Acute pancreatitis is a rare complication of hydatidosis and the successful use of endoscopic sphincterotomy associated with extraction of hydatid membranes has been rarely reported. We describe a young man who developed acute pancreatitis after rupture of an echinococcus cyst, located at the left hepatic lobe, into the biliary tract. The cause of pancreatitis was confirmed by endoscopic retrograde cholangiopancreatography, which revealed the presence of a daughter cyst impacted in the major papilla. After sphincterotomy and removal of hydatid membranes from the biliary tract, the patient presented rapid resolution of pancreatitis and made an uneventful recovery. Hydatidosis is a zoonosis that is generally caused by infection with Echinococcus granulosus. The disease is endemic in many countries, including those around the Mediterranean Sea, Central Asia, Far East and Latin America . Liver iWe herein describe the first case of hydatid acute pancreatitis by documentation of impaction of hydatid membranes in the papillary orifice.A 31-year-old Greek farmer was admitted to the Department of Internal Medicine with pain in the epigastric area that had started 3 days earlier. The pain was intense and constant, radiating to the back and associated with nausea and vomiting. There was no history of drug or alcohol consumption.9/L , eosinophils 0.6 × 109/L , total bilirubin 47 μmol/L , alkaline phosphatase 238 IU/L , gamma glutamyl transferase 312 IU/L , asparate aminotransferase 1 IU/L , alanine aminotransferase 178 IU/L , serum amylase 3254 IU/L , lipase 4234 IU/L , and C-reactive protein 38 mg/L .Clinical examination revealed an acutely ill patient, mildly jaundiced and febrile (38°C) and was remarkable for epigastric tenderness and a palpably enlarged left liver lobe 2 cm below the right costal margin. Laboratory data demonstrated a high leukocyte count of 14.5 × 10Abdominal computed tomography (CT) showed a large multilocular cyst in the left hepatic lobe with rupture into the biliary tract, dilated main biliary ducts (common bile duct diameter 22 mm) and a diffusely swollen pancreas with indurations around it . PositivA hydatid cyst is normally well tolerated in humans until its development results in pressure on adjacent structures . The cysDespite the mechanism of hydatid, pancreatitis is generally considered to be unknown; we believe that it could be explained by the mechanical obstruction of the papillary orifice by daughter cysts resulting in reflux of a mixture of bile and hydatid material into the pancreatic duct and increase of intrapancreatic pressure, which triggers the inflammatory cascade of acute pancreatitis.Our case is very intriguing because for first time an endoscopic image depicted hydatid membranes impacted in the papillary orifice, thus confirming our hypothesis of acute pancreatitis development. The rapid resolution of pancreatitis after endoscopic sphincterotomy and removal of hydatid membranes further strengthens our hypothesis. The efficacy and safety of endoscopic sphincterotomy for the treatment of hydatid cysts with rupture into the biliary tract is well established ,16. HoweWe referred our patient for surgical extirpation of hepatic hydatid cyst because surgery remains the treatment of choice ,2, despiOur case demonstrates that the cause of acute pancreatitis, after rupture of a hepatic echinococcus cyst into the biliary tract, is the impaction of hydatid membranes in the papillary orifice. Endoscopic sphincterotomy and removal of membranes is an effective and safe treatment.


Retiform hemangioendothelioma (RH) is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy. Treatment of RH is controversial. We present a case of a 44-year-old Asian male presenting with an unresectable RH of the pelvis. The patient was treated with concurrent low-dose Cisplatin and External beam Radiation (4140cGy in 180cGy per fraction). This is the first report of a clinical complete response and a long-term local control of this rare tumor. This has significant clinical implication, since it gives the first evidence of treatment of this rare tumor using concurrent low-dose chemotherapy and radiation. Retiform hemangioendothelioma (RH) is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy. Treatment of RH is controversial though most reports have described surgical resection followed by adjuvant radiation (RT) with or without chemotherapy. We describe a case report of a 44-year-old Asian male presenting with an unresectable RH of the pelvis and successful treated with concurrent chemotherapy and radiation.2) was given concomitantly with radiation. The patient tolerated the treatment well and had an excellent clinical response  is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy that has been classified as a distinct type of low-grade cutaneous angiosarcoma. Up to date, a total of 25 cases were described in the literature –11. FirsMorphologically, RH presents as exophytic mass or nodule or a plaque, dermal or subcutaneous, that grows slowly. Most tumors present in young to middle-aged adults Range 9–78 years) . Most le8 years .Ulex earopaeus agglutinin (UEA) (7). The spindle cells are positive for UEA-1 and CD 31 but not for FVIII-Rag and CD34 [Clinically, RH lesions could be located in the dermis (predominantly) with extension into the subcutaneous tissues or striated muscle as was also reported as in our case . A charaand CD34 . In summand CD34 .Other vascular tumors that morphologically can present similarly to RH and need to be differentiated from RH include Dabska's tumor, lymphoma, dermatofibrosarcoma protuberans, hemangioma, bacillary angiomatosis, cutaneous metastases, blue-rubber bleb nevus syndrome, Kaposi's sarcoma, targetoid hemosiderotic hemangioma, hobnail hemangioma, epitheloid hemangioendothelioma, and polymorphous hemangioendothelioma of lymph node , 2, 7, 9 Treatment of choice for RH has been surgical excision with histopathologically tumor-free margins. In the cases reported up to date, patients were treated initially by surgical excision. Approximately 50% of the cases recurred –13. Ampu


