Solitary fibrous tumors (SFTs) of the liver are rarely described; only 38 cases have been reported in literature, most of which have shown benign clinical characteristics, and only 3 of these cases exhibited malignant variants. In this study, we present a 24-year-old woman with a 1-month history of a rapidly enlarging abdominal mass and a CT showing an exophytic heterogeneous liver mass with a firm parietal bone mass. The patient underwent a transcatheter arterial chemoembolization (TACE) before operation, and an extended right hepatectomy and craniectomy with a negative margin was performed under general anesthesia. The masses showed histological features of oval spindle cells haphazardly arranged in the classic short-storiform or so-called patternless pattern of solitary fibrous tumors. The tumor cells showed positive immunohistochemical reactions to CD34 and bcl-2. The tumor recurred in the residual liver 2 months after operation, metastatic osteoblastic lesions in the thoracic and lumbar vertebrae were identified 3 months after the operation, and lumbar vertebrae metastasis 7 months after operation paralyzed the patient. The patient underwent percutaneous ethanol injection therapy (PEI) and chemotherapy, but the patient died because of the uncontrolled tumor 16 months after the initial operation. To our knowledge, this is the first case of malignant solitary fibrous liver tumors with skeletal metastasis. Solitary fibrous tumors (SFTs) are a rare soft-tissue neoplasm that was initially described most commonly in the pleural cavity. In the More than 80% of SFTs are benign, asymptomatic and slow-growing tumors, but malignant and symptomatic forms, most often in the pleura, have been described ,7. CliniHowever, surgical experience with SFTs of the liver is notably limited. In one series, 13.5% of 37 pleural SFTs metastasized . SFT isA 24-year-old, unmarried, nulliparous woman had presented one month earlier with upper right abdominal discomfort, distention, and vague right quadrant pain. The patient was a shop assistant for 4 years with no known history of potential environmental hazardous substances exposure. In addition, she was also a nonsmoker. Except for being a carrier of hepatitis B, she had no relevant medical or family history. For the further evaluation of her symptoms, she was referred to the referral hospital, where a contrast enhanced computer tomography (CT) study revealed a 30-cm exophytic heterogeneous mass in the right lobe of the liver Figure . In a 2-On examination, abdominal distension was present. Palpation revealed tenderness and a large, firm mass in the right hypochondrium and epigastrium, and the liver was 12 cm below the lower edge of the ribs. There was no evidence of ascites, edema, hepatic failure, or cirrhosis stigmata. A firm mass of 4 cm×5 cm×3 cm was present on the right parietal bone without any tenderness  of the tumor-supplying vessels a few days before operation Figure . The resA postoperative MRI examination in July 2010 demonstrated that the postsurgical residual lesion was still large. At follow-up one month later, the patient had severe back and waist pain. An MRI identified metastatic osteoblastic lesions of the thoracic and lumbar vertebrae Figure .The patient underwent four percutaneous ethanol-injection therapies (PEIs) and was treated with four rounds of chemotherapy that included adriamycin (40 mg on days 1-3), ifosfamide (3.0 g on days 1-4), and mesna . Adjunctive radiation therapy was not administered. Despite the treatment, the tumor relapsed rapidly and was markedly enlarged. At follow-up seven months later, the patient was paralyzed; the patient died 16 months after the initial operation.Hepatic SFTs are unusual neoplasms, with 38 previous cases worldwide being reported in the literature. SFTs typically affect adults aged 54.6 years 16-83) with no underlying predisposing factors. In a review article of 14 published cases, a strong female predilection was noted, and the average follow-up period was 27 months ,18. Most6-83 withClinically, hepatic SFTs cause symptoms only after reaching a certain size, or when vital structures are involved. SFTs are not known to grow invasively, and the course of the disease following complete surgical resection is usually benign. The patient may be asymptomatic or have non-specific clinical manifestations, including increased abdominal volume and circumference and abdominal pain, distention, and discomfort, as seen in our patient, or other symptoms, such as anorexia, nausea, vomiting and weight loss. Fever, hypoglycemia, abnormal liver tests and biliary-duct compression that leads to cholestasis and progressive jaundice are less commonly seen ,13,16,19The radiological findings of SFTs are unspecific and cannot distinguish between benign and malignant tumors. The diagnosis should be suspected if imaging reveals a solitary, highly vascular, well circumscribed, encapsulated mass that shows heterogeneous enhancement (due to the varied density of the collagen component) in CT and MRI images, especially if the enhancement is hypointense on the T2-weighted MRI and shows progressive enhancement in the delayed phase . Most beSeveral authors believe that fine-needle aspiration also helps to establish a diagnosis, if the tissue sample is adequate and the fine-needle aspiration can be analyzed for immunomarkers; there has been one successful case report of this practice . Other aThe tumor may be found on either the right or left side of the liver. The average tumor weight, where available, was 3,184 g and ranged from 1,850 g to 4,500 g. The mean of the largest diameter was 17.8 cm (2-32 cm) presenteIn the present case, the liver SFT diagnosis was based on the association of characteristic histological and immunohistochemical features  with the immunohistochemical staining profile of CD34 (+), Bcl-2 (+) and SM-actin (-). These characteristics differentiate SFTs from other liver tumors, such as primary hepatocellular carcinomas (CD34-negative), leiomyomas (smooth-muscle actin-positive and CD34-negative) and mesotheliomas  . HoweverA hepatic SFT is often a benign neoplasm that has a good prognosis after surgery without recurrence. Most authors believe that postoperative adjuvant chemotherapy or radiotherapy should be reserved for incomplete resections and/or pathological features of malignancy ,26. CurrWhen complete resection is impossible or when there is recurrence, radiotherapy can be used, but a negative surgical margin does not mean that radiotherapy is unnecessary. Postoperative radiotherapy may theoretically reduce the risk of local recurrence; therefore, postoperative radiotherapy should be used as an adjunctive therapy in all patients . In our SFTs of the liver are a rare neoplasm that cannot be definitely diagnosed by characteristic histological and immunohistochemical properties. A complete surgical resection with tumor-free margins is the best choice for treatment. Postoperative adjuvant chemotherapy or radiotherapy should be used in malignant SFTs; however, the use of these therapies in benign SFTs is still controversial.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.QL participated in the design of the study, and drafted the final version of the manuscript. LP participated in design of the study and manuscript drafting, carried out histopathological evaluation. YL participated in histopathological evaluation and helped in drafting the manuscript. YA tracked the clinical data. ZL helped in drafting the manuscript. YH tracked the clinical data. All authors read and approved the final manuscript.


A 72-year-old man presented with painless frank haematuria. Investigations included intravenous urogram and abdominal/pelvic CT which revealed a marked focal thickening of the wall of the inferior aspect of the left renal pelvis extending into the lower pole calyx and into the pelviureteric junction resulting in left hydronephrosis. Urine cytology demonstrated clusters of malignant keratinised squamous cells and schistosome ova. He underwent left laparoscopic radical nephroureterectomy and histology revealed moderately differentiated keratinising squamous cell carcinoma in the renal pelvis. Schistosoma haematobium there is a strong association with urinary tract pathology [In countries with a high prevalence of athology . A recogathology . Schistoathology . In nonschistosomiasis endemic areas, SCC of the urothelium is rare and typically occurs in association with chronic inflammation associated with long-term indwelling catheters, urinary tract calculi, bladder diverticula, and chronic urinary tract infections. Only 5% of all urothelial tumours present in the upper urinary tract . The Swedish Cancer Registry, Hölmang et al. , reportsSCC of the upper urinary tract is a rare occurrence, and an association of SCC of the renal pelvis with schistosomiasis has not been previously described. We present a case of SCC of the renal pelvis which was diagnosed by urine cytology with schistosome ova alongside malignant keratinised squamous cells.Schistosoma infection. Urine cytology is a valuable tool in the investigation of patients with haematuria and is pivotal in the diagnosis of urogenital schistosomiasis. Management depends on the extent of organ involvement but in the absence of metastatic disease, usually involving radical surgical resection with systemic treatment for schistosomiasis.Squamous cell carcinoma of the renal pelvis is a rare complication of A 72-year-old man presented with two episodes of painless frank haematuria. Preceding lower urinary tract symptoms only consisted of mild hesitancy and straining with a moderate stream. There was no other significant urological history. He had smoked previously for 40 years at three packs a day. He was a submariner during the Second World War and then spent 30 years allegedly travelling in South America.Ultrasound was reported as normal besides a mildly enlarged prostate. Flexible cystoscopy demonstrated a tri-lobar obstructing prostate with normal bladder urothelium. An abnormal intravenous urogram showed dilatation of the left pelvicalyceal system with blunting and fullness of the calyces. There was an unusual crescentic shape enhancement seen inferiorly at the left renal pelvis . A CT scThere was no growth on culturing mid-stream urine. Urine cytology revealed clusters of malignant keratinised squamous cells and schistosome ova Figures . He was The patient presented five months later with multiple lung lesions. He declined chemotherapy and unfortunately died 10 weeks later.S. mansoni, S. hematobium, and S. japonicum [S. hematobium due to adult schistosomes producing ova in venules of the genitourinary tract. Although involvement of urogenital organs differs markedly, it appears to correlate with the degree of venous circulation. Hence, the urinary bladder, lower ends of ureter, and seminal vesicles are most commonly affected owing to a rich venous supply [Schistosomiasis is a parasitic infection that infects 250 million people worldwide. Ten species of schistosomes can infect humans, including s supply . S. hematobium infection, particularly the endopelvic part of the ureter. Whilst SCC of the bladder is well described in the literature, SCC of the upper urinary tract in association with S. hematobium infection is rarely cited [In the upper urinary tract, ova can be found in all layers of the ureter and can lead to fibrosis and stricture formation . Ureterily cited .Squamous metaplasia is a precursor for malignant change and associated with chronic inflammation of the urothelium. Case reports of upper tract nonschistosomiasis SCC, not unsurprisingly, describe predisposing factors associated with chronic inflammation, in particular nephrolithiasis . SeveralS. haematobium ova in the urine. The ova count can be used to assess the severity of infection, and the viability of ova in any given sample has been assessed using Congo Red [In this case, diagnosis of urinary schistosomiasis is based on the detection of ongo Red . Novel dongo Red . Cystoscongo Red . S. hematobium. Praziquantel induces tetanic paralysis and decreases glucose absorption in the trematode by interfering with ionic exchange. Metrifonate is an organophosphate that also causes paralysis by irreversibly blocking cholinesterase [Praziquantel or metrifonates are systemic treatments of choice in esterase . Surgical management of upper tract SCC associated with schistosomiasis should follow the same principles as for any upper tract SCC. Radical nephroureterectomy is the treatment of choice for patients without metastasis and there appears to be only little benefit for neoadjuvant and adjuvant radiotherapy/chemotherapy . The majIn those patients at risk for upper tract SCC, a high index of suspicion should be maintained to improve detection of disease at an earlier stage. In a case series which included nephrectomies performed for nonfunctioning kidneys secondary to staghorn calculus, the first indication of malignancy was the incidental finding of SCC on histological examination of the nephrectomy specimen . This fu


