient three months after surgery.From 2001 to 2009, the patient was visited every year. The patient has been free of local recurrence or distant metastasis of the disease for eight years after surgery.10The tumor usually affects individuals older than 60 years of age. Its incidence in young people is less than 5%.1 Feldman & Norman11 for the first time in the 1970s described primary malignant tumor of bone that satisfied the histopathologic criteria of MFH. MFH of bone constitutes approximately 5% of primary bone tumors and less than 1% of malignant tumors of bone.1 This tumor mostly occurs in the mathaphysis of long bones of patients aged 10–60 years, commonly in the distal femur and proximal tibia.12 20% of MFH cases in bone occurs in abnormal bone. MFH is very rare in the head and neck region, with involvement of only 5% to 6% of cases.1 The mandibulomaxillary region is the most common area for MFH in the skull.5 MFH of the jaws is a highly malignant tumor that recurs, metastasizes, and commonly results in death despite aggressive surgical therapy.6 Most of the patients with MFH of maxilla and mandible show a painful mass or dental problems and the average interval from the first symptom until diagnosis is 3.5 months. Similar features have bean reported for osteosarcoma.13Malignant fibrous histiocytoma (MFH), the most common soft tissue sarcoma of late adulthood, was first described by O’Brien & Stout in the 1960s.14 described this tumor with four types of histologic presentation, i.e. storiform pleomorphic, mixoid, giant cell, and inflammatory. Many cases of MFH of bone were previously diagnosed as pleomorphic reticulum cell sarcoma, osteolytic fibroblastic osteosarcomas or spindle cell and giant cell sarcoma. MFH of bone is differentiated histologically from osteosarcoma by features such as spindle shape fibroblastic cells in a storiform pattern without osteoid formation. It is usually difficult to differentiate MFH from other malignant tumors of bone including osteosarcoma, leiomyosarcoma, fibrosarcoma, dedifferentiated chondrosarcoma and lymphoma.13MFH is a neoplasm with fibroblastic nature and facultative histiocytic differentiation. Goldblum et al16The diagnosis of MFH is based on positive immunostaining with vimentin, α1-antitrypsin, and α1-antichymotrypsin.1 In one case, paresthesia of the lower lip is reported.7Signs and symptoms of the MFH in head and neck region are similar among different cases which consist of a painful swelling that may be tender on palpation, similar to the present case.9 In the present case, according to the patient’s history, the only relative point was presence of two cases of breast cancer in his family. We did not find any obvious inducing environmental factor or any history of systemic disease. Therefore, the genetic background seems to be the most important point in the etiology of this case.The etiology of this tumor is unknown and seems to be multifactorial.1MFH of jaws commonly result in death despite aggressive therapy.6 One of the most important points of this case is the survival of the patient free of local recurrence and distant metastasis, without involving the curettage of the eye in the treatment, which is a very rare instance. In most of the previous cases, the patient has died despite surgical excisions with wide margins.9 Therefore, the prognosis of this case seems to be better than most similar cases and the reason may be the early diagnosis and surgical intervention with wide margins, checked by frozen sections during the surgery in almost 20 points followed by radiotherapy and chemotherapy. The interval between the first symptom and diagnosis in this case was almost 1.5 months which is shorter than the average interval reported in other cases (3.5 months).13The prognosis of this tumor is often unfavorable and recurrence rate is approximately 44–48%. Metastases more commonly occur in the lung (90% of metastasis) followed by lymph nodes (12%), bone (8%) and liver (1%). About 5% of cases already reveal metastasis when the primary tumor is diagnosed.18 Frozen sections during surgery covering all margins of the lesion seem to play an important role in preventing local recurrence, as employed in this case in 20 points around the margins. Radiotherapy after surgery is usually indicated. MFH is often resistant to chemotherapy; however, use of vincristin, cyclophosphamid, dactiomyein, Adriamycin, cisplatin (cisdiamminedichlro platinum), tyrosine kinase inhibitors and dacarbazine (DTIC—Dimethyl Triazeno Imidazole Carboxamide) have been reported.19MFH of bone aggressively infiltrates adjacent tissues or between muscle fibers and this can be responsible for a high recurrence rate. Surgical excision with wide margins, even excision of clinically normal soft tissues, must be the procedure of choice.The management of malignant fibrous histiocytoma of bone should be based on an early histopathologic diagnosis and immuno-histochemical evaluations to perform radical surgery followed by radiotherapy and chemotherapy. Close follow-up and monitoring after treatment is also important because recurrence is a frequent problem and early metastasis to the lungs is common.