We report one case of a 78-year-old woman who referred to our hospital because of a progressive right hemiparesis. On clinical examination a painless large soft mass in the left parietal region was observed. CT and MRI revealed an extra-axial mass in the in the left fronto-temporo-parietal region. The lesion was totally excised despite the bleeding tendency. Histology disclosed the presence of a plasmacytoma. Postoperative, the patient developed an epidural hematoma that required immediate evacuation. On further investigation active tuberculosis was detected. On follow up examination 1 year later no tumor recurrence or evidence of multiple myeloma was detected. Plasmacytes are responsible for the production of antibodies, consisting an important factor of the immune system. Plasmacytomas are referred to benign lesions that may progress to multiple myeloma, a fatal neoplasm . Skull pA 78-year-old Caucasian woman from Greece was referred to our hospital because of a progressive weakness of the right upper and lower extremities and inability to walk. The clinical examination revealed a right hemiparesis, positive ipsilateral Babinski and Barré signs and a painless large soft mass in the left parietal region, which could be easily missed because of her long hair. The patient suffered from headache during the last 6 months, without alterations in mental status, as it was reported from her daughter.99mTc-Tetrofosmin was performed. There was increased radiotracer accumulation consisted with a lesion of high metabolic activity and vascularity [Figure A brain CT was performed and revealed an extradural mass that was homogenous enhance after intravenous contrast administration. There was bone erosion and the lesion had regions of calcifications. MRI that ensued showed a large (9.8 × 9 × 3 cm) extra-axial mass with isointense signal on T1-weighted images, hyperintense signal on T2- weighted images with intense and homogeneous contrast enhancement Figure . The lesy Figure .The patient was operated upon via a left fronto-temporo-parietal craniectomy. Intraoperative a soft purplish, easily separated from dural surface lesion was discovered that was not encapsulated and was originated from the diploe. The overlying skin was normal and the galea was not involved. There was a bleeding tendency, nevertheless the lesion was totally excised and a cranioplasty with bone cement was performed. Histology revealed a highly cellular tumor, composed of monoclonal plasma cells positive for immunoglobin lambda light chain. The diagnosis was plasmacytoma of the skull. Six hours later the patient developed an epidural hematoma that required an immediate evacuation Figure . The patPostoperative the patient received treatment for tuberculosis  and adjuvant treatment for the plasmacytoma . One year after the initial diagnosis the patient was free of recurrence without having received additional radiotherapy.Multiple myeloma has an incidence of 4 cases per 100.000/year and constitutes approximately 1% of all malignant neoplasms and 15% of all blood neoplasm's . It is cPlasmacytoma of the skull is a rare finding . It may 99mTc-TF in order to functionally characterize the lesion. 99mTc-TF is a lipophilic cationic diphosphine routinely used for myocardial perfusion imaging. Its whole uptake mechanism depends mainly on regional blood flow and cell membrane integrity [99mTc-TF that is explained by the high vascularity and metabolic activity of the tumor, but also by the lesion's absence of blood-brain barrier which allows leakage of the 99mTc-TF from the cells. Nevertheless, further investigation is necessary to determine the usefulness of 99mTc-TF brain SPECT in the diagnosis of this tumor type.On radiological investigation CT and MRI may provide clues to narrow the differential . We alsontegrity . In our Total surgical resection followed by adjunctive radiation therapy has been advocated as an effective treatment in the majority of skull plasmacytomas . NeverthTo conclude, plasmacytoma of the cranial vault is a rare clinical entity that demands neurosurgeon's and hematologist's cooperation. Laboratory and imaging evaluation are of paramount importance to exclude systematic involvement. Careful surgical resection if total may be adequate for the disease control. Nevertheless, close follow-up with regular life-long examinations are important in order to avoid a generalized incurable disease.99mTc-TF: 99mTc-Tetrofosmin.CT: computed tomography; MRI: magnetic resonance imaging; 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.SV and DP conceived of the study. AZ, GA and SV drafted the manuscript. AZ, DD, GF collected the data and performed the literature search. EM and DP are consultants in charge for the patient. ST and AF performed the SPECT study and analyzed the results. GA and EM participated in its design and coordination. GA, SV, AF and ST critical review the final version of the manuscript. All authors read and approved the final manuscript.


Mantle cell lymphoma (MCL) is a moderately aggressive variety of non-Hodgkins lymphoma. Extranodal presentation of MCL is well known, the intestine being a common site. The incidence of colorectal involvement is relatively rare. Moreover, the recurrence of the disease at laparotomy scar site is even more rare. We report an unusual case of incision line recurrence in a case of colonic MCL occuring three years after initial treatment. Mantle cell lymphoma (MCL) is a moderately aggressive variety of Non Hodgkins Lymphoma (NHL) accounting for about 7% of adult NHL. A signifA 62-year-old male gave a history of pain in left upper quadrant of abdomen for which he underwent investigation in 2005. He had suffered altered bowel movements, which had been progressively increasing over a period of three years. He was found to have a lump in left upper quadrant of abdomen with features suggestive of sub acute intestinal obstruction. There was no associated history of bleeding per-rectum. He did not have any B symptoms and CT scan of abdomen revealed a single large proliferative mass in the splenic flexure of the large gut. Colonoscopic biopsy was performed and was reported as lymphomatoid variant of Mantle cell lymphoma of the large gut. He subsequently underwent left hemicolectomy with ileo-colic anastomosis. Postoperative histopathology confirmed the colonoscopic biopsy report. Unfortunately, no immunohistochemistry was performed at this stage. His bone marrow study and staging workup were negative. He was thus classified as Stage IE with IPI score of 1. He subsequently underwent six cycles of chemotherapy using the CHOP  regime. He had been on regular follow-up since then and had remained asymptomatic.He had recurrence of abdominal pain in December 2008. This time he was found to have a mass in the supra umbilical region at the site of laparotomy incision scar . He alsoet al. It is a distinct clinicopathologic entity of NHL, characterized by a monotonous proliferation of small- to medium-sized lymphocytes with co-expression of CD5 and CD20 with CD10 and CD 23 negativity and moderately aggressive and incurable clinical course. MCL comprises 2.5-7% of all NHL, and the GI tract is involved in about 20% of cases.[Primary gastro-intestinal (GI) tract lymphoma accounts for only 1 to 10% of all GI malignancies. Nevertheof cases. The mostThe prognosis of GI MCL is guarded. Response to chemotherapy is seen in up to half of the patients. COP (cycRadiotherapy has limited role in management of mantle cell lymphoma. Surgery too is of little use except in making tissue diagnosis. Chemotherapy remains the mainstay of treatment. The pattern of recurrence seen in this particular case might be a result of per-operative seeding of disease at anterior abdominal wall. This in itself is a very rare mode of disease recurrence and is rarer in gastrointestinal lymphoma cases. It is possible that avoidance of laparotomy might have prevented the morbidity of anterior abdominal wall recurrence in the first place. This case, hence, reiterates the importance of IHC in management of lymphomas and suggests the futility of major surgical intervention in mantle cell lymphomas.