Spontaneous splenic rupture is well recognized in the context of hematological malignancies (lymphoproliferative and myeloproliferative disorders); a few case reports have also linked solid tumors, such as pancreatic and liver cancer, with the occurrence of spontaneous splenic rupture. This is the first case report of lung cancer as a likely cause of spontaneous splenic rupture.A 61-year-old Caucasian woman presented to our hospital with non-specific symptoms. She developed an 'acute' abdomen and went into a state of shock within twelve hours of her presentation. She was diagnosed with spontaneous splenic rupture with radiology and following a laparotomy. She made an uneventful recovery postoperatively and was simultaneously found to have a bronchial adenocarcinoma.Spontaneous splenic rupture is a potentially fatal but often unrecognized cause of acute abdomen. It should be routinely considered in the differential diagnosis of acute  abdomen and when present it should be promptly dealt with, most commonly with a laparotomy. Once the diagnosis is confirmed there should be an aggressive drive to identify an underlying etiology; malignancy is the commonest culprit. Solid tumors should be considered as underlying causes despite being less common than hematological neoplasms. This case report demonstrates lung malignancy as an underlying precipitating cause of spontaneous splenic rupture. Splenic rupture is a rare albeit potentially catastrophic event that causes an 'acute' abdomen and hemodynamic instability. It should be urgently investigated, diagnosed and treated, often with splenectomy and less often with conservative management or with splenic artery embolization.Splenic rupture can be divided into traumatic (or non-spontaneous) and atraumatic (or spontaneous). Diagnostic criteria were developed by Orloff and Peskin in 1958 [Spontaneous splenic rupture (SSR) can be subdivided further into true and pathological rupture corresponding to normal and pathological appearances of the spleen on histological examination. Therefore, in essence, the original Orloff and Peskin criteria describe 'true' SSR. A systematic literature review  of 845 cHematological malignancies  comprise the majority of the neoplastic causes, with solid tumors rarely reported in the literature (such as hepatocellular  and pancThe investigation of choice for confirming the diagnosis is computed tomography (CT) of the abdomen , which het al. [In a systematic review by Renzulli et al.  splenectet al. .A 61-year-old Caucasian woman, previously in good health, presented with a three-day history of feeling generally unwell with dizziness, vomiting, abdominal, left lower chest pain and shoulder pain. She denied any sore throat, feeling feverish or other symptoms suggestive of an influenza-like illness. There was no cough or sputum production. There was nothing in the history to suggest a recent viral or other infective process including human immunodeficiency virus or acquired immune deficiency state and no history of any trauma or injury. She had no significant previous medical or surgical history and was not taking any medications. There was no background or family history of cancer, hematologic or clotting disorders. She was a smoker of approximately 40 pack years and had unlimited exercise tolerance. She worked as a nurse and there was no history of previous exposure to asbestos or other occupational hazards.On examination she was ill-looking, conscious and orientated with a blood pressure 89/49 mmHg, heart rate of 72 beats per minute, saturations 97% on a non-rebreather mask and temperature of 36.9°C. The admission examination revealed normal cardiac examination and bibasal inspiratory crepitations with left upper quadrant and epigastric tenderness on abdominal examination.9/L with neutrophils of 17 × 109/L and a C-reactive protein of 7 mg/L. Urea and electrolytes, liver function and coagulation tests were in the normal range. A blood film examination did not reveal any atypical lymphocytes or other abnormalities. A posteroanterior and lateral chest radiograph was performed . Inspection revealed skin and conjunctival pallor. Clinical examination revealed new abdominal signs of generalized abdominal tenderness with guarding, rigidity and absent bowel sounds, consistent with an acute abdomen. A repeat test showed her hemoglobin level had dropped to 5.0 g/L.She was stabilized with multiple blood transfusions and underwent an urgent computer tomography scan of her thorax and abdomen, which showed an 8.5 × 3.6 cm left hilar mass with extensive mediastinal adenopathy, bibasal small effusions and consolidation, and a large splenic hematoma of 15 × 12 cm with high attenuation suggestive of active bleeding Figure . There wPneumococcal and meningococcal vaccines were administered and our patient was then promptly taken to theater for laparotomy. On examination of her internal organs at laparotomy, other than hemorrhage and rupture of her spleen, there was no other gross abnormality and no evidence of disease in her other intra-abdominal organs. She spent two days being ventilated in our intensive care unit and subsequently made a full recovery from the surgery. She was commenced on life-long penicillin V and was subsequently discharged home a week after admission, fully mobile and independent in terms of her activities of daily living.Her splenic histology was negative for hematologic or other malignancy and no other pathology was identified. She then underwent a bronchoscopy; transbronchial needle aspiration revealed numerous malignant nodes consistent with non-small cell carcinoma. Histology of bronchial biopsies confirmed invasive adenocarcinoma. This was consistent with likely stage IV in view of the likely bone metastases found on CT. She was referred to the local oncologist and her case was discussed at the lung cancer multidisciplinary team meeting. In view of the diagnosis and staging, a palliative treatment pathway was agreed from the outset and our patient received palliative chemotherapy. She died five months after her presentation.To the best of our knowledge, this is the first case report of SSR in association with lung cancer. Lung cancer with splenic rupture has been reported either in the context of splenic metastasis , after tThe mortality rate for conservatively managed spontaneous splenic rupture was 22% at 30 days; mortality was 30% versus 5% for malignant and benign underlying disease, respectively . The traIt is possible that our patient had two concurrent, unrelated pathologies: bronchial adenocarcinoma and a true SSR. However, given the well-described association between neoplastic disease and SSR, the above case reports linking the two conditions and the rarity of true spontaneous splenic rupture, it is likely that the two conditions are linked with the former precipitating the latter.Our case illustrates the need for extra vigilance to make the diagnosis of splenic rupture in the context of a lack of trauma. The exact pathophysiology that links lung cancer with SSR (in the absence of splenic metastasis or use of chemotherapy or pegfilgrastim) remains unclear. The possible explanations include a hypercoagulable state secondary to the underlying malignancy.In summary, this case demonstrates that lung cancer could potentially precipitate SSR; it could even present itself as SSR as in our patient. This association is of relevance to a wide variety of health care professionals including acute and general physicians, general surgeons, oncologists, radiologists and others. The other important learning point from this case is that although SSR is a rare cause of acute abdomen, we should bear in mind that it is fatal if misdiagnosed and left untreated; it should therefore be considered in the differential diagnosis of a wide range of medical and surgical conditions.Written informed consent was obtained from the patient for publication of this manuscript 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.AK contributed to the medical care of this patient, the manuscript preparation and revision, and also performed the literature review. NA contributed to the medical care of this patient, the manuscript preparation and revision. HV contributed to the radiological review of the case report and the manuscript revision. All authors read and approved the final manuscript.


EWSR1 and WT1 genes. The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease. Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation. Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [90Y]Yttrium microsphere liver embolization.Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated with a unique chromosomal translocation t(11:22)  that involves the  Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by Gerald and Rosai Yttrium microspheres given by hepatic artery embolization with evident metabolic response on PET-CT [Recently, Subbiah et al. reported the case of a young patient with hepatic metastasis of DSRCT resistant to chemotherapy successfully treated with [n PET-CT . Given tDespite its rarity, several new procedures have been tested in DSRCT in this particularly severe tumor affecting children, without evidence of clinical utility. The combination of Ewing-sarcoma-based polychemotherapy and debulking surgery represent the standard of care as of early 2012. Not surprisingly, those patients having successful surgical debulking and responsive disease to chemotherapy appear to have the best outcome compared to groups of patients who do not achieve both favorable outcomes. The impact of new techniques such as HIPEC or IMRT needs to be clearly defined, ideally in the context of prospective randomized clinical trials since the retrospective data to date give no sense of a positive survival signal. Whole genome sequencing of DSRCT is ongoing to identify mutations, single nucleotide polymorphisms or copy number changes associated with these tumors to explore pathogenesis and open medical therapeutic possibilities.