Neurogenic tumours of the larynx, particularly schwannomas are rare. We report a case report of a schwannoma in a 30-year-old woman which was excised endoscopically. The aim of this paper is to highlight this rare condition and management options to the otolaryngological community. A 30-year-old lady presented to the outpatient clinic with progressive dysphonia and exertional dyspnea over a 2-year period. There were no associated systemic symptoms. She was a nonsmoker and consumed alcohol occasionally. On examination, she was dysphonic but not stridulous or dyspneic. Flexible nasoendoscopy revealed a soft tissue swelling in the supraglottic region starting from the posterior commissure blocking the view of the vocal cords. Computer tomography (CT) showed a well circumscribed 1.5 × 1.4 cm low attenuation midline supraglottic mass, commencing just below the aryepiglottic folds and extending onto sclerotic arytenoids . Those affecting the head and neck are commonly intracranial  or parapharyngeal . Neurogenic tumors of the larynx  therefore remain extremely rare. Indeed, it represents less than 1.5% of all benign tumours (the majority being papillomas and chondromas) .The origin of the schwannoma is presumed to be in the internal branch of the superior laryngeal nerve . Often hFrom a clinical perspective, it is vital to differentiate a schwannoma from a neurofibroma for 2 reasons. Firstly, neurofibromas have a higher potential of recurrence and malignancy (approximately 10%). Secondly, the presence of neurofibromas should also alert the physician to the exclusion of neurofibromatosis. The definitive diagnosis is established histologically.Patients typically present with dysphonia, stridor, dyspnea, dysphagia, globus sensation, or lateral neck lump. On laryngoscopy, most lesions appear as a smooth submucosal lesion, usually confined to the aryepiglottic fold or false vocal cord. It may obstruct the view of the laryngeal inlet and result in reduced mobility of the vocal cord. The latter may be due to “pseudofixation” of the cricoarytenoid joint as a result of mass effect of the lesion . Almost CT and MRI are valuable in defining the nature and extent of the lesion, with MRI offering superior soft tissue delineation. Typically, the lesion is sharply demarcated, round or oval, isoattenuated with muscle, and often heterogeneously enhanced . CalcifiThe definitive diagnosis is performed histologically. Enger and Weiss established three histological criteria for the diagnosis of schwannoma: encapsulation, presence of Antoni A and/or Antoni B stroma, and S-100 protein positivity . In AntoSurgery forms the mainstay of treatment of laryngeal schwannoma. A tracheostomy may be required to secure the airway. The surgical approach depends on the size and location of the lesion. Smaller lesions can be approached endoscopically with or without a laser. Larger tumours may require an external approach, for example, lateral thyrotomy, lateral pharyngotomy, or laryngofissure technique . Wide exDespite its rarity, neurogenic tumors of the larynx need to be recognised. The primary management involves securing the airway. Despite various imaging modalities, the distinction between Schwannoma and neurofibromas can only be made histologically. Complete resection of the lesion is necessary to prevent recurrence.


Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this paper illustrated the difficulties with which routine anatomical pathologist is faced when dealing with rare renal poorly differentiated neoplasm in adults. Primitive neuroectodermal tumors (PNETs) are small, round-cell tumors of neural crest origin classically found in the central nervous system (CNS) but more recently characterized in the periphery . PeripheEwing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney. Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date –7. RenalWe present here two cases of Ewing's sarcoma/primitive neuroectodermal tumor occurring in two young women with an unusual presentation. The diagnosis in both cases was incidental during visits to the emergency room for other health problems.A 32-year-old female presented to the emergency room with right flank pain that irradiates to the groin. She was clinically diagnostic with kidney stones and indeed a kidney stone was identified. However, the computed tomography scan revealed a large right renal mass and right ovarian cystic mass. The patient underwent a right-sided laparoscopic radical nephrectomy along with an excision of the ovarian cystic mass. Her postoperatively course was uneventful except for some pain in the surgical incision site. Grossly, an 11 cm cyst adenofibroma of the right fallopian tube and ovary was identified. In addition, the kidney showed an 8.3 cm encapsulated tumor located at the pelvic adipose tissue within the renal capsule . MicroscA 21-year-old female presented to the emergency room with a left foot fracture, nasal fracture, and loss of her pregnancy caused by a motor vehicle accident. A month later, the patient returned to the emergency room with complaints of shortness of breath, icterus, and dark urine. A chest-computed tomography scan revealed pulmonary embolism. She underwent a pulmonary embolism protocol which included a computed tomography of the chest, abdomen, and pelvis. The exam showed extensive central bilateral pulmonary embolism and a large right atrial thrombus in continuity with thrombotic material within the inferior vena cava. In addition, her right kidney had poorly contrast-enhancing raising the possibility of a right renal vein thrombosis extending into the right atrium with subsequent pulmonary embolism. She underwent a pulmonary tumor embolectomy, right atrial mass removal, and tumor embolectomy thrombectomy of the hepatic inferior vena cava as well as placement of an inferior vena cava filter. The patient underwent a sternotomy with cardiopulmonary bypass. She started chemotherapy with etoposide and ifosfamide combination therapy. The tumor invaded the renal vein and extended to the resection margin. The histological examination of the right atrial, pulmonary artery, and caval vein thrombus revealed islands of uniform small round blue cells with scant cytoplasm, uniform nuclei, and stippled chromatin intermixed with necrosis and blood clot . The viaThe diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The most challenging part is the differential diagnosis which is broad with morphological and immunohistochemical overlapping features between the entities. Therefore, mostly frequently molecular biology techniques need to be applied for a definitive diagnosis. We reported 2 cases of renal primitive neuroectodermal tumor/EWING tumor with an unusual clinical presentation which were incidentally found during imaging. These cases illustrated very well the difficulties with which general pathologists are faced in the routine anatomical pathology examination when faced with renal poorly differentiated neoplasm in adults.Both cases had an uncanny presentation. In case number 1, the 8.3 cm kidney mass was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. Moreover, it was associated with a benign ovarian tumor and the diagnosis of renal primitive neuroectodermal tumor/EWING was only possible after extensive immunohistochemical and molecular studies, which were mainly performed to rule out other entities that were considered due to the unusual presentation and associated ovarian tumor. In literature-reported renal primitive neuroectodermal tumor/EWING, the clinical presentation was often nonspecific, comprising abdominal pain, palpable mass, and hematuria, and most reported cases were diagnosed incidentally by image studies –8. Few tOur second case report was even more unusual. The Case 2 presented with respiratory problems after being involved in an automobilistic accident where multiple fractures were acquired. The clinical presentation suggested pulmonary thormboembolism, which was confirmed by image; however, after histopathological examination of the thrombectomy material small blue cell tumor of uncertain origin was regarded as the cause for the extensive thromboembolic event. Later on, a nephrectomy was performed and a renal primitive neuroectodermal tumor/EWING was diagnosed. As far as literature reports, there are few reports of renal primitive neuroectodermal tumor/EWING with tumor thrombus extending to the inferior vena cava , 10. HowAs far as disease progression, our cases had a better response to chemotherapy and surgery than the previous reported cases of renal primitive neuroectodermal tumor/EWING . The outRenal primitive neuroectodermal tumor/EWING tumor should be differentiated from other small blue cell tumors arising in the kidney such as blastemal predominant Wilms