The high frequency of local recurrence occurring after endoscopic piecemeal resection (EPMR) for large colorectal tumors is a serious problem. However, almost all of these cases of local recurrence can be detected within 1 year and cured by additional endoscopic resection. We report a rare case of recurrent advanced colonic cancer diagnosed 11 years after initial EPMR treatment.A 65-year-old male was diagnosed with a sigmoid colon lesion following a routine health check-up. Total colonoscopy revealed a 12 mm type 0-Is lesion in the sigmoid colon, which was diagnosed as an adenoma or intramucosal cancer and treated by EPMR in 1996. The post-resection defect was closed completely using metallic endoclips to avoid delayed bleeding. In 2007, at the third follow up, colonoscopy revealed a 20 mm submucosal tumor (SMT) like recurrence at the site of the previous EPMR. The recurrent lesion was treated by laparoscopic assisted sigmoidectomy with lymph node dissection.When it is difficult to evaluate the depth and margins of resected tumors following EPMR, it is important that the defect is not closed in order to avoid tumor implantation, missing residual lesions and to enable earlier detection of recurrence. It is crucial that the optimal follow-up protocol for EPMR cases is clarified, particularly how often and for how long they should be followed. Endoscopic mucosal resection (EMR) is indicated for the treatment of adenoma and intramucosal or submucosal superficial (SM1: less than 1000 μm from the muscularis mucosa) colorectal cancers because of its minimal invasiveness, negligible risk of lymph-node metastasis-4. Howeven bloc EMR. However, as a result the lesion was removed by piecemeal resection (2-pieces). The post-resection defect was closed using metallic endoclips.The patient was a 65-year-old male with a history of radical prostatectomy for prostate cancer. Following a positive faecal occult blood test, a total colonoscopy was performed at a previous hospital in 1996 and a sigmoid colon lesion was identified. He was referred to our hospital for more precise examination and treatment. Colonoscopy revealed a 12 mm type 0-Is lesion in the sigmoid colon. We diagnosed the lesion as an adenoma or an intramucosal cancer and tried to remove this lesion by Histopathological examination revealed a well differentiated adenocarcinoma with low grade atypia, and the depth of invasion was intramucosa without lymphovascular invasion, cut end margin negative Figure . We consIn 2007, the third follow up colonoscopy revealed a protruding submucosal tumor (SMT), 20 mm in size at the site of the 1996 EPMR Figure  and 4. TIn this case, preoperative examination and histopathological findings revealed no evidence of prostate cancer recurrence. The gross configuration of the SMT-like lesion did not support the diagnosis of a primary colonic cancer, and the lesion was diagnosed as a recurrence of the sigmoid colon cancer previously removed by EPMR, with the biopsy specimen very similar to the initial EPMR specimen.Endoscopic resection for early colorectal cancers has been used throughout the world since the 1970 s and EMR with a submucosal injection technique allows the removal of large colorectal lesions. However, local recurrence frequently occurs after EPMR, which is a serious problem,6. PreviResults from this case do not support the need for routine long-term follow up colonoscopy. However in cases where it is difficult to evaluate truly the surgical margin and depth of invasion after EPMR, it is important in order to avoid missing residual lesions and to detect recurrent lesions earlier, that where suitable the defect is not closed and follow up colonoscopy should be performed at appropriate intervals.EPMR: endoscopic piecemeal mucosal resection; ESD: endoscopic submucosal dissection; EMR: endoscopic mucosal resection; SMT: submucosal tumor.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 journalThe authors declare that they have no competing interests.TK collected the data and wrote the report, and was involved in drafting the manuscript. TS was involved in drafting the manuscript. TM revised the manuscript critically for important intellectual content. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/10/87/prepub


Acinar cell carcinoma (ACC) represents only 1–2% of pancreatic cancers and is a very rare malignancy. At the time of diagnosis only 50% of the tumors appear to be resectable. Reliable data for an effective adjuvant or neoadjuvant treatment are not available.A 65-year old male presented with obstructive jaundice and non-specific upper abdominal pain. MRI-imaging showed a tumor within the head of the pancreas concomitant with Serum-Lipase and CA19-9. During ERCP, a stent was placed. Endosonographic fine needle biopsy confirmed an acinar cell carcinoma. Laparotomy presented an locally advanced tumor with venous infiltration that was consequently deemed unresectable. The patient was treated with five cycles of 5-FU monotherapy with palliative intention. Chemotherapy was well tolerated, and no severe complications were observed. Twelve months later, the patient was in stable condition, and CT-scanning showed an obvious reduction in the size of the tumor. During further operative exploration, a PPPD with resection of the portal vein was performed. Histopathological examination gave evidence of a diffuse necrotic ACC-tumor, all resection margins were found to be negative. Eighteen months later, the patient showed no signs of recurrent disease.ACC responded well to 5-FU monochemotherapy. Therefore, neoadjuvant chemotherapy could be an option to reduce a primarily unresectable ACC to a point where curative resection can be achieved. Symptoms like weight loss, abdominal pain, nausea and vomiting are non-specific and mostly related to either locally advanced tumors or metastasis [Acinar cell carcinoma (ACC) of the pancreas is a very rare tumor entity, since only 1% of the exocrine pancreatic malignancies derive from acinar cells [tastasis .In general, half of the patients have advanced disease with either metastasis or a locally unresectable tumor at the time of diagnosis. Median overall survival at this stage is about 19 months, and has been reported to be between ductal adenocarcinoma of the pancreas  and pancreatic neuroendocrine neoplasma  . After tUp to now, ACC of the pancreas has been described by few case reports ,5-7. OnlWe report for the first time a partial tumor remission under mono-chemotherapy with 5-FU and subsequent potential curative resection of a primarily unresectable ACC.A 65-year old male with obstructive jaundice (bilirubin 126 μmol/l) and non-specific upper abdominal pain was admitted to the hospital in December 2005. Laboratory parameters showed pathological values: ALAT/ASAT 10.2/4.5 μmol/l (< 0.85/< 0.85 μmol/l), YGT 23.1 μmol/l (< 1.19 μmol/l), amylase/lipase 5.1/23.2 μmol/l (0.22–0.88/< 1.00 μmol/l) with a mild elevation of the inflammatory parameters (leukocytes 13.6 gpt/l and CRP 22 mg/l), and CA 19-9 was elevated to 968.8 U/ml. The tumor markers CEA and AFP were within the normal ranges. There were no signs of subcutaneous fat necrosis or arthralgia.On MRI T1 weighted images, an isointense tumor in the pancreatic head was visible. The axial diameter of the tumor was about 4 cm; it contacted the portal vein and the superior mesenteric vein and it seemed to infiltrate the hepatoduodenal ligament. Two enlarged lymph nodes were noted at the upper and lower margin of the pancreatic head Figure . DiffereIn the histology of the tissue, the normal acinic structure of the exocrine pancreas was effaced. The irregularly shaped tumor cells with eosinophilic cytoplasm had rounded dark nuclei and were growing in solid sheets. No ductal structures could be observed in the tumor tissue Figure . Markers2/d); treatment occurred over eight weeks with a break of two weeks per cycle. There were no major complications during the chemotherapy. Only one time the patient showed mild diarrhea and a common cold. An uropathy was treated with systemic antibiotics. Overall, the patient stayed in good general condition. The biliary stent was changed twice without any complications. CA 19-9 was determined as a tumor marker after each chemotherapy-cycle. After the second cycle, CA 19-9 already showed a normal value of 11.0 Units/ml; after one year, CA 19-9 was 18.6 