Echinococcus infestation is endemic.A solitary primary hydatid cyst in the subcutaneous abdominal wall is an exceptional entity, even in countries where the We report a case of a 70-year-old Caucasian woman who presented to our hospital with a subcutaneous mass in the para-umbilical area with a non-specific clinical presentation. The diagnosis of subcutaneous hydatid cyst was suspected on the basis of radiological findings. A complete surgical resection of the mass was performed and the patient had an uneventful post-operative recovery. The histopathology confirmed the suspected diagnosis.Hydatid cyst should be considered in the differential diagnosis of every subcutaneous cystic mass, especially in regions where the disease is endemic. The best treatment is the total excision of the cyst with an intact wall. Echinococcus granulosis, the life cycle of which has been well described [Hydatid disease is a parasitic infestation that is caused by escribed . Endemicescribed ,2. The lescribed . The solA 70-year-old Moroccan Caucasian woman presented to our hospital with a subcutaneous cystic mass in the right para-umbilical abdominal wall which had been evolving for six months. Her physical examination revealed an abdominal parietal mass 6 cm in diameter that was palpated 5 cm to the right of the umbilicus. It was cystic, fluctuant, mobile, and painless. The overlying skin was normal. An abdominal ultrasound showed a rounded cystic mass that was limited within the right para-umbilical abdominal wall and measured 60 mm. No other abdominal cystic mass was found. The pre-operative examinations  revealed no abnormalities. The hydatid serology was negative. Surgical exploration revealed that the mass was attached to the subcutaneous adipose tissue but was not associated with any muscular or cutaneous structure Figure . The macThe mechanism of the primary subcutaneous localization is unclear ,4. The iIn our case, the hydatid cyst was located subcutaneously. The patient had not undergone previous surgery for any hydatid cysts, which were never found in other organs. Therefore, our patient was diagnosed as having a primary subcutaneous hydatid cyst.In a large series of patients from Greece, the frequency of extra-hepatic and extra-pulmonary hydatidosis was 9% . HoweverThe clinical course is non-specific and depends on the site of involvement, the size of the cyst, and the pressure caused by the enlarged cyst . UsuallyRadiological imaging  is useful in rendering the diagnosis, showing the size, localization, relationship to adjacent organs, and type of the cyst. It can also be used to search for another hydatid location ,4. The rThe best treatment option is complete surgical excision of the intact cyst, which avoids leakage of cyst content that can cause anaphylaxis and local recurrence ,2,8. If Hydatid cyst should be considered in the differential diagnosis of every subcutaneous cystic mass, especially in regions where the disease is endemic. The best treatment is the total excision of the cyst with an intact wall.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.AO, KA, and HE operated on the patient. KHI took the photos. KM participated in follow-up. All authors participated in writing the case report and revising the draft. All authors read and approved the final manuscript.


Teratomas are neoplasms characterised by an abnormal growth of tissues derived from the three germinal layers. The term ‘teratoma’ is derived from the Greek root ‘teratos’, meaning monster. Germ cells develop in the embryo and subsequently become the cells that make up the reproductive system. During fetal development, these cells follow a midline path and descend into the pelvis as ovarian cells or the scrotal sac as testicular cells. The presence of germ cells in extragonadal sites is because of the failure of these cells to migrate along the urogenital ridge. Therefore, teratomas occur in order of decreasing frequency in the ovaries, testes, anterior mediastinum, retroperitoneum, sacrococcygeal region and cranium.Liver teratomas are very rare; of the 25 hepatic teratomas described in the literature, only five have occurred in adults. The majority of the cases were in female children below the age of three, mostly arising in the right lobe of liver.We report a case of an adult male with benign mature teratoma arising in the left lobe of liver, compressing the common bile duct and causing obstructive jaundice. A 46-year-old man was admitted for progressive jaundice and pyrexia occurring arbitrarily for a month. He also complained of episodes of chills and rigors, and showed significant weight loss. The patient was passing pale coloured stools and had pruritus. He also had a history of vomiting associated with upper abdominal pain. There was no history of bleeding tendencies, and the patient claimed to be non-alcoholic. His previous medical history was normal.On clinical examination, he revealed jaundice with scratch marks seen on the skin of the arms and abdomen. His urine was dark coloured, and there was a palpable tender firm epigastric mass extending to the right hypochondrium.9 and platelet count of 320 x 109. Hepatitis serology was negative, and the diagnosis was obstructive jaundice secondary to possible pancreatic malignancy.The blood examination was consistent with obstructive jaundice: total bilirubin 99 mmol/L (3-17 mmol/L) conjugated bilirubin 93 mmol/L (0-3 mmol/L), alanine transaminase 127 IU/L (30-65 IU/L), aspartate transaminase 50 IU/L (15-37 IU/L), alkaline phosphatase 196 IU/L (50-136 IU/L) and GGT 124 IU/L (15-85 IU/L). A full blood count showed a haemoglobin level of 127 g/L, white blood cell count of 11.7 x 10An ultrasound examination revealed a heterogenous solid mass measuring 5.0cm x 6.5cm x 8.0cm with echogenic areas in the epigastrium, medial to the gallbladder. The left lobe of liver was atrophic, and the intra and extra hepatic biliary ducts were of normal calibre.A contrast enhanced computed tomography (CT) examination  and 2 reMagnetic Resonance Cholangiopancreaticography (MRCP) was performed to further evaluate the biliary system and characterise the lesion. The mass, seen in the CT examination, was identified as a mixture of fat and soft tissue measuring 8.5cm x 6.3cm x 6.7cm. There was no communication between this mass and the biliary tree  and 4. Ten mass with the tumour and the atrophic left lobe of liver. The operating surgeons were of the opinion that the mass could represent an angioliposarcoma, because of its gross appearance.Exploratory laparatomy was performed the same day and revealed a fatty encapsulated tumour lying below segment IV of the liver compressing the CBD. The hepatic artery was seen coursing through the mass, and had to be ligated before it was successfully resected The gallbladder was also removed. The rest of the liver, mesenteric vessels, spleen and bowels were normal. Gross pathologic examination showed the tumour mass attached externally to a piece of liver tissue, which was resected. The cut section showed fatty tissue, cystic areas containing sebaceous material and hair admixed with a firm fibrotic tissue. The resected hepatic artery was thick and fibrotic with a short course draining into the tumour.The histology showed a mixture of mature adipocytes, hyalinised fibrous tissue, bony trabeculae and calcification. The attached liver showed cholestasis. The final pathologic diagnosis was mature benign teratoma. An ultrasound of the testes was performed and found to be normal. The patient was discharged and is currently on follow up at the surgical clinic.Teratomas are congenital neoplasms characterised by an abnormal growth of a combination of tissues derived from ectodermal, mesodermal and endodermal germ layers. This combination of tissues is unrelated to the organs where the tumour originates . These pThese tumours mostly occur in the ovaries or the sacrococcygeal region in children. Less common anatomical locations include the testes, mediastinum and central nervous system. There have been rare cases of these tumours occurring in gastrointestinal tract, liver, nasal sinuses, cervix and thyroid .Teratomas of the liver are rare neoplasms accounting for less than 1% of all teratomas. Even in paediatric patients where they are most commonly seen, they account for less than 1% of all liver neoplasms. In adults, benign teratomas do not directly affect the general condition of the patient; compression of the surrounding structures causes symptoms of abdominal distension, nausea and vomiting.Occasionally acute abdominal pain can develop with malignant neoplasms, which are thought to cause rapid degeneration . Hepaticen masse as it was encased within the tumour. A left hepatectomy was subsequently performed.In our patient, although the histology did not demonstrate tumour cells of hepatic origin, during the surgery, it was evident that the teratoma originated from the left lobe of liver in view of its common peritoneal attachments as well as capsular encasements. The CBD was compressed by the large tumour, which explained the occurrence of jaundice. The hepatic artery had to be ligated before it was resected As previously mentioned, a teratoma is histologically composed of a variety of tissue elements. In our case, the histology revealed a mass containing solid adipose tissue, bony trabeculae, hair, fibrillary neural tissue and calcification.Plain radiographs usually demonstrate a soft tissue mass with lucent fat opacity and calcifications, either rim like or chunky. An ultrasonography may demonstrate a hypoechoic  or anechoic (representing the cystic portion of this mass) component.Calcification demonstrates posterior acoustic shadowing. Bony tissue and calcification appear as high density foci on CT while fat is readily identified as hypodense with a HU of -60 to -100, demonstrating a density similar to subcutaneous fat. CT permits unequivocal identification of fat as adipose tissue and the characterisation of fluid as sebum, serous or complex. A component of a tumour that has both a CT attenuation value of fat and a horizontal interface with another more dependent type of fluid, most likely represents sebum .In magnetic resonance imaging (MRI), calcification and bone produce no signal. Solid adipose tissue is of high signal intensity on both T1 and T2-weighted images. Fluid components are typically hypointense on T1 and hyperintense on T2-weighted images. The dermoid plug or Rokitansky protuberance is described by Anderson and Kissane  as an ouMRCP was useful in this case to demonstrate the compression of the CBD by the adjacent teratoma. Furthermore the delineation of the large vessels and the relationship of surrounding structures, as seen on the MRI was helpful in surgical planning, because all teratomas should be removed completely if possible, regardless of its benignity or malignancy.In conclusion, the case described here represents a rare case of liver teratoma with interesting and characteristic CT and MRI correlation.


Primary spinal non-Hodgkin's lymphoma is extremely rare, and the occurrence of spinal dumbbell-shaped lymphoma is exceptional. We present a case of primary spinal dumbbell-shaped lymphoma to clarify the diagnosis and the management of these lesions. A 45-year-old man presented with sensory symptoms for 8 months. Magnetic resonance imaging of the thoracic spine demonstrated a dumbbell-shaped lesion at the D4–D6 level with spinal cord compression and right foraminal extension at D4–D5 level. The patient underwent D4–D6 laminectomy, with a subtotal resection of the mass. Diffuse large B-cell lymphoma was diagnosed in the pathological examination. He underwent local spinal radiotherapy and chemotherapy. Follow-up evaluation at one year demonstrated no evidence of relapse. Although highly unusual, lymphoma should be included in the differential diagnosis for spinal dumbbell-shaped tumours. After surgery and adjuvant therapy a long-term clinical and neuroradiological followup is mandatory. Spinal cord compression during the course of non-Hodgkin's lymphoma (NHL) is well described in the literature and occurs in 0.1 to 10.2% of patients –5. Here A 45-year-old man presented with thoracic back pain since 8 months. The medical history indicated no abnormalities. At the initial clinical examination, the neurological status was normal. Computed tomographic (CT) scan and magnetic resonance (MR) imaging demonstrated a dumbbell-shaped enhancing lesion at the D4–D6 level compressing the spinal cord Figures  and 2. TThe patient underwent a D4–D6 laminectomy with the removal of a soft grayish epidural mass, suggestive for epidural lymphoma. The paraspinal component was subtotally resected.Light microscopy revealed diffuse large B-cell lymphoma with follicular G3 areas . The immThe postoperative course was uneventful. The staging evaluation revealed no systemic disease. The patient was treated with local spinal radiotherapy and chemotherapy (CHOP-14 for six cycles). At the last followup, one year after surgery, the patient was neurologically intact and restaging performed at this time demonstrated no evidence of relapse .The term “dumbbell-shaped tumor” traditionally refers to tumors with a combination of intraspinal and paravertebral involvements showing an hour glass shape. This term has broadened with time to include tumors connecting two or more separate anatomical regions such as intradural, extradural, and paravertebral spaces . The dumDumbbell tumors include neurogenic neoplasms , nonneurogenic tumors (meningiomas and sarcomas), and hematopoietic neoplasms (lymphoma or solid leukemic infiltrates). Although this wide histopathological variety, 86% of dumbbell-shaped tumors are schwannomas (69%), neurofibromas (12%) and meningiomas (5%) [Primary spinal dumbbell-shaped NHLs are extremely rare, and including our report four reported cases documented an extraforaminal component associated with an epidural lymphoma –5. ThreePreoperative diagnosis of spinal dumbbell-shaped lymphomas is difficult and requires a high index of suspicion. In fact, on the basis of preoperative MRI, two of the four patients were thought to have a nerve sheath tumor. MR imaging features in primary lymphomas of the CNS include a hypointense appearance on T1-weighted images, a hyperintense appearance on T2-weighted images, and homogeneous enhancement after the administration of contrast material . HoweverAlthough highly unusual, lymphoma should be included in the differential diagnosis for spinal dumbbell-shaped tumours. Soft tissues infiltration in the paraspinal space provides a clue for preoperative diagnosis. After surgery and adjuvant therapies a long-term clinical and neuroradiological followup is mandatory.