tumor, neuroblastoma, rhabdomyosarcoma, lymphoblastic lymphoma, small cell neuroendocrine carcinoma, desmoplastic small blue cell tumor, and synovial sarcoma , 12. AllCD99 was the former marker for Ewings' sarcoma; however, it is also expressed in other tumors such as synovial sarcoma, Wilms' tumor, vascular malignancy, neuroendocrine tumors, and lymphoblastic lymphoma, and therefore nowadays is not considered a reliable marker of tumor origin , 7, 8. OFrom the neuroendocrine marker, CD56 was positive in both cases whereas synapthophysin was positive only in the second case in a cytoplasm dot pattern. This pattern has been considered as an unspecific pattern, but a recent publication described the pattern in oligodendrogliomas, and therefore the pattern seems to indicate neural origin .The features favoring the diagnosis of blastemal Wilms tumor are young age and positivity of blastemal elements for vimentin, low molecular weight cytokeratin, epithelial membrane antigen, and WT1. Both cases in our study were positive for cytokeratin and vimentin. However, WT1 was negative and EWSR1 gene was identified by FISH, and therefore adult Wilms' tumor was ruled out.Renal neuroblastomas are also similar morphologically to renal primitive neuroectodermal tumor/EWING tumor. However, they usually occur at the age of 5 years and show a fibrillary background or a ganglionic differentiation facilitating the diagnosis. Immunohistochemistry is very useful to differentiate it from renal primitive neuroectodermal tumor/EWING tumor because neuroblastomas are consistently positive for NSE and chromogranin and mostly negative for CD99.Rhabdomyosarcoma is often positive for smooth muscle actin, desmin, and myogenin; all of them were negative in our patients. Desmoplastic small blue cell tumors are also positive for desmin, and synovial sarcoma was ruled out using the FISH for the X-18 SYT translocation, which was negative.Finally, lymphoblastic lymphoma could be excluded by performing analysis with LCA and other hematopoietic markers; however, in the second case the background was positive for lymphoid markers. The differential was performed by morphology of the tumor cells which had stippled chromatin and pleomorphism whereas the lymphoid cells formed a uniform monotonous population with small dark nuclei in the background.In our cases, the molecular analysis identifying the translocation was a supportive and strong confirmatory tool, especially with the confusing immunohistochemical profile. Fluorescent in situ hybridization analysis using the 22q12 LSI, EWSR1, and Dual-Color Break-Apart Probe showed the translocation pattern 22q12 for the EWSR1 gene. The probe used in our cases is from Vysis  and is commercially available, and data have been published using this probe. The probe is an EWSR1  Dual-Color, Break-Apart Rearrangement Probe, which consists of a mixture of 2 FISH DNA probes. The first is a 500 kb probe labeled in spectrum orange and flanks the 5 V side of EWSR1 gene and extends inward into intron 4. The second probe is 1100 kb labeled in spectrum green and flanks the 3 V side of the EWSR1 gene. There is 7-kb probe between the 2 probes. The known breakpoints within the EWSR1 gene are restricted to introns 7 through 10V. Approximately 90% of the translocations involving EWSR1 gene result in t translocation characteristic of EWS/PNET.Because most renal primitive neuroectodermal tumors/EWING tumor of the kidney are poorly differentiated and have a broad morphological spectrum, it is important to have genetic confirmation for the presence of t for the accurate identification of these tumors, and the FISH probe is a good and easy method to do so.In summary, we report two cases of primitive neuroectodermal tumor/EWING tumor arising in the kidney confirmed by morphology, immunohistochemistry and FISH. The unusual presentation in our cases delayed the diagnosis, being both tumors diagnosed incidentally. Thus, we believe that these cases added some features to the already broad spectrum of presentations described in renal primitive neuroectodermal tumor/EWING tumor and also showed the importance of molecular analysis together with morphological and immunohistochemical features for confirmation of this poorly differentiated neoplasm.


Follicular dendritic cells are non-phagocytic, non-lymphoid cells of the immune system that are necessary for antigen presentation and the regulation of reactions in the germinal centers of the lymph nodes. Follicular dendritic cell sarcoma is an unusual cancer, particularly in the intra-abdominal region. In the present report we describe an unusual case of retroperitoneal follicular dendritic cell sarcoma that emphasizes the difficulty of diagnosing and treating this tumor. Retroperitoneal follicular dendritic cell sarcoma has only been rarely reported in the literature to date.A 46-year-old Chinese woman of Han ethnicity presented with chronic right lower quadrant abdominal pain over the preceding 4 weeks. The tumor was resected and submitted to histopathological examination. The case was verified as retroperitoneal follicular dendritic cell sarcoma by microscopic examination and immunohistochemical analysis. After diagnosis, she received postoperative radiotherapy and chemotherapy. She has survived 3 years postoperatively, although she has a pulmonary metastasis.Retroperitoneal follicular dendritic cell sarcoma may demonstrate aggressive potential. This study indicated that postoperative adjuvant radiotherapy