Units/ml. After five cycles of treatment (one year), a CT for restaging was performed again. The images showed an obvious reduction in the size of the tumor. The slightly hyperintense area in the head of the pancreas had diameters of barely 2.4 × 1.1 cm, with a small (1.0 cm) residual precaval lymph node  in the pancreatic head was confirmed. Transcutaneous abdominal ultrasound showed no signs of unresectability or metastatic spread. The PET scan indicated no vital tumor tissue in the pancreatic head.We decided upon operative reexploration with the aim of total tumor resection. A PPPD without any intraoperative complications was performed; because of adhesions at the portal vein, a partial resection (3 cm) of the vessel had to be carried out simultaneously. All intraoperative histopathological examinations of the resection margins were without any tumor tissue. The postoperative hospitalization stay proceeded regularly and without any adverse events.The histopathological examination of the resected tumor tissue showed areas of necrosis surrounded by fibrotic stroma with a folliculoid lymphatic reaction Figure . In betwAny further adjuvant therapy was refused by the patient. Regular checkups were done in our pancreatic clinic. As of now, about 18 months after operation, the patient shows no clinical symptoms or ultrasonographic or laboratory signs of recurrent disease. CA 19-9 is 8.1 U/ml.Acinar cell carcinoma of the pancreas is a very rare tumor entity, and in the literature there are only small retrospective studies and a few case reports on this matter ,2,4,9-11Commonly referred clinical symptoms are upper abdominal pain, weight loss and nausea/vomiting ,2. In ouThe initial elevated tumor marker CA 19-9 (968.8 Units/ml) was used to monitor the efficiency of the therapy, especially because we could not detect any elevation of other tumor markers, e.g., AFP, which were mentioned as possible markers of acinar cell differentiation within these tumors ,13,14.Tatli et al.  describeThe diagnosis of an ACC cannot be made with a high reliability unless histopathological examination has been performed . But in Endocrine and acinar tumors, as well as even rarer pancreatoblastoma, normally lead to few formation of fibrosis and thus are often cell-rich tumors with cells not arranged orderly and rounded inconspicuous nuclei. Differentiation between endocrine and acinic tissue can be achieved by immunohistology, because endocrine tissue will react with antibodies against synaptophysin or chromogranin. Antibodies against trypsin or lipase can be used to prove acinic differentiation, but, as ACC are scarce, only a few centers have these antibodies in stock or are accustomed to using it ,17. WithReviewing the literature, operative resection of the tumor seems to be the only curative treatment of ACC ,2,4,12. However our knowledge of the efficiency of any neoadjuvant or adjuvant treatment with radiation or chemotherapy for ACCs is limited. Only in small studies or case-reports are the effects in patients with mostly unresectable ACCs described ,7,12. FoHowever, our case is unique and of great importance, since never before has any reduction of the tumor size (from 4 cm to 2.4 cm) under neoadjuvant mono-chemotherapy with 5-FU been observed, followed by the curative resection of an ACC. Comparison of the histology of the biopsy and the surgical specimen showed large differences, because after chemotherapy there were necrotic areas, and the vital tumor tissue was much reduced. Highly cellular tumor tissue of relatively uniform nuclei had vanished, and instead, little groups and scattered single tumor cells were fenced in by dense fibrosis, and cellular pleomorphism indicated degenerative changes after chemotherapy.As mentioned above, we used the chemotherapeutical agent 5-floururacil. 5-FU is commonly used for adjuvant treatment of pancreatic ductal adenocarcinoma. The ESPAC-1 trial especially showed significantly positive impact on long-term survival in patients with PDA .In the present case, after neoadjuvant treatment with 5-FU only minimal tumor tissue remained in situ, and it could be removed completely with negative margins (R0) by surgery. Therefore, 5-FU seems to have a significant effect on ACC tumors, as shown in our patient. However, any final statement is not possible yet, as it has only been 18 months since surgery.Considering our case, it is important that any patient with an ACC be restaged, and, if possible, re-explored in a specialized high volume center in particular, because surgical resection seems to be the only curative therapy. If the ACC is unresectable, the patient should be treated by 5-FU chemotherapy either with a neoadjuvant or palliative intention.Chemotherapy is not yet effective in pancreatic ductal cancer. Thus, effects of chemotherapy on tumor tissue are normally not seen, unlike in other cancers, and no grading system can be applied. Acinar cell carcinomas develop by different pathways than ductal carcinomas. As shown in this case, neoadjuvant chemotherapy with 5-FU might well be a valuable step in the management of these patients.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.MD, FR, SK and RG performed surgery, follow up patient and helped in preparation of the manuscript. FD prepared the draft of the manuscript. CS prepared the pictures of the manuscript. Histopathological examinations were performed by DDD. All authors read and approved the final manuscript.


We report a rare case of a voluminous vaginal myxoid leiomyoma in a 27-year-old nulliparous woman. Magnetic Resonance Imaging (MRI) revealed a mass arising from the vagina, with inhomogeneous signal intensity on spin-echo T1W and T2W images. MRI accurately defined the tissue planes between the lesion and the adjacent structures and suggested its benign nature. The mass was completely resected by means of transvaginal approach and the diagnosis of myxoid leiomyoma was confirmed histologically. To the best of our knowledge, this is the first report describing the MRI features of vaginal myxoid leiomyoma. Leiomyoma is the most common benign mesenchymal tumor of the vagina in adult women. Since thWe report a rare case of a voluminous myxoid leiomyoma arising from the vagina. The emphasis is on the value of MRI in the diagnosis and management of such a lesion.A 27-year-old nulliparous woman was admitted to our hospital for dyspareunia, pressure symptoms on the urinary tract, vaginal pain, and fetid vaginal discharge for 20 days. On vaginal exploration there was a palpable, soft mass in the anterior wall of the vagina. Transvaginal Ultrasound (TVUS) showed a round (approximately 7 cm in diameter) heterogeneous pelvic mass, with well-defined contours , arisingIn order to confirm the tumor origin and its relationships with adjacent organs, we performed MRI using a 1.5-T superconducting system . Sagittal, axial, and coronal T2W (TR / TE = 5000 / 100) fast spin-echo images and axial T1W (TR / TE = 460 / 14) spin-echo images, using a section of 7 mm and an intersection gap of 1 mm, were obtained.Axial T1W MRI revealed a well-circumscribed, isointense, relatively heterogeneous, rounded (7.5 cm in diameter) pelvic mass that displaced and compressed the urinary bladder anteriorly . On axiaSurgical excision by means of a transvaginal approach was performed. Gross examination revealed a well-circumscribed soft mass, with a maximum diameter of 7.5 cm. The histology showed the typical features of a myxoid leiomyoma . AbundanVaginal leiomyomas may be up to 5 cm in size and may sometimes simulate malignancy . Knowledge of tumor features, especially with regard to tumor margins, relationship with adjacent structures, and size, is essential for differentiating between benign and malignant lesions preoperatively.The value of MRI in the evaluation of vaginal masses has already been established. On MRI, 6et al., –12 10–12et al. did the et al.In our case, the T2W coronal images established the tumor origin by demonstrating acute angles between the lesion and the vagina. In spite of the large size and the inhomogeneous signal intensity of the mass on T1W and T2W images, MRI suggested the benign nature of the tumor on the basis of its regular contours and the absence of invasion of the adjacent pelvic organs. Also, MRI provided useful information for conservative surgical treatment (enucleation of the tumor).


Neuromuscular and vascular hamartoma (NMVH) is a very rare stricturing condition of the small intestine, occurring focally and causing recurrent obstructive symptoms or occult chronic gastrointestinal bleeding. Salas et al. : 705-9) proposed the term of "Neuromesenchymal hamartoma" for the cases of NMVH with participation of mesenchymal tissues.We present the case of a 60-year-old male patient admitted twice in a month with abdominal pain. On the third admission with clinical signs of acute abdomen, an exploratory laparotomy was performed. The clinical and laboratory findings that occurred after the patient's evaluation, the intraoperative findings and the pathological features of this lesion are reported. Neuromuscular and vascular hamartoma (NMVH) is a very rare stricturing condition of the small intestine, occurring focally and causing recurrent obstructive symptoms or occult chronic gastrointestinal bleeding. It is a hyperplasia of varied tissue of the submucosa such as smooth muscle bundles, peripherals nerve tracts, vessels and ganglia. In our case there is an additional pathological feature: the diffuse fatty infiltration of the submucosa. Salas et. al. proposed the term of "Neuromesenchymal hamartoma" for the cases of NMVH with participation of mesenchymal tissues .th day. The past medical history included hypertension and neither previous gastrointestinal disease nor surgical intervention was reported.A 60-year-old male patient was admitted twice in a month with abdominal pain and sings of intestinal obstruction on X-ray figure . Both tiA thorough clinical examination including colonoscopy, gastroscopy, computed tomography (CT) scan and magnetic resonance imaging of the abdomen was performed. Endoscopic procedures revealed no pathology. On CT scan a mass was diagnosed involving the mesentery in close proximity with a loop of small intestine with thickened wall. figure .Laparotomy revealed a single 30 cm long stricture of the small intestine with brown discoloration extending approximately 10 cm and edema of the bowel wall and mesentery. Enlarged mesenteric lymph nodes were present as well.Presuming Crohn's disease or other colitis, 80 cm length of small intestine was resected and anastomosed. Cholecystectomy was also performed due to cholelithiasis. The postoperative course of the patient was uneventful.Grossly the specimen showed a stenosis of about 25 cm, the serosa in that region was thick with a bluish discoloration of 10 cm length. The mucosa was brown, friable with superficial ulcerations and absence of plicae mainly at the centre of the stenosis. The mesenterium was obvious thick with a nodular appearance figure .Microscopically the submucosa was extremely thick with extensive fatty infiltration and mixing of abnormal intermingled non-myelinated nerve bundles, interspersed large ganglion cells, hyperplastic smooth muscle tissue that originates mainly from the thickened muscularis mucosa and less from the muscularis propria, and hemangiomatous vessels figure , 5, 6.The vessels showed muscular hypertrophy and locally ectasia and were observed also in the serosa. Small areas of fibrosis were seen everywhere. The mucosa was ulcerated and severe inflammed by lymphoplasma cells and less eosinophils. Foci of inflammation were seen in the subserosa. There was no transmural inflammation, granulomas or fissuring. No microorganism with histochemical stains was observed. Hyperaemic, thick wall vessels were observed in the mesenterium. The regional lymph nodes showed no significant morphological alterations.The neuromuscular and vascular hamartoma of small bowel has been reported in <15 cases up to today . In all In summary, the gross characteristics and the morphological features, the clinical history and the postoperative health status of the patient support the diagnosis of Neuromesenchymal hamartoma of the small bowel.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.ET was responsible for editing, english editing, search of the literature. GV was responsible for english editing, correction, editorship of the manuscript. NS was responsible for the search of the literature. KP was responsible for english editing, correction. KM was responsible for the histology consulting and pathology examination. KC was responsible for editing.


Eosinophilic granuloma is very rare benign bone tumor which presents in more than 90% in children under the age of ten. There is predominance for males. It is usually found at flat and long bones. The skull and vertebral spine is often affected. We report a case of 57 year-old man who gradually developed local pain at his skull and orbit. A soft, movable, palpable and tender mass was found at the left temporal bone. The pain deteriorated after an accidental injury at skull and remained so. The clinical examination revealed no pathological findings. The patient was a doctor who smoked and consumed alcohol daily. He had a history of cardial infraction and psoriatic arthritis. X-rays and CT revealed a round lytic defect at the skull. Its borders were sharp and its size was 1.6 × 1.8 cm. No periostic reaction or bone formation was noted. Scintigraphy depicted a lytic lesion without radionuclide enhancement. Thus we suspected an eosinophilic granuloma. An attempt to excise the tumor failed as it had already eroded the underlying temporal bone. The external meninga was affected but not the internal one. Histological diagnosis with dominance of Langerhans cells set the diagnosis. A second surgery was done and the eosinophilic granuloma was extracted. After eight months the gap was bridged with plastic heterologous transplant. After the curettage the patient received antibiotics and five cycles of radiotherapy. The aesthetic result was excellent. The patient's head has a normal hairy appearance. No tenderness, swelling or recurrence is recorded until now.Eosinophilic granuloma is of unknown aetiology but uncontrolled proliferation of Langerhans cells, previous inflammations or tumors and autoimmune disorders are suspected. Due to the co-existence of psoriatic arthritis and eosinophilic granuloma to our patient we assume that an autoimmune mechanism is probable. Eosinophilic granuloma of bones is the mildest and commonest manifestation of Histiocytosis-X malady . It affeWe present a case of a man at the age of 57 who developed eosinophilic granuloma at the skull without having any previous history of bone tumors but psoriasic arthritis. To our knowledge this is the first reported case of co-existence of the two diseases.A 57-year-old man was admitted on March 2006 to our hospital referring acute pain when accidentally injured by a plastic object at head. He mentioned a palpable, soft, immovable and tender mass at the left temporal bone. His left orbit was also painful. He had already used analgesic and anti-inflammatory drugs. No fever, elevated regional temperature nor lymphadenopathy was recorded. The physical and neurological examination had no pathological signs. He did not present numbness, weakness or remarkable tendon reflexes and muscular tone. The mobility of the head was normal. The vision and hearing was not affected.The patient had a height of 1.78 m and weighted 80 kgrs. He belongs to Arabic nation (born in Lebanon) but he has the Greek ethnicity living in Greece the last 40 years. 31 years ago psoriasic arthritis was diagnosed with the joint of the left elbow mainly affected. He received no therapy for the disease. 