About 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated endocrine tumors account for only 2.6% of all neuroendocrine tumors. Following the first two case reports of somatostatin-secreting tumors in 1977, fewer than 200 cases of somatostatinoma have been reported. These tumors of the duodenum are usually silent and asymptomatic, but can cause gastrointestinal symptoms. Depending on the localization of the tumor, multiple surgical procedures can be performed, ranging from local resection to pancreaticoduodenectomy.Here, we report a case of a submucosal duodenal mass in a 42-year-old Turkish White man presenting with nausea, vomiting, fatigue and abdominal pain. The treatment decision of pancreaticoduodenectomy made preoperatively was later altered to intraoperative removal via local resection with sphincteroplasty.Tumors of the periampullary region are considered highly malignant, and the Whipple operation is usually the only procedural treatment. In the current case, we decided not to perform pancreaticoduodenectomy but to excise the mass intraoperatively, and consequently avoided unnecessary resection of the pancreas and anastomosis to undilated hepatic and pancreatic ducts. This protective strategy prevented duodenum- and pancreas-related morbidity. Langhans is often credited with the first description of a gut carcinoid tumor in 1867 . Since tIn our case, because histopathologic findings were insufficient and unconfirmed preoperatively, a pancreaticoduodenectomy was planned, but after intraoperative exploration, the mass was thought to be a NET and resected locally, with frozen section examination showing malignancy-free margins. Neuroendocrine cell tumors should always be considered if found in the periampullary region, as in our case. Here, we have reported a case of nonfunctional duodenal somatostatinoma showing regional lymph node and sphincter of Oddi metastases, and reviewed the literature focusing on the latest clinical outcomes of this disease.The patient was a 42-year-old Turkish White man presenting with nausea, vomiting, fatigue and abdominal pain. Laboratory data revealed no abnormalities in blood chemistry or tumor markers. An enzyme-linked immunosorbent assay disclosed positivity for HBsAg. No abnormal findings were observed in the chest X-ray. Computed tomography revealed thickening at the second and third portions of the duodenal wall and a polypoid contrast-enhanced area. Because a gastroduodenoscopy showed no abnormalities, an endoscopic retrograde cholangiopancreatography (ERCP) was performed. The ERCP revealed a duodenal mass. Biopsy specimens derived by ERCP were highly suspicious for malignancy. Subsequent color Doppler examination performed for abdominal aorta and portal vein invasion did not reveal any abnormalities. The plan was to perform a pancreaticoduodenectomy, and 10 days later the patient underwent surgery. A mobile, polypoid, palpable tumor was further uncovered in the third portion of the duodenum, distal to the duodenal papilla. Pancreas, papilla and duodenal wall except the third portion seemed to be free from the polypoid mass. Considering the possibility of a NET tumor, the relatively invasive procedure was postponed, complete tumoral tissue was excised locally, and the specimen sent for frozen section examination. Pathological examination revealed that resected tumor margins were free of malignancy. However, owing to the proximity of the tumor to the ampulla of Vater, a sphincteroplasty was conducted to prevent potential obstruction. In addition, cholecystectomy and choledochotomy procedures were performed, and a T-tube inserted into the choledochus. Finally, all the enlarged lymph nodes were resected. Pathological examination with paraffin embedding and staining disclosed a 1.7cm well-differentiated, Grade 1 neuroendocrine carcinoma (NEC) (Figure Carcinoid tumors of the duodenum are generally low-grade NETs. The term ‘neuroendocrine’ is derived based on similarities between these cells and neural cells in terms of expression of specific proteins, such as synaptophysin, neuron-specific enolase and chromogranin A . These tIn our patient, the tumor originated from the third portion of the duodenum and was measured as 1.7cm. The patient presented with nonspecific gastrointestinal symptoms, such as abdominal pain, fatigue, nausea and vomiting. The serum insulin, glucagon, gastrin, somatostatin and urine 5-hydroxyindoleacetic acid levels were not assessed because the mass was not considered a NET preoperatively. Histopathologically, a characteristic structure is the psammoma body, which has been identified in 68% of duodenal somatostatinomas . On silvet al. [Primary surgical resection of the tumor and regional lymph nodes is the only curative treatment for gastrointestinal NETs. This is usually possible in about 20% of patients . There iet al.  identifiet al. . AdjuvanNETs of the small bowel are increasing in incidence. With access to new diagnostic and imaging techniques, detection of these tumors in the United States of America has increased by 300% to 500% in the past 35 years. Simultaneously, prognosis of these tumors has improved considerably . Data fren bloc resection. If the tumor is an adenocarcinoma or carcinoid, en bloc resection and systemic lymph node dissection are indicated [Tumors of the periampullary region are considered highly malignant, and the Whipple operation is usually the only procedural treatment. This procedure is a pancreaticoduodenectomy, which is fairly invasive with a number of postoperative potential complications, such as pancreatic fistula, gastroparesis, and malabsorption. Overall survival after Whipple operation for pancreatic adenocarcinoma is about 20% at 5 years after surgery. However, the optimal resection extent of duodenal tumors has not been defined as yet. A number of researchers advocate pancreaticoduodenectomy  for all ndicated . Others ndicated -28. In tTumors of the periampullary region are considered highly malignant, and the Whipple operation is usually the only procedural treatment. In the current case, we decided not to perform pancreaticoduodenectomy but instead to excise the mass intraoperatively, and consequently avoided unnecessary resection of the pancreas and anastomosis to undilated hepatic and pancreatic ducts. This protective strategy prevented duodenum- and pancreas-related morbidity.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.OK and YSS were major contributors in writing the manuscript. YD evaluated the literature and statistical data. HB, GU, and VT analyzed and interpreted the patient data. EP performed the histopathological examination. All authors read and approved the final manuscript.


Introduction. Hepatobiliary cystadenomas are rare benign cystic tumors and have a potential for recurrence and malignant transformation. The diagnosis may be very difficult because of absence of typical imaging feature in some cases. Case Presentation. In this paper, the authors discuss a 57-year-old woman who presented a jaundice related to hepatobiliary cystadenoma. Biological and radiological examinations have led to surgery, and the diagnosis is made after a histological examination of surgical specimens. Conclusion. This observation illustrates a hepatobiliary cystadenoma revealed by jaundice. Histology examination contributed to diagnosis. The authors discussed the mechanisms of biliary obstruction and differential diagnoses through a review of the literature. Hepatobiliary cystadenomas (HBCs) are rare benign cystic tumors , occurriA 57-year-old woman was admitted to our hospital for investigation of epigastric and right hypochondriac pain and jaundice. Laboratory test results revealed liver dysfunction  922 IU/L; cholestasis: glutamyl transpeptidase (GGT) 1705 IU/L; alkaline phosphatase (PAL) 177 IU/L). The hydatid serology was negative. Liver MRI with cholangioMR was performed Figures . Axial TIn the draining cyst, fluid analysis found elevated carbohydrate antigen CA19.9 (higher than 70 000 U/mL), and carcinoembryonic antigen (CEA: 5.8 ng/mL) without any scolex or other pathogenic agents. The proposed diagnosis was a cystadenoma probably communicating with biliary ducts. Intraoperative endoscopic retrograde cholangiography revealed a bud into the left extrahepatic bile duct. Surgery consists on a left hepatectomy associated with cholecystectomy and intrabiliary tumoral bud resection. Both upper biliary confluence and common bile duct were free of tumor and note resected. Macroscopically, two cystic masses of 4.5 × 5 cm and 1.5 × 1.5 cm large were found in segment IV of the liver. The inner wall lining was smooth, without any infiltrative pattern. The cystic cavities were filled with clear mucinous fluid. Histopathological analysis confirmed the diagnosis of typical HBC harboring mesenchymal stroma , withoutHBCs represent approximately 5% of intrahepatic cystic lesions . Among tThe presence of thick wall, endoluminal buds, and septations at imaging investigation is suggestive of the diagnosis of HBC. The diagnosis may be very difficult because of absence of typical imaging feature in some cases, or sometimes with unusual clinical presentation such as jaundice. The pathology confirmation is needed for optimal treatment.The authors declare that they have no conflict of interests.