and chemotherapy could extend the survival of a patient with retroperitoneal follicular dendritic cell sarcoma. Their main function is to trap and present antigens and immune complexes to B cells. FDCs are found mainly in the germinal centers of the lymphoid tissue, where they form a tight meshwork. FDCs feature complement receptors and human leukocyte antigen-DR on their surfaces ,2. Lymphet al. in 1986,et al.. We repoA 46-year-old Chinese woman of Han ethnicity presented with a 4-week history of chronic right lower quadrant abdominal pain without other associated symptoms. A general systemic examination was normal except for a large mass in her right lower quadrant. There was no significant past medical history. Routine biochemical and hematological tests were within normal limits. A chest X-ray also revealed no abnormalities. A subsequent abdominal computed tomography (CT) scan revealed a 8.6×6.0cm oval-shaped mass, with a clear boundary and mild-moderate enhancement, in her lower right retroperitoneal region, pressing against her right psoas muscle and her right iliac artery Figure A.She underwent an operation to resect the mass. Surgical exploration revealed a retroperitoneal mass measuring 9×6cm adjacent to her transverse colon, her right psoas muscle and her vertebral column and covering her inferior vena cava. No associated lymphadenopathy was observed. Because the mass had infiltrated her right psoas muscle and was attached to her right iliac artery and her inferior vena cava, most of the gross tumor was resected, and the margin was positive. A CT scan revealed a liquid dark area in the surgical area after the operation Figure B.A cut section revealed a homogenous solid mass with a rough surface. Multiple sections were taken from various areas of the tumor. Microscopic examination revealed that the tumor was composed of fat to long spindle cells with multinodular infiltration that were arranged in a storiform pattern Figure . The tumShe received postoperative sequential chemotherapy and radiotherapy. Three cycles of chemotherapy with a standard dose of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) were initiated in July 2010. A total radiation dose of 60Gy was then administered over 6 weeks, followed by another three cycles of chemotherapy with CHOP. To evaluate the efficacy of the adjuvant therapy, an abdominal CT scan was performed, which revealed that the liquid dark area had shrunk significantly (Figure Hospital records indicate that the patient has reached 3 years of follow-up. Although she is alive, she has a right pulmonary metastasis.et al.[FDCS is a rare tumor that originates in the germinal centers of the lymph nodes in FDCS and has low malignant potential. Monda et al. first deet al.. The moset al.,6-9.Pathological diagnosis is challenging  and may A complete surgical excision is the appropriate initial therapy . AdjuvanAs in this patient, retroperitoneal FDCS may demonstrate aggressive potential. This study indicated that postoperative adjuvant radiotherapy and chemotherapy could extend the survival of a patient with retroperitoneal FDCS.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.CHOP: Cyclophosphamide, doxorubicin, vincristine and prednisone; CT: Computed tomography; FDCs: Follicular dendritic cells; FDCS: Follicular dendritic cell sarcoma.The authors declare that they have no competing interests.TY and QY contributed equally to this work; TY, QY and XZ analyzed and interpreted the data from our patient and drafted the manuscript. HZ and JL performed the histological examination of the tumor. All authors read and approved the final manuscript.


Leiomyosarcoma (LMS) of the gastrointestinal tract is an extremely rare high-grade neoplasm with poor prognosis. For advanced LMS with distant metastasis, the decision as to the choice of the most appropriate therapeutic strategy, including chemotherapy and surgery, is difficult. Here, we present an unusual case of LMS of the sigmoid colon with liver metastases and gastric cancer. The survival of this patient was prolonged by a combined modality therapy involving chemotherapy and surgery.A 66-year-old woman who had been diagnosed with advanced gastric cancer and multiple liver metastases was referred to our hospital. The initial treatment with docetaxel and S-1 considerably reduced both the gastric cancer and liver tumors; consequently we performed surgical resection. Pathological examination revealed that no viable tumor cells remained in the stomach and chemotherapy resulted in complete remission of the gastric cancer. The liver tumors were immunohistochemically diagnosed as LMS. A tumor of the sigmoid colon was subsequently discovered and the liver tumors were found to have recurred. The surgically resected sigmoid colon and liver tumors were all immunohistochemically diagnosed as LMS. These findings indicated that the multiple liver metastases arose from the LMS in the sigmoid colon, and that they were accompanied by advanced gastric cancer. We performed another surgical resection and administered chemotherapy to treat the recurring liver metastases. The patient survived for 4 years and 10 months after initial presentation at our hospital.Colonic LMS is rare and its joint occurrence with gastric cancer is extremely unusual. Although LMS is a high-grade neoplasm, a multimodal therapeutic approach can increase patient survival time even when multiple liver metastases are present. Leiomyosarcoma (LMS) of the gastrointestinal (GI) tract is extremely rare, and only a few reports have been published in reviews of GI mesenchymal tumors ,2. LMS fA 66-year-old woman diagnosed with advanced gastric cancer and multiple liver metastases was referred to our hospital in March 2003. She was not a carrier of the hepatitis virus, or an alcoholic with previous hepatic disease. Furthermore, she did not have a family history of malignant neoplasia. Gastrointestinal fiberscopy upon admission showed an irregular ulcerative lesion on the anterior wall of the gastric corpus Figure , and theWe administered chemotherapy with docetaxel and oral S-1 to treat the gastric cancer and liver metastases . SeventeAscites and peritoneal disseminated lesions were not evident during the procedure, which included total gastrectomy and partial liver resections for all liver tumors. Pathological examination revealed that no viable tumor cells remained in the stomach and chemotherapy resulted in complete remission of the gastric cancer. The liver tumors were immunohistochemically positive for smooth muscle actin (SMA), desmin and h-caldesmon, and negative for c-KIT, CD34 and S-100  during this therapeutic process; this gradually increased in size to 3 cm in diameter. Colonoscopy revealed a lesion protruding from the submucosa with a normal mucosal surface Figure . AlthougA second surgical resection of the four liver tumors and a partial colon resection proceeded at 2 years and 5 months after the commencement of first-line chemotherapy. The sigmoid colon and liver tumors were all immunohistochemically diagnosed as LMS Figure . This fiThe patient underwent a third liver resection to treat two new liver metastases that developed at 7 months after the second procedure. This was because we considered that there might be only a limited chance of a good response to chemotherapy in the case of the LMS, and all of the liver metastases were also completely resectable. Thereafter, despite ifosfamide and epirubicin chemotherapy followed by dacarbazine, multiple liver and lung metastases eventually developed and the patient died at 4 years and 10 months after the first presentation at our hospital.The incidence of LMS in the GI tract is extremely rare and GIST account for most GI mesenchymal tumors. Recent reports describe the classification of only three among 262 GI mesenchymal tumors as being LMS , and onlThe reported clinical features of LMS of the GI tract are gross polypoid and intramural types that can arise from either the muscularis mucosae or the propria ,2,9. NeiKIT mutations [The histogenetic, clinicopathological and immunohistochemical profiles of LMS and GIST differ . Whereasutations ,6,7. An utations ,11. The Moreover, in the diagnosis of the present case, LMSs in both the liver and colon were possibly metastatic tumors that originated from primary LMS in another organ. Actually, LMS of the left thigh that metastasized to the liver, large bowel and lymph nodes has been reported . HoweverSurgical resection is the most frequent approach to treating LMS ,6-8. OneChemotherapy generally plays a limited role in the treatment of LMS ,14. FurtWe initially considered that the liver tumors were metastases arising from gastric cancer. However, we resected the stomach and liver tumors because all of the tumors remained small. They proved to be completely resectable and de novo lesions did not appear during the first year of chemotherapy. Had the first chemotherapy regimen been ineffective and had the number of liver tumors and/or the size of the gastric cancer increased, surgical resection in our patient would not have been indicated. This was because of the fact that these factors would have pointed to a decreased probability of complete resection being achievable.RASSF1A, were also indicated in the occurrence of these tumors [The tumorigenesis of gastric cancer and LMS has been reported to involve various factors -22. Comme tumors ,24. Furte tumors . Intragae tumors . In the Colonic LMS is rare and its occurrence in combination with gastric cancer is extremely unusual. Although the diagnosis in our patient was complicated by the presence of both gastric cancer and LMS, an immunohistochemical study of surgical specimens confirmed the final diagnosis of LMS of the sigmoid colon with multiple liver metastases. We concluded that the multimodal approach comprising chemotherapy and complete surgical resection controlled the LMS, even with multiple liver metastases present, and improved the survival of this patient.Written informed consent was obtained from one of the patients’ relatives for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Series Editor of this journal.CT: Computed tomography; GI: Gastrointestinal; GIST: Gastrointestinal stromal tumor; LMS: Leiomyosarcoma; PET-CT: Positron emission tomography/computed tomography; SMA: Smooth muscle actin.The authors declare that they have no competing interests.All of the authors have read and approved the final manuscript. Dr YH was responsible for the design and drafting of the manuscript; Drs JH and KT were responsible for the conception and revision of the manuscript, and also the pathological diagnosis; Drs ME and YA carried out the surgical operation and clinical management of the patient; Dr KK undertook the pathological diagnosis; and Dr MO was responsible for the final review and revision of the manuscript and the supervision of the study.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/12/98/prepub