10 years ago he presented acute myocardial infarction and undertook bypass. Since then he received drug therapy for hypertension. The patient smoked 2 packs of cigarettes since the age of 25 and consumed 1-2 glasses of alcohol per day. No family history of tumors is known.Blood examination revealed no pathological findings, neither elevation of leukocytes nor of erythrocyte sedimentation rate. Heart ultrasound revealed no present pathology.A first attempt to excise the lesion failed as there was an erosion of the temporal bone. Material for histological examination was extracted and we found a domination of histiocytes. No malignancy was suspected. We continued with radiological depiction of the skull.Plain radiographs demonstrated a large oval-shaped osteolytic area in the left temporal bone figure . Its borA radical excision of the lesion on health borders of 2 cm was performed. The tumor was gelatinous and brownish without hematomas. It was perforating the whole diploe and part of the external meninga. We excised a piece of a diameter of 5 cm including the lesion and used a periosteal transplant to bridge the gap. No cranioplasty was done at first time. The patient received vancomycin and cefoxitime for two days. Material was sent for histological examination which showed many Langerhans cells, eosinophils, neutrophils and lymphocytes. No fibroblasts or giant cells were found. The S-100, CD1a and Langering proteins were positive. There were necrotic elements too. Diagnosis of eosinophilic granuloma of the skull was set. The patient was administered five cycles of low-dose post-surgical radiotherapy were administered.Carnioplasty with a heterologous transplant (Porex) was performed eight months later. The aesthetic result was excellent and there were no complications. On March 2008, a new CT of the brain depicted no further lesion and the aesthetical result was excellent.Eosinophilic granuloma is one of the rarest bone tumors representing less than 1% of them. In 90% of the reported cases it appears in children under the age of ten. There is a certain predilection to males (2:1) . It usuaRadiological depiction of eosinophilic granuloma is necessary as to determine the activity and nature of the tumor. Plain radiograph depicts its size and borders. The cortex of the affected bone may present as thin, eroded or thickened due to new bone formation . EspeciaThe aetiology of eosinophilic granuloma remains unknown but recently there have been some assumptions. The prevailing ones are inflammatory processes, autoimmune disorders and an out of control proliferation of Langerhans cell . It is nEosinophilic granuloma does not lead to malignant transformation. If it expands elsewhere but bones then is called Hand-Schuller-Christian disease and may manifest as diabetes insipidus, cerebellar, hypothalamic and with other central nervous system symptoms. The prognosis depends on the age of diagnosis and the number of foci.This tumor may heal without treatment but there is a chance of relapsing during the first year after diagnosis. Thus, even after surgical curettage a careful follow-up must be conducted. If a secondary lesion appears then chemotherapy or further excision has to be done .This is one of the rarest cases of eosinophilic granuloma of the skull developing in an adult patient. This is the first reported case of co-existence of eosinophilic granuloma with psoriatic arthritis. It is important to include eosinophilic granuloma in the differential diagnosis of bone lesions in adult subjects because of the possible expansion of the disease if untreated. We suggest surgical curettage and plastic bone transplant at second time and radiotherapy as principal treatment. A follow-up of a year is necessary for probable recurrence.CT: computed tomography; IL: interleukin; MRI: magnetic resonance imaging; PG: prostaglandin.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors contributed equally to this work. PK concentrated the patient's data, histological examinations, history and radiological depictions. PK and AV interpreted the findings and contributed in writing the manuscript. AM and GP analyzed the patient data, reviewed the current literature and contributed in writing the manuscript. All authors read and approved the final manuscript.


Undifferentiated Liver Sarcoma, also known as Undifferentiated Embryonal Sarcoma of the Liver, is a rare, highly malignant neoplasm which affects mostly the pediatric population, although a few cases have been reported in adults. It accounts for about 13% of pediatric hepatic malignancies.We report a case of undifferentiated liver sarcoma in a 14-year-old Chinese boy who presented with non-specific right hypochondriac pain. Exploratory laparotomy with tumor resection was performed, followed by adjuvant chemotherapy.Undifferentiated Liver Sarcoma is a rare, highly malignant hepatic neoplasm affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence shows that long-term survival is possible after complete surgical resection and postoperative chemotherapy. Undifferentiated Liver Sarcoma (ULS), also known as Undifferentiated Embryonal Sarcoma of the Liver, is a rare, highly malignant neoplasm which affects mostly the pediatric population, although a few cases have been reported in adults. It accounts for about 13% of pediatric hepatic malignancies. Only about 150 cases have been reported in the literature. Undifferentiated Liver Sarcoma was named as a separate entity by Stocker et al on the basis of an Armed Forces of Pathology (AFIP) series. [A 14 year old Chinese boy presented to a district hospital with a 5 day history of non-specific right hypochondriac pain. There was no history of jaundice, fever, anorexia or weight loss. He did not have any other associated symptoms. He had no significant past or family history, and no history of drug intake or allergies. His general physical examination was unremarkable. Yellowish discoloration of skin or sclera, spider naevi and palmar erythema all were absent. Systemic examination revealed massive hepatomegaly. His blood count and liver function tests were normal. Alpha fetoprotein was also normal. Ultrasonography revealed a large mass in the right lobe of the liver. He was referred to our hospital for further management. Contrast enhanced CT Scan revealed a large, hypodense mass of 14 × 15 × 15 cm in the right lobe of the liver. Figure . SarcomaOur patient's postoperative recovery was uneventful. He was given 6 cycles of adjuvant chemotherapy, AIM Regimen , which was well tolerated. A post-chemotherapy CT scan showed no signs of recurrence and our patient is alive and well 6 months after surgery.Undifferentiated Liver Sarcoma is a rare, highly malignant neoplasm almost exclusively found in the pediatric population although a few cases have been reported in adults. Undifferentiated Liver Sarcoma was described as a separate pathological entity by Stocker et al in 1978. .Primary hepatic neoplasms are the third most common solid malignant tumors in children after Wilms Tumour and Neuroblastoma and they account for about 2% of total solid pediatric malignancies. Primitive mesenchymal tumor, although rare, is the fourth most common pediatric malignant hepatic tumor following Hepatoblastoma, Infantile Haemangioendothelioma and Hepatocellular Carcinoma. Primitive mesenchymal tumors represent about 9%-15% of all hepatic tumors in children. Only about 150 cases have been reported in the literature.ULS is a malignancy of older children with the majority of cases diagnosed in children between the ages of 6–10 years. The incidence decreases after 10 years of age. There is a slight male preponderance in children (1:0.65).  The righStocker et al presented a series of 31 cases which showed ULS as having a poor prognosis with median survival of less than a year. Recent evidence shows that, with the advent of modern surgical procedures and the use of pre and post operative chemotherapy, there has been an increase in the overall survival rate. A recent review of published cases revealed a better outlook with 37.5% of patients alive without disease for an average of 37.5 months.[The clinical features of ULS are often non-specific and may have varied presentation ranging from sharp abdominal pain, fever, anorexia, diarrhea or a solitary liver cyst. In our case the only presenting complaint was non-specific right hypochondriac pain. Fever is usually due to hemorrhage and necrosis. Rupture into the tumor, peritoneal cavity or pleura can be present.  JaundiceRadiographs of the abdomen are usually normal. The lesion can be detected by ultrasound, CT and MRI. MRI localizes the lesion more accurately than the other methods, with good resectability correlation. It also can detect vascular invasion, biliary obstruction and hilar adenopathy. .ULS is a neoplasm with a primitive mesenchymal phenotype. Tumor size often exceeds 10 cm and they can be as large as 30 cm. Buetow et al reviewed the pathological and radiological findings in 28 cases of ULS.  PathologAlthough there is no standard treatment mentioned in the literature for Undifferentiated Liver Sarcoma, surgery with neo adjuvant or adjuvant chemotherapy  remains ULS: Undifferentiated Liver Sarcoma; UELS: Undifferentiated Embryonal Liver Sarcoma; PCK: Phosphoenolpyruvate Carboxy Kinase; EMA: Epithelial Membrane Antigen.The author(s) declare that they have no competing interests.All authors read and approved the final manuscript.Written consent was obtained from the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal.