Hepatocellular carcinoma rarely metastasizes to the orbit. We report a 45-year-old male, HBV+, HIV+, with a past history of a liver transplant for ELSD (end-stage liver disease) with hepatocellular carcinoma and recurrent HCC, who presented with proptosis and diplopia of the left eye. CT scans of the head revealed a large, irregular mass in the left orbit causing superior and lateral destruction of the orbital bone. Biopsy specimens of the orbital tumor showed features of metastatic foci of hepatocellular carcinoma. Only 16 other cases of HCC metastasis to the orbit have been described in literature, and this is the first case in a previously transplanted HIV+, HBV+ patient. Hepatocellular carcinoma (HCC) accounts for 85–90% of all primary liver cancers. Each year, approximately half a million people worldwide are newly diagnosed with HCC. The majority of HCC cases develop on a substrate of chronic hepatitis B (HBV) and C virus (HCV) infection. ESLD (end-stage liver disease) and HCC are increasing causes of mortality in human-immunodeficiency-virus- (HIV-) seropositive patients. Coinfection with HIV may accelerate the progression of chronic HBsAg-positive hepatitis to cirrhosis and may foster the rapid progression of regenerative nodules to dysplastic nodules and HCC. OLT (orthotopic liver transplantation) in HIV patients with ESLD with or without HCC is an emerging option in this set of patients, especially since the administration of HAART can guarantee a good viral-immunological framework.Approximately 50%–75% of patients with HCC develop metastases during the disease course . The mosWe report a case of orbital metastasis from HCC in a 45-year-old HCV+, HIV+ male with a past history of a liver transplant for hepatocellular carcinoma, and recurrent HCC subjected to multiple therapies.A 45-year-old man presented to our department in December 2010 with proptosis of the left eye lasting about one month . He comp His past medical history was positive for HIV and HBV infection since 1986 and consequent cirrhosis and hepatocellular carcinoma (HCC). In July 2006, he had undergone a liver transplant for ESLD with concomitant HCC, with a marginal organ. Histologic evaluation of the native liver pointed out the presence of a cancer thrombus within the right branch of the portal vein. In December 2008, he was subjected to a partial surgical resection of the transplanted liver for a recurrence of HCC. He started therapy with sorafenib in 2009, but took the drug irregularly and reduced the dosage for the concomitant occurrence of diarrhea. He presented a new liver and pulmonary recurrence in December 2009. The liver recurrence was treated with selective transarterial chemoembolization, while the pulmonary metastases were treated by local resection of the middle lobe of the left lung. After histological examination of the pulmonary lesions confirmed the HCC recurrence, the patient started systemic chemotherapy with liposomial doxorubicin. In July 2010, the patient presented a new HCC recurrence to the lungs and liver and therefore underwent level II chemotherapy with 5-FU and subsequently with capecitabine. In December 2010, he presented with proptosis and diplopia of the left eye, and AFP was high: 3750 ng/mL. On the suspicion of an orbital metastasis of HCC, the patient underwent computed tomography (CT) scans of the orbit that showed an extraconal mass involving the superior and lateral wall of the left orbit with destruction of the orbital bone, incorporating the lateral rectus, with calcifications Figures . Percutaα-fetoprotein were negative.Microscopic examination showed a diffuse infiltrate of large polygonal cells with a finely eosinophilic cytoplasm and roundish-oval hyperchromatic nuclei containing one or a few large nucleoli. The growth pattern was trabecular with the formation of occasional well-defined lumina. The nuclear pleomorphism and mitotic activity were moderate . The neoAt electron microscopy, the neoplastic cells were fairly uniform in shape, size, and structure. They contained numerous mitochondria, a well-developed rough endoplasmic reticulum, intracytoplasmic bile products, and abundant glycogen. Bile canaliculi with stubby microvilli and cell junctions were also evident . The patA mass in the orbit is rarely a case of metastasis, featured in only 3–9% of all orbital tumors . Tumor cThe clinical manifestations of metastatic carcinoma are proptosis, ophthalmoplegia, diplopia and acquired strabismus, conjunctival hyperemia, pain, and diminished vision if the tumor infiltrates the choroids , 3.The histopathological diagnosis of HCC is based on the presence of large, polygonal cells, a trabecular pattern, and endothelial cuffing. Differential diagnosis must be made for renal cell carcinoma, that can be present with similar cytological features and architecture, and also shows similar staining for cytokeratins . The twoThe diagnosis of HCC metastases can be confirmed by the use of immunohistochemistry . HCC metRecently, therapy for advanced HCC was revolutionized by the advent of Sorafenib, a multikinase inhibitor with an antiangiogenic activity targeting the VEGF pathway. Rapamycin, also used as an immunosuppressant in this patient, showed a strongly increased antiproliferative activity targeting the m-Tor pathway in HCC, when paired with Nexavar. Two recent reports have pointed out that discontinuous, low-dose treatment with antiangiogenics might elicit tumor invasiveness and metastasis –13. Many factors in this patient may have contributed to this unusual presentation of HCC metastasis: the immunosuppression caused by HIV infection, the documented invasion of the vascular bed by HCC on removal of the liver, and the possibility of pulmonary metastasis of HCC .Although orbital metastases are rare, the ophthalmologist should be aware of this clinical form and prognostic implications  and make efforts to palliate the patient's symptoms as far as possible.


Giant cell reparative granuloma (GCRG) is an uncommon and nonneoplastic reactive tumor that involves the maxilla and mandible in the region of the head and neck. It is rare in the nasal cavity, and it might be misdiagnosed. We reported a very aggressive GCRG with intracranial invasion, which was treated surgically via a combined approach of a lateral rhinotomy with a craniotomy by bilateral coronal incision. The pathology was consistent with GCRG. A short literature review about diagnosis, clinical behavior, and treatment of this tumor entity is given. The term giant cell reparative granuloma (GCRG) was first introduced by Jaffe in 1953 . GCRGs cA 49-year-old women was admitted to the department of otorhinolaryngology in an affiliated hospital with a 2-month history of right-sided nasal obstruction, epistaxis, proptosis, and diplopia. The mass was biopsied endoscopically. Massive bleeding from the tumor was seen at that time. Histopathological diagnosis was reparative giant cell granuloma. Oral steroids (30 mg prednisolone) were administered due to rapid growth of the tumor for two weeks. Since the treatment was not effective, the patient was sent to our hospital. She was a nonsmoker, and there was no history of facial trauma and previous radiation exposure. Her past medical history was unremarkable, and there was no significant family history. Physical examination revealed that the mass completely obstructed the right nasal cavity and partially the left side. The outer surface of the mass was smooth and displaced the right eyeball towards the lateral side, and consequently led to the diplopia. There were no palpable masses or lymphadenopathies in the neck. Routine hematological and biochemical test results including serum calcium, alkaline phosphatase, phosphorus, renal function, and parathyroid hormone were within normal limits.Computerized tomography (CT) scanning showed expansile mass and with a multilocular appearance and extension into the orbit, ethmoid sinus, maxillary sinus, and intracranial region .The patient underwent surgical resection via combined approach of a lateral rhinotomy with a craniotomy by bilateral coronal incision after bilateral maxillary artery embolism. The mass originated from the localization of the nasal cavity and eroded lamina papyracea, medial wall of maxilla, and anterior skull base. There was strong adhesion to the dura and the medial rectus; therefore, the mass was excised en bloc except above lesion, and then we performed additional resection for complete surgical excision. Skull base reconstruction was done by cranial bone and pericranial flap. Definitive histologic examination revealed a fibroblastic proliferation with rich osteoclast-like polynuclear giant cells interspersed with spindle-shaped stroma. Figures . There w Giant cell reparative granuloma most commonly involved the mandible. To our knowledge, this is the first report of GCRG from nasal cavity with intracranial invasion. GCRG of the craniofacial bones has to be distinguished from aneurismal bone cyst, giant cell tumor, and Brown tumor. Aneurismal bone cyst usually arises from the vertebrae and the long bones, not the facial bone. The histological differential diagnosis is accomplished with the absence of both the chondroid matrix and the new bone formation in case of giant cell tumor. In addition, a true giant cell tumor shows more giant cells and mitotic activity. Furthermore, Ca, P, and PTH levels are in normal limits in GCRG, and it can be differentiated form Brown tumor of hyperparathyroidism. GCRG has two clinical forms: central-endosteal and peripheral-soft tissue forms . The per The treatment of GCRG is usually surgical. The common proposal for the treatment is surgical excision or curettage and 80% of the cases can be cured with these modalities. It may recur after incomplete removal in 10% to 15% . The rec Because of the possibility of recurrence especially in aggressive type, it should be acquainted with the alternative treatment of GCRG. The use of steroid has been reported . It is s In conclusion, we have reported a case of aggressive GCRG originated nasal cavity with intracranial invasion. We have to put the high priority on the complete resection of GCRG, considering other alternative methods.


Capsular warning syndrome (CWS) is rare (1.5% of TIA presentations) but has a poor prognosis (7-day stroke risk of 60%). Up to date, the exact pathogenic mechanism of CWS has not been fully understood. We report the clinical presentations and high-resolution MRI (HR MRI) findings of two cases with capsular warning symptoms.Case 1 was a 63-year-old man with a history of hypertension with recurrent episodes of left hemiparesis and dysarthria lasting 10 ~ 30 minutes. Case 2 was a 54-year-old woman with repetitive episodes of transient left hemiparesis and dysarthria lasting about 10 minutes. Capsular infarctions on DWI were demonstrated in the territory of a lenticulostriate artery in both 2 patients. HR MRI disclosed atherosclerotic plaques on the ventral wall of the MCA where enticulostriate arteries were arisen from, although traditional digital subtraction angiography showed normal. Aggressive medical therapy with dual antithrombotic agents and statin was effective in these two cases.Our HR MRI data offer an insight into the pathophysiology of CWS which might be caused by atherosclerotic plaque in non-stenotic MCA wall. HR MRI might be a useful modality for characterizing atherosclerotic plaques in the MCA and detecting the pathophysiology of the CWS. The term of capsular warning syndrome (CWS) was first described in 1993 in patients who presented repeated stereotyped episodes of subcortical transient ischemic attacks ,3. Up toA few recent studies have confirmed the feasibility of using high-resolution magnetic resonance imaging (HR MRI) to depict the MCA wall and plaques in vivo ,5. We reA 63-year-old man with a history of hypertension was seen with recurrent episodes of left hemiparesis and dysarthria lasting 10 ~ 15 minutes in the emergency room. His blood pressure was 136/94 mmHg. Neurological examination on admission was unremarkable. Treatment of antithrombotic drug (aspirin 200 mg) and hydration was given immediately. During the first 48 hours hospitalization he experienced 9 times episodes of left hemiparesis, left central type facial palsy, and dysarthria lasting 13 to 36 minutes. His neurologic status fluctuated between 5 and 11 on the National Institutes of Health Stroke Scale (NIHSS) and he couldn’t relieve completely after the last episode with left mild hemiparesis and facial palsy (NIHSS 3). Brain MRI showed an acute infarct on right putamen with rostral extension to the corona radiates . Brain MRI demonstrated an acute right-sided infarct on posterior limb of internal capsular Figure A. Brain This is the first study to date on HR MRI findings of MCA cross-section in patients with CWS. Capsular infarctions were demonstrated in the territory of a lenticulostriate artery on DWI in both 2 patients. Although cerebral vascular angiography showed no abnormalities, HR MRI disclosed atherosclerotic plaques on the ventral wall of MCA where enticulostriate arteries were arisen from. These HR MRI findings suggested that MCA atherosclerotic disease may play an important role in pathophysiology of the CWS in our cases.The ‘capsular warning syndrome’, a term introduced by Donnan et al., denotes repetitive transient ischemia attacks causing stereotyped unilateral motor, sensory, or sensorimotor deficits that simultaneously affect the face, arm, and leg without cortical symptoms . CWS is The exact pathogenic mechanism of CWS has not been fully understood. Various mechanisms have suggested, including small vessel disease, embolism from the heart, vasospasm, peri-infarct depolarization, and, in rare instances, atherosclerotic disease of the MCA ,7-10. SmDifferent treatment modalities, including hydration, antiplatelet agents, intravenous thrombolysis and intracranial artery angioplasty, have been proposed in patients with CWS ,9,11,16.In summary, Our HR MRI data offer an insight into the pathophysiology of CWS which might be caused from atherosclerotic plaque in non-stenotic MCA wall. Early recognition of this clinical presentation and the accompanying stroke mechanism may guide the initial management and prognosis. Further studies using HR MRI in larger sample subjects are expected for highlighting this mechanism and guiding the acute treatment decision for CWS patients.“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 Series Editor of this journal”.The authors declare that they have no competing interests.LZ and JN were responsible for writing the article. MY was responsible for collecting case information. ML was responsible for collecting and analyzing imaging data. WX, BP and LC were responsible for revising the article. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/14/16/prepub


Benign cementoblastoma is a rare ectomesenchymal odontogenic tumor that originates from the root of the tooth and that is characterized by the formation of cementum-like tissue. A 60-year old man was referred to us complaining of pain in his right jaw. The patient underwent TC dental scan of the mandible, which highlighted the presence of three well-circumscribed, round, unilocular neoformations of radiopaque appearance with a radiotransparent edge, one of which was in close contact with the roots of the lower right second molar. Microscopic examination of the greater sample consisted, in its central portion, of dense mineralized acellular trabeculae of basophilic tissue cement-like, devoid of vessels, adhering to the root of the tooth, while peripherally was observed a zone of vascularized osteoid surrounded, occasionally, by a thin rim of cementoblasts mixed with fibrous tissue and inflammatory elements. This lesion was diagnosed as cementoblastoma. The second lesion appeared radiologically and histologically entirely identical to cementoblastoma, but it did not show the intimate association with the root of involved tooth. After a careful review of the literature, the diagnosis of residual cementoblastoma was made. The clinicopathologic features, treatment, and prognosis of this rare tumor are here discussed for the young dental practitioner. It u005 as anexamination of the patient's medical history revealed good general health, absence of systemic diseases, and smoking habit (10 cigarettes∖day). The clinical examination showed discrete oral hygiene, thick and flat periodontal biotype, class II malocclusion with marked loss of vertical size, multiple missing teeth in the site 1.4-1.5-3.5-3.6-3.7-4.5-4.6., presence of swelling, hard elastic consistency, and crepitus on palpation in the region corresponding to the elements 4.4-4.7 and in the edentulous area 4.6, 4.5. The orthopantomography revealed on the upper jaw radicular element in 1.4 region, conservative restoration in 1.1-2.1-2.2-2.6, the presence of radiopaque unilocular lesion adjacent to the root apex of the element 1.6 while the lower arch showed a carious radiolucency at the crown of element 4.7 and other lesions spread to the mandibular body. Particularly, these lesions were thus located: three unilocular round shaped radiopaque lesions with a perilesional radio transparent rib next to the edentulous sites 3.6-4.6 and in contact with the roots of the element 4.7, whose appearance is suggestive for a neoformation , extraction of root element in endodontic treatment on site 1.4, a conservative endodontic treatment of the element 4.4 in order to resolve endodontically periapical radiolucent lesion and endodontic treatment of 4.7 to cure the acute pulpitis. After two months, following the resolution of acute symptomatology, in order to better analyze the lesions that were evident in the orthopantomography, the patient underwent TC dental scan of the mandible , which hanaesthesia with a full-thickness flap, osteotomy using rotating tools, enucleation of the lesion corresponding to the edentulous area 4.6 and the full enucleation on site 4.7 with the annexed dental element (The surgical treatment that included the enucleation of the only two lesions on the right jaw, the ones involved in the acute pain, was performed in local and regional  element  because The specimens were sent to surgical pathology for definitive diagnosis. Both lesions were examined in toto  and the The location and the histological presentation of benign cementoblastoma is totally identical to osteoblastoma , 8. The


Ovarian adenosarcoma is a very rare tumour for which treatment options vary. We will consider the option of optimal cytoreductive surgery followed by adjuvant chemotherapy consisting of ifosamide (mesna) and adriamycin to prevent systemic metastasis, and will observe the role of serial CA-125 levels as a follow-up marker.We report a case of ovarian adenosarcoma in a 38-year-old woman presenting with abdominal pain, distention due to massive ascites. She had undergone total abdominal hysterectomy 8 months previously for abnormal uterine bleeding. She underwent paracentesis followed by optimal cytoreductive surgery. A post-operative histopathologic diagnosis of primary adenosarcoma was confirmed. She was assigned a stage III C cancer. She received five cycles of ifosamise (mesna) and adriamycin and is on follow-up with serial CA-125 levels. There is no evidence of recurrence clinically, biochemically, or radiologically for more than 12 months.Multimodality treatment comprising optimal cytoreductive surgery followed by ifosamide (mesna) and adriamycin-based chemotherapy may be an option for treatment of these aggressive tumours. Follow-up with serial CA-125 values in advanced stage adenosarcoma of the ovary is a novel observation which needs to be researched. Mullerian adenosarcoma is very rare. In 1974, Clement and Scully described uterine adenosarcomas for the first time –10, and A 38-year-old multiparous woman presented with history of abdominal distension and pain for almost 3 weeks. She had undergone hysterectomy 8 months previously for abnormal uterine bleeding. Following abdominal paracentesis for massive ascites, a mobile mass was palpated measuring about 12 × 10 × 6 cm. Upper abdominal fullness was noted. Her haemogram, serum biochemistry, serum antibodies for HIV and HBsAg were negative with normal chest X-ray and cardiac evaluation. The serum CA-125 value was 142 U/ml. A CT scan of the abdomen and pelvis revealed multiple heterogeneously enhancing soft tissue masses within omentum, mesentry, perihepatic regions, and the pelvis. The ovaries were not seen separately. There was moderate ascites suggestive of malignant ovarian lesion with metastasis  and 2. PDuring laparotomy, 1 L of ascites was drained, a huge, vascular omental cake with multiple nodular deposits were seen . The rigThe IHC revealed neoplastic spindle cells which were positive for CD10 and negative for inhibin, C-kit, Calretiniun, SMA, S100, and Mic2, and the epithelium was positive for CK7 and EMA  and 8. SShe received five cycles of ifosamide with mesna and adriamycin every third week. She is on follow-up for more than 12 months, and there is no clinical, radiological, or biochemical evidence of recurrence.Extrauterine Müllerian adenosarcomas are rare tumours. They can arise from the ovary, fallopian tubes, round ligament, pouch of Douglas, vagina, bladder, colon, and even peritoneum , 9, 11.The World Health Organization defines adenosarcoma as a biphasic tumour characterized by the proliferation of Müllerian epithelium of benign appearance or occasionally labelled atypical when absorbed in or covering a predominant sarcomatous stroma .The present case has many clinical, therapeutic, and prognostic implications.First, the adenosarcoma was arising from a previously normal ovary, which was salvaged for hormonal functions just 8 months earlier. These extrauterine Müllerian adenosarcomas occur at a younger age than their uterine counterparts and have more aggressive clinical behaviour because of invasion to adjacent pelvic organs at the time of diagnosis. Similar observations have been confirmed by many authors , 7, 9.Second, homologous (pure) adenosarcomas arising from the ovary without associated endometriosis in either of the ovaries is very rare. Majority of the case reported have been associated with past, present, or concurrent evidence of endometriosis. The exact association is not clear in the literature as shared by many authors , 10, 11.Third, ascitic fluid cytology may not be contributory, a frozen section may reveal the presence of malignant stromal tumour which needs further categorization and hence the importance of histopathology to delineate the benign and the malignant component of the tumour and immunohistochemistry to categorise the sarcomatous stromal component around which revolves the adjuvant therapy, prognosis and survival of the patients , 11. To et al [Fourth, a raised CA-125 level being uncommon in adenosarcoma has been observed by authors as depicted in et al  in an eaMultimodality treatment comprising optimal cytoreductive surgery followed by adjuvant ifosamide (mesna) and adriamycin-based chemotherapy may be an option for the treatment of these aggressive tumours. Follow-up with serial CA-125 values in advanced stage adenosarcoma of the ovary is a novel observation which needs to be researched.


Epidural lipomatosis is a rare entity to cause spinal cord compression and neurological deficits. This is usually associated with excess of steroids in the body either because of endogenous source as in Cushings disease or exogenous intake as in some diseases like systemic lupus erythematosus, in some endocrinopathies or in morbid obesity. But in some cases no cause has been found. Such idiopathic cases of spinal epidural lipomatosis have also been reported. Here, we report a case of idiopathic spinal epidural lipomatosis with relapsing and remitting paraparesis which is quite unusual. Treatment depends upon neurological status of the patient. We operated the patient as he had significant neurological compromise and he improved significantly. Subsequent reports on this rare clinical entity also implicated the administration of steroid as the major cause for spinal epidural lipomatosis.Spinal epidural lipomatosis is defined as pathological overgrowth of the normally presented extradural fat and often causes dural impingement. Symptomatic epidural lipomatosis was first described by Lee 2] without any evidence of Cushings syndrome or endocrinopathies. Systemic examination was normal. Neurological examination revealed increased tone in lower limbs with preserved muscle bulk. Power at hip and knee was 3/5 while at ankle and toes it was 0/5  with brisk knee and ankle jerks. Bilateral planters were extensor. There was a graded sensory loss below D10 level. Local examination did not reveal any spinal deformity or tenderness. He was diagnosed to have compressive myelopathy in thoracic region. His hematological and biochemical investigations were normal. Cerebrospinal fluid (CSF) examination was also normal. X-ray spine was unremarkable, but magnetic resonance imaging (MRI) thoracic spine showed posterior epidural mass in dorsal spine extending from D5 to D7 level with maximum thickness of 10 mm and causing cord compression. This mass was homogeneously hyperintense in both T1W1 and T2W1 images suggestive of fat [Figures We report an 18-year-old male who presented to neurosurgery outpatient department with complaints of fluctuating paraparesis for 6 months. He noticed weakness of lower limbs when he woke up one morning; he failed to move his lower limbs but after half an hour, he started moving with persistent unsteadiness and difficulty in walking. This deficit also recovered the same evening. He remained asymptomatic for the next 4 months when he again developed ascending weakness of lower limbs along with spasticity, and in the next 3 months, he was not able to stand even with support. He developed retention of urine for which a Foley’s catheter was installed. He had constipation for 1 month. This patient denied history of trauma, fever, drug intake like steroids, antithyroid drugs, and infections. Physical examination revealed a non-obese patient [weight 65 kg, height 167 cm, body mass index (BMI) 23.30 kg/mSpinal epidural lipomatosis is characterized by abnormal deposition of unencapsulated fat in epidural space. This con5Males are affected more than females, with 75% of reported cases being males.–12 Thora13MRI spine is diagnostic choice of imaging. It shows uniformly hyperintense fat collection in epidural space on both T1W1 and T2W1 sequences. Spinal a2102) nor had excess steroids. Surgical intervention is indicated in severe progressive neurological deficits, which include decompressive laminectomy and resection of epidural adipose tissue.[1210Treatment depends upon severity of neurological deficits. Conservative treatment has been reported to be successful in many patients, i.e., weaning of exogenous steroids or treatment of Cushing disease and weight loss.15 But outissue.121011 DecoTo conclude, spinal epidural lipomatosis should be considered as differential diagnosis of epidural mass causing compressive myelopathy with relapsing and remitting course of paraparesis. It may be present even in the absence of endocrinopathies or in non-obese patients. MRI spine with contrast is diagnostic and early diagnosis is important as minor symptoms may be relieved on conservative treatment while advanced and progressive disease requires surgical intervention.


The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC). A 3-year-old boy was referred to our hospital with nausea and vomiting since 2 months before admission and massive abdominal mass in the right upper quadrant of abdomen, extending up to the midline. Physical examination revealed a unilateral, palpable, firm, abdominal mass extending to more than 10 cm below the right costal margin .The results of initial laboratory tests were as follows: WBC = 5100, PMN = 52%, lymph = 45%, Hb = 8, platelets = 109000, LDH = 15714, ESR = 7, uric acid = 6, BUN = 15, creatinin: 0.7, SGOT = 260, SGPT = 25, bilirubin total = 2.2, bilirubin direct = 0.9, PT = 13, and PTT = 36. Bone marrow aspiration and biopsy were normal. Bone survey showed permeative lesions, some erosion in distal of right femur and lateral of middle right tibia, but bone scan was normal. Abdominal ultrasound revealed a large abdominal mass. So, computed tomography (CT) scan of abdomen and pelvis for more evaluation was performed, which showed an extensive tumoral lesion, size of 110 × 56 × 90 mm (intracapsular) in right kidney with dilatation of calyceal system, some deformity, and some scattered calcification. The tumor extended beyond the abdomen midline till the left paravertebral region, while bilateral adrenals were not seen. Hydronephrosis and some deformities of right kidney were seen. Left kidney was normal . MagnetiOn day 2 of admission, laparatomy was done; tumor was associated with massive hemorrhage, and just biopsy was done. After surgery, he suffered from persistent hypertension. So, treatment was started to control the blood pressure. The results of histopathology report indicated neuroblastoma with poor stroma, poor differentiation, and MKI < 100/5000 neuroblast: unfavorable Figures  and 5. IOn day 6 of after surgery, the patient developed dyspnea, pleural effusion; therefore, pleural tap was done and then chest tube was inserted. He suffered from massive bleeding. Evaluation for bleeding disorders was in favor of overt DIC: fibrinogen = 140, FDP > 320, D-Dimer = 32/9, low platelet count, and long PT and PTT. He received supportive therapy, including packed red blood cell, platelet, FFP, cryoprecipitate, and Novoseven because of continous bleeding. Finally, bleeding was stopped. Now, patient is clinically stable. Our plan for this child is chemotherapy with N6 protocol, second-look surgery, and then autologous stem cell transplantation based on stage of disease, unfavorable histopathology report, and N-Myc gene amplification result.Primary IRNB is a rare clinical entity . Lall etA higher incidence of hypertension (66–100%) has been associated with intrarenal neuroblastoma as compared to 27% reported in the literature for neuroblastoma, probably because of compression of renal vessels, increased renin release from the kidney, and a high circulating level of catecholamines. Lall et al. reported hypertension in all their cases of intrarenal neuroblastoma [About primary intrarenal neuroblastoma, Fan (2012) wrote, if the tumor primarily involves the kidney without evidence of involvement of other primary sites, then it is considered a primary renal tumor. It is possible, and in fact speculated, that some so-called primary renal neuroblastomas actually arose from the immediate pararenal/hilar region, which, for all practical purposes, should be managed in the same or similar manner . The facThere are some reports about association of neuroblastoma and disseminated intravascular coagulation (DIC) –7. Our pPrimary intra renal is an extremely infrequent tumor in kidney. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor presents an important challenge since both tumors have major differences in prognostic and therapeutic response.


Polyorchidism is defined as the presence of more than two testes. Triorchidism is the most frequent presentation. This anomaly is extremely rare, and approximately a hundred cases were described in the literature. We report a case of triorchidism presenting with inguinal hernia and penoscrotal hypospadias in a three-year-old male and briefly discuss current management of polyorchidism. Management remains controversial especially if there is no associated abnormality identified. The absence of any concomitant disorder and if testicular tumour can be ruled out by sonography or magnetic resonance imaging, surgical exploration with biopsy could be unnecessary. On the contrary, surgical exploration has the advantage of allowing for fixation of the testes to prevent torsion and determination of testicular outflow tracts and estimating reproductive capacity. Polyorchidism is defined as the presence of more than two testes, and it is a rare congenital abnormality. The first accepted case was a post mortem report by Ashfeld in 1880 . We descA three-year-old boy was admitted with the complaint of left inguinal swelling. On physical examination, a left sided inguinal hernia and penoscrotal hypospadias were found. Both testes were palpated in the scrotum. Other physical findings were normal. The family history was unremarkable. The diagnosis of right inguinal hernia and penoscrotal hypospadias was made. At the time of operation the right inguinal region was explored initially. Hernia sac and cord structures traversing the internal inguinal ring were identified. Exploration revealed two separate testicles within a single tunica vaginalis. Supernumerary testis was seen smaller than the other one, and it lacks an epididymis and vas . After pPolyorchidism is an urogenital curiosity defined by the presence of more than two testes confirmed by histology. This anomaly is extremely rare, and approximately over a hundred cases were described in the literature. Although it can remain asymptomatic, polyorchidism is often associated to processus vaginalis anomalies and undescended testis in childhood. Supernumerary testes may have scrotal, inguinal, or abdominal location; they are more frequently on the left side, and their size is often smaller than both ipsilateral and contralateral testes . As in tThum  proposedPolyorchidism is usually identified during repair of inguinal hernia and orchidopexy in children. In our case, inguinal hernia and penoscrotal hypospadias were involved. Commonly associated anomalies are testicular maldescent (40%), inguinal hernia (30%), testicular torsion (13%), hydrocele (9%), and hypospadias (1%) . InfertiWhen a polyorchidism is suspected of palpable mass in the groin or scrotum, sonography is the effective, noninvasive modality for use in its investigation and preoperative evaluation. On sonography, an accessory testis usually displays a fine granular echotexture similar to that in the normal testis. Color doppler sonography can provide further information about the blood flow pattern in the testis. MRI may provide confirmation when the results of sonography are inconclusive .The management of polyorchidism has been still controversial. In the past it was common practice to remove the supernumerary testicle with removal of the smaller mass . More reSome authors claim that conservative treatment is the appropriate choice. They suggest that supernumerary testis, even in ectopic locations, should be preserved if they appear normal and are potentially functional. They believed that the absence of any concomitant disorder and if testicular tumour can be ruled out by sonography or magnetic resonance imaging, surgical exploration with biopsy could be unnecessary , 5. On tThe diagnosis of polyorchidism is usually incidental. According to our opinion, if polyorchidism is associated with doubtful concomitant pathology, surgical intervention should be undertaken. However, in uncomplicated polyorchidism, conservative management with close magnetic resonance imaging or ultrasonography observation should be recommended.


Ectopic ACTH Syndrome (EAS) is extremely rare in pediatric age group. Sarcomatous tumors causing EAS are even rarer. A nine year and 4 months old male Filipino presented with a 6 months history of gradually enlarging mass on the left distal thigh. He experienced increasing appetite, rapid weight gain of about 27kgs in 5 months, recent onset of widespread acne and darkening of skin around the neck. Physical Examinations revealed cushingoid face, widespread acne, facial hair, phlethora, buffalo hump, truncal obesity, purple abdominal striae and severe acanthosis nigricans on the neck and axilla. Non-inflamed mass and slight tenderness over the left distal thigh were noted. Patient was hypertensive. Weight & height for age were at Z score above 3 and -1, respectively. Body Mass Index at Z score above 2. His pubertal development was classified as Tanner stage 1 testicular and pubic hair development. Work ups revealed hypernatremia, hypokalemia, metabolic alkalosis and elevated levels of serum cortisol and ACTH. Low and high dose dexamethasone suppression tests failed to suppress serum and urine free cortisol levels, findings which were consistent with ectopic ACTH secreting tumor. Radiological findings were normal skull and chest roentgenograms and normal computed tomographic scan of chest and abdomen. Magnetic resonance imaging of Hypothalamo-pituitary was also normal. An Immunohistochemistry result of left distal thigh mass was consistent with Ewing Sarcoma. The patient received chemotherapy with Vincristine, Doxorubicin and Cyclophosphamide. Unfortunately, patient succumbed to death 5 days post chemotherapy due to severe sepsis.


Duodenal gangliocytic paraganglioma (DGP) is a rare tumor that characteristically occurs in the second part of duodenum. These appear as submucosal masses that protrude into the lumen of a duodenum. Gastrointestinal bleeding is the commonest manifestation of DGP. Metastatic spread to regional lymph nodes occurs rarely. Surgical resection is the treatment of choice for DGP. A case of a DGP is reported in young female who presented with a recurrent upper gastrointestinal bleeding. Upper gastrointestinal endoscopy (UGIE) documented a mass in the ampullary region with ulceration in its middle which was bleeding. Recurrent gastrointestinal bleeding necessitated an emergency pancreaticoduodenectomy. Histopathology of specimen documented gangliocytic paraganglioma. Duodenal gangliocytic paraganglioma (DGP) was first described by Dahl et al.  in 1957 A 20-year-old female presented with passage of black tarry stools. On general physical examination, she had an anxious look, pallor, tachycardia of 98/min, and B.P of 100/70 mm Hg. per abdominal examination was normal. Digital per rectal examination confirmed malena. She had resuscitation and the transfusion of 3 units of packed red cells. Emergency upper gastrointestinal endoscopy (UGIE) revealed fleshy exophytic sub mucosal tumor with ulceration in its middle arising in the second part of a duodenum. A biopsy was taken which was reported as an adenocarcinoma. Hemoglobin of 8.3 g/dL and hematocrit of 40% was present. A mass in the ampullary region was documented on abdominal sonography. CECT scan (contrast-enhanced computed tomography) abdomen revealed a mass in periampullary region with no evidence of lymphadenopathy or any metastasis. On the fourth day of admission, patient had another episode of a massive UGI bleed  and had the transfusion of three more units of packed red cells. 14 hours after the last episode of UGI bleeding patient had another episode of a malena with hematemesis. Four more units of packed red cells were transfused. After resuscitation, patient had emergency surgical exploration. Pylorus preserving pancreaticoduodenectomy in view of an ampullary mass (adenocarcinoma) was done. No regional lympahadenopthy was present. A polypoid mass measuring 5.0 × 3.2 × 1.5 cms. was seen arising from the second part of duodenum with ulceration in its centre.On cut section, the tumor was tan, white, and yellow and was well demarcated from the normal surrounding tissue . There wNo cytomorphologic atypia was present. Necrosis and mitotic figures were not present Figures . ImmunohDGP is a rare neuroendocrine tumour. The commonest site of origin is the second part of a duodenum, follwing duodenum in gut this can arise from jejunum and pylorus. Lung, bronchus, appendix, mesentery, and nasopharynx are other rare sites in body where gangliocytic paraganglioma can arise , 6. Age Cell of origin of gangliocytic paraganglioma is an embryonic celiac ganglion or from pluripotent stem cells located at the base of the intestinal glands . HamartoThis tumor consists of a complex neoplastic infiltration with a component resembling carcinoid or islet cell tumor, admixed with proliferation of spindled neurofibrillary cells and larger polygonal cells demonstrating gangliocytic differentiation. There may be stromal hyalinization in some cases. Cellular pleomorphism may be prominent, but mitoses and necroses are rare. Previously, it was reported that documented lymph node metastasis has epithelioid cells, but there are reports that metastatic deposits of tumor in regional lymph nodes have the DGP components  .Gangliocytic paraganglioma are considered benign, and treatment is complete excision of tumor. Surgical or an endoscopic resection can be used for excision. Careful assessment prior to local excision is necessary. In case of diagnostic dilemma, lesion is large, or there is a suspicion of lymph node involvement, pancreaticoduodenectomy with lymph node dissection is recommended. For those who had local resection only, meticulous followup is mandatory. Transduodenal laparoscopic resection with intraoperative duodenoscopy is a valuable treatment for benign gangliocytic paraganglioma of the duodenum which is unresectable by upper gastrointestinal endoscopy . Recentl


We report a hitherto not documented case of primary mucinous cystadenoma arising in the spermatic cord within the right inguinal canal of a78-year-old man. The tumor was painless, hard and mobile. A computed tomography scan on the pelvis revealed an oval shaped, low attenuation mass, measuring 5.0x2.5x2.1 cm, that was present adjacent to the vas deferens. Grossly, the excised mass was multicystic mucinous tumor, filled with thick mucoid materials. Microscopically, the cystic wall was irregularly thickened. The cystic epithelium commonly showed short papillae lined by a single layer of columnar to cuboidal mucinous epithelial cells without significant stratification or cytologic atypia. Goblet cells were also frequently present. Immunohistochemically, the neoplastic cells showed positive reaction to carcinoembryonic antigen, cytokeratin 20, CDX2, epithelial membrane antigen, and CD15. However, they were negative for PAX8 and Wilms’ tumor 1 protein. Pathological diagnosis was a papillary mucinous cystadenoma of the spermatic cord. Although mucinous cystadenoma in this area is extremely rare, it is important that these lesions be recognized clinically and pathologically in order to avoid unnecessary radical surgery.http://www.diagnosticpathology.diagnomx.eu/vs/1720965948762004The virtual slide(s) for this article can be found here:  Primary tumors of the spermatic cord can be of many types, but cystadenoma is especially exceptional. To the best of our knowledge, this is the third report of primary cystadenoma in the spermatic cord since the original report by McCluggage et al. in 1996 . The cliA 78-year-old man presented with a painless right inguinal swelling that was firstly noticed 3 months ago. The right inguinal mass was hard mobile. Otherwise, there were no other specific symptoms. During physical examination, spermatic cords could be palpated on both sides. The testis and epididymis appeared normal. Routine blood analysis was all within normal limits. Ultrasound imaging on the right inguinal area showed a lobulated hypoechoic mass, measuring 5.0 cm, without internal vascularity. A computed tomography (CT) scan on the pelvis revealed an oval shaped, low attenuation mass, measuring 5.0x2.5x2.1 cm and showing a well demarcated smooth margin in the right inguinal area , cytokeratin 20 , CDX2  and epithelial membrane antigen . They were also focally positive for CD15 , but negative for PAX8  and Wilms’ tumor 1 protein   duct anlages on the basis of its location and microscopic appearance. In the present case, neoplastic mucinous cells revealed cytoplasmic positivity to CD15 and EMA which was similar to immunohistochemical pattern of epithelial cells of normal vas deferens. Furthermore, the tumor was encircled by a rather degenerated, thin smooth muscle coat that was identified from Masson trichrome stain. Taken altogether, the features would be reminiscent of mesonephric duct anlage structure. Additionally, the tumor cells showed diffuse positive reaction to CK20, CDX2 and CEA which is characteristic of intestinal epithelial cells, and frequent occurrence of goblet cells in the epithelium. These findings of intestinal differentiation are those not expected in normal vas deferens, but may be related to versatile phenotypic expression frequently seen in mucinous neoplasms elsewhere. We also performed PAX8 immunohistochemical stain to identify if there is a possible evidence of Müllerian differentiation in the tumor. PAX8 is a member of the paired box (PAX) family of nuclear transcription factors and is important in organogenesis of the thyroid, kidney, and Müllerian system. PAX-8 is known to be expressed in a variety of ovarian epithelial tumors . In the It is well known that the histology of mucinous cystadenoma is commonly found in female ovary. Generally, ovarian mucinous cystadenomas are large, unilateral, multilocular cystic tumors containing mucoid material. Microscopically, they are composed of glands and cysts lined by a single layer of columnar cells with abundant intracellular mucin. Cellular stratification is minimal, and nuclei are basally located with only mild atypia. Papillary formation in ovarian mucinous cystadenoma is unusual, but when it is present, the papillae are simple and short . These eStrictly speaking, it is very rare for us to be aware of any primary tumors of the spermatic cord within the inguinal region that could readily be confused microscopically with papillary cystadenomas. However, when we would take consideration of differential diagnosis, benign papillary mesothelioma or multicystic mesothelioma should be considered firstly under the list of differential diagnosis. This rare tumor of the tunica vaginalis usually appears in young men. Grossly, it consists of a hydrocele sac with papillary or adenomatous excrescences and cystic or solid areas. Microscopically, the tumor is comprised mostly of variably sized papillary formations with fibrovascular cores and covered by cuboidal, columnar, or flattened mesothelial cells with large vesicular nuclei and glassy eosinophilic cytoplasm ,10, wherJudging from histologic features, the present case mucinous papillary cystadenoma is a benign, noninvasive lesion that is speculated with no capacity for distant metastasis or recurrence, although its precise natural biology is poorly understood due to their extreme rarity.Although clinical follow up observation is relatively short in the present case, the tumor is most likely speculated to be a benign neoplastic process when we combined histological findings with clinical histories . Mucinous papillary cystadenoma is an extremely rare neoplastic lesion of the spermatic cord. Clinically, there are no specific symptoms other than palpable mass, and radiologic imaging could give an impression of cystic or metastatic tumor arising in the inguinal region. The desirable treatment of choice is a local complete excision with sparing of the spermatic cord. However, it is important that the nature of these lesions be recognized clinically and pathologically in order to avoid unnecessary radical surgery.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.CEA: Carcinoembryonic antigen; CK20: Cytokeratin 20; EMA: Epithelial membrane antigen; WT-1: Wilms’ tumor 1 protein; CDX2: Drosophila caudal-type homeobox 2; PAX8: Paired box 8.The authors declare that they have no competing interests.JY-KIM carried out the immunohistochemical staining and wrote the most of the manuscript. YT-LEE collected the patient’s clinical information. HJ-KANG, DY-PARK, and GY-HUH participated in pathological investigations and the review of related references. CH-LEE also participated in pathological investigations, revised manuscript for important intellectual content and had given final approval of the version to be published. All of the authors have read and approved the final manuscript.