Vernix Caseosa peritonitis is a rare post caesarean section complication with only 19 case reports in the literature to date. Vernix caseosa spilt at the time of caesarean section is thought to incite an inflammatory reaction, causing symptoms resembling an acute abdomen.We discuss three Caucasian patients (aged 32 to 43 years) who presented in our health sector in Sydney with vernix caseosa peritonitis. Each had a protracted course with significant comorbidities requiring surgical and medical intervention. This contrasts with other reports suggesting that a rapid resolution can be expected.This cluster may be a consequence of the rising caesarean section rate, a heightened local awareness of the condition and possibly a result of leaving material in the paracolic gutters intraoperatively.Our aim is to increase awareness among our obstetric and surgical colleagues of the characteristic clinical presentation and intra-operative findings of vernix caseosa peritonitis. We also point out that, in contrast to those presented here, not all patients require laparotomy. There have been only 19 cases of vernix caseosa peritonitis (VCP) reported in the literature to date . We presThe typical presentation is severe abdominal pain -12,14, pPreviously, many additional and often extensive excisional procedures have been performed on suspicion of other pathologies such as appendicectomy ,3,5,7,9,9/L) was present but both an abdominopelvic CT and CT pulmonary angiogram (CTPA) were normal. At laparotomy, cheese-like fibrinous debris was found throughout the peritoneal cavity. Peritoneal lavage was performed and an omental biopsy obtained after exclusion of other pathologies and on suspicion of VCP. Histological examination showed acute fibrinous inflammation of the serosal surface and early epithelioid granulomata associated with fetal squamous cells. Antibiotics were commenced and her symptoms rapidly resolved allowing discharge 5 days post-laparotomy. Five weeks later, she was readmitted to hospital for management of recurrent abdominal pain. All her blood tests and an upper abdominal ultrasound were normal. She was managed conservatively and discharged 3 days later. Ten weeks post-delivery, she represented for a second time, now with a persistent discharge from the edge of the abdominal incision. An abdominal wall wound sinus originating superficial to the rectus sheath was identified and excised allowing a rapid resolution of her symptoms. A collection of fetal squamous cells and a granuloma containing VC was observed within the sinus tract that was lined with inflammatory granulation tissue containing aggregates of histiocytes and multi-nucleated giant cells. Two years later, she has continued to have ongoing pain culminating in a recent laparoscopic excision of a VC abscess on the anterior abdominal wall adjacent to the liver, confirmed on histopathology.A 40-year-old nulliparous Caucasian woman was delivered by an uncomplicated elective lower segment caesarean section (LSCS) at 39 weeks gestation for breech presentation after an unremarkable antenatal course. She developed generalised peritonism, dyspnoea and pyrexia (39.3°C) 5 days later. A neutrophilic leucocytosis (white cell count (WCC) 15 × 10A 32-year-old primiparous Caucasian woman had previously had a normal vaginal delivery but underwent elective LSCS at 39 weeks gestation for breech presentation. She had had a normal medical and antenatal history and although the LSCS was uncomplicated, she developed abdominal pain, flu-like symptoms and myalgias 3 days post-delivery. A diagnosis of urinary tract infection was made on the sixth postoperative day as she now complained of dysuria and her temperature was 41°C. Oral antibiotics were commenced and her condition improved, allowing her to be discharged. On day eight, she represented with further pyrexia, dysuria and severe abdominal pain requiring opiate analgesia. Urine culture was negative and an abdominopelvic ultrasound scan was normal. Once more, her symptoms settled with intravenous antibiotics and she was discharged a week later. She continued to experience intermittent episodes of pain culminating in a third admission 3 months postpartum. On this occasion, abdominopelvic CT and MRI identified an 8 cm inflammatory mass involving the wound. Laparotomy revealed a wound abscess and sinus formation tracking to the broad ligament that required extensive debridement and division of adhesions. Biopsies from the wound and inflammatory mass showed a chronic inflammatory granulomatous process associated with extravasated fetal squames and foreign material. A month later, she experienced further pain and dysuria. She underwent extensive investigation for thrombo-embolism, osteomyelitis, tuberculosis and further histological and microbiological investigation of wound biopsies that were normal. A repeat histopathological review of her previous biopsies diagnosed VCP. A urological opinion concluded that paravesicular inflammation was responsible for her urinary symptoms. In all, her pain continued for 7 months post-caesarean section before resolving.A 43-year-old woman had had 12 first trimester miscarriages and terminations of pregnancy before the current pregnancy with an otherwise unremarkable medical and antenatal history. She underwent an uncomplicated emergency LSCS for failure to progress in the first stage of labour. Four days post-delivery, she developed shoulder-tip pain in association with abdominal distension, peritonism and pyrexia. An abdominopelvic CT was unremarkable and as her clinical picture was not improving despite analgesia and antibiotics, a laparoscopic investigation was undertaken. VC extruded through the Hassan port at entry and was also scattered throughout the peritoneal cavity (Figure We have presented three cases of post-caesarean section peritonitis in patients from the same health sector over a 24-month period. The two adjacent maternity units involved have 2600 and 2400 deliveries per year with caesarean section rates of 42% and 33%, respectively. Both rates have increased 2.5% every 5 years over the last 20 years that we have records for and both surpass the national average rate of approximately 30%, leading us to believe that an increasing CS rate, with a consequent increase in caesarean complications, is responsible for this cluster. Certainly, the incidence in our area of late has heightened awareness of this entity among our local obstetric, surgical and histopathological community and with increasing awareness in the general community, we may see a paradoxical rise in reported cases that may have previously been undiagnosed or assumed to be due to other disease processes. Mopping the paracolic gutters of excess debris and blood before closure to reduce postoperative pain has been traditional teaching and is certainly routine practice within our teaching hospital and those surgeons involved in all our cases. That said, the increasing caesarean section rate we are seeing, mirroring that of the nation, may contribute to more surgical laxity in this area and an increased incidence of VCP.The health population in our area comprises a moderate to high socio-economic group and ages of our patients ranged from 32 to 43 years of age where there may be an as yet undiagnosed element of immunological hypersensitivity. If this is the case, then the volume of VC needed to incite a reaction is probably small and mopping of the paracolic gutters may not be helpful. However, two of our patients were primipara suggesting that hypersensitivity reaction is less likely given there has been inadequate time for sensitisation to occur, even from an antenatal priming event. In keeping with hypersensitivity reactions, there are too few cases reported to see whether multipara have a more exaggerated response and this is a potential focus for future research.The principal symptoms of VCP are generalised severe abdominal pain, pyrexia, peritonism and elevated white cell count with inconclusive or normal imaging. Other causes of peritonism are more likely including intraperitoneal sepsis, endometritis and iatrogenic ureteric/bowel injury. We agree that there should be no hesitation in undertaking further emergency laparoscopic or open surgical investigation should the clinical presentation be such that it warrants exclusion of these other more common pathologies. However, the observation of white cheese-like plaques and/or VC within the peritoneal cavity upon entering should raise the suspicion of VCP. Appropriate serosal biopsies are needed to confirm the diagnosis in the absence of other identifiable aetiologies. Our review of the literature found that most reported cases had significant additional procedures of laparotomy that, with hindsight, may have compromised recovery. The subsequent normal histological findings in the excised organs highlight the need for improved awareness among surgeons to reduce the morbidity from additional surgery.Various conservative treatments have been tried although many authors omitted to outline their postoperative management ,4-7,13. With an increasing caesarean section rate, the incidence of post-caesarean complications such as vernix caseosa peritonitis is also rising. Obstetricians should be aware of this condition to avoid unnecessary invasive procedures with the mainstay of management being exclusion of other more common pathologies, obtainment of biopsies to achieve histological diagnosis, and analgesia. The pathological process seems not as innocuous as once was thought and vigilant monitoring after diagnosis is required as delayed morbidities may arise necessitating timely intervention.Written informed consent was obtained from the patients for publication of this case series 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.RB conceived writing this case series after the initial patient presented with VCP in his practice and the second case presented in a colleague's practice. OS performed the literature search, compiled the case histories and results, and wrote the skeleton manuscript. AM and OS were both involved in the care of case three. All authors read, edited and approved the final manuscript.


Villous adenomas of the urinary tract are an uncommon, well-recognized entity, described in different locations. However, the occurrence of this lesion in the female urethral diverticulum is very unusual. We present the first case of a recurrent villous adenoma with high-grade dysplasia unassociated with adenocarcinoma, arising from a urethral diverticulum. A 75-year-old African-American female presented with urethral prolapse complaining of mild voiding difficulty, stress incontinence, and mild spotting of blood. Histological examination revealed a papillary lesion with finger-like processes lined by pseudostratified columnar epithelium with abundant goblet cells. There were focal areas with stratification to the luminal surface and loss of nuclear polarity and atypical mitoses, interpreted as villous adenoma with high-grade dysplasia. The lesionrecurred at one year without evidence of malignant transformation. We also present a brief literature review of urothelial villous adenomas. Villous adenomas of the urinary tract are a well-recognized but uncommon entity. Several case reports and a number of studies detailing the pathologic features and prognosis of villous adenomas in the urethra, bladder, and urachus have been published –4. HowevA 75-year-old African-American female presented with a urethral mass and evidence of urethral prolapse. She had mild voiding complaints including stress incontinence and mild spotting of blood. Physical examination revealed a mass arising in a diverticulum protruding from the urethra. Cystoscopic examination was performed, and a biopsy was obtained. Subsequently, diverticulectomy was performed. The patient tolerated the operation well and was discharged with no untoward events. The lesion recurred a year after and reexcision was performed. Subsequent 6-month follow-up has shown no evidence of residual neoplasm.Gross examination of the biopsy specimens consisted fragments of tan-red tissue, measuring 1.8 × 1.5 cm for excision and 3 × 2 cm for the reexcision specimen.Histologic examination of all specimens revealed a papillary lesion with finger-like processes lined by pseudostratified columnar epithelium with abundant goblet cells. The cells demonstrated nuclear stratification, crowding, hyperchromasia, prominent nucleoli, and abundant mitoses, including atypical forms, interpreted as villous adenoma with high-grade dysplasia Figures . ImmunohPrior to Cheng et al. report of 23 cases of urinary tract villous adenoma from two large tertiary institutions , only isVillous adenomas of the urinary tract and the colon share the same microscopic pathologic features. Histologically, villous adenomas from both sites are characterized by papillary projections of pseudostratified columnar epithelium with mucin-producing goblet cells. Cytologic features include nuclear stratification, loss of cellular polarity, and nuclear hyperchromasia. Coexisting invasive adenocarcinoma can be present , 5. PapiImmunohistochemical features of adenoma and adenocarcinoma are also similar. Urinary tract adenomas exhibit positive staining for cytokeratin 20 (CK 20) , 5 and cThe exact histogenesis of glandular lesions in the urinary tract is uncertain; however, embryological development of the urinary tract may give some insight into its origin. The cloaca is divided by the urorectal septum into a dorsal rectum and a ventral urogenital sinus. The urogenital sinus gives rise to the majority of the urinary bladder, including the bladder trigone and the neck of the bladder. Remnants of cloacal epithelium may explain the origination of villous adenomas in these structures as well as in the urethra , 12. AnoThe differential diagnoses of villous adenoma of urinary tract include cystitis glandularis and adenocarcinoma. Cystitis glandularis has been reported as a potential mimic of bladder adenocarcinoma , 15. HisThe association between villous adenoma and coexistent adenocarcinoma has been established in a number of cases , 5. ThouPrognosis is usually excellent in patients with conventional villous adenoma and those cases demonstrating in situ adenocarcinoma . HoweverIn conclusion, we described the first case of recurrent villous adenoma arising in a urethral diverticulum unassociated with adenocarcinoma. It is important to recognize this uncommon lesion which has the potential of malignant transformation and/or recurrence and confusion with the far more common cystitis glandularis.


Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis. It has become generally accepted that DIPNECH is a precursor lesion to pulmonary carcinoid tumors.Here we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.This case contributes to a better understanding of the disorder and its associated pathologies. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an exceedingly rare disorder and only 40 cases have been described in the literature to date . AccordiHere we report on a patient with DIPNECH who coincidently developed a pulmonary adenocarcinoma. The 60-year-old female patient was initially referred to our hospital because computed tomography scans revealed a tumor-like lesion measuring 2.9 cm in its widest diameter in segment 2  of the right lung. Additionally, several lesions as large as 0.6 cm were evident in segments 4  and 6  of the right lung. These lesions were suggested to represent metastases of the lesion in segment 2. A CT scan of the chest Fig.  was indiDIPNECH is an exceedingly rare disease involving generalized proliferation of pulmonary neuroendocrine cells, which leads to an occlusion of the bronchial lumina and consequent clinical symptoms such as shortness of breath. Due to its neuroendocrine origin, its similar morphology to pulmonary carcinoids and particularly due to its association with pulmonary carcinoids, the disease is considered to be a precursor lesion for theses entities. Only 40 cases of DIPNECH have been reported in the literature to date  and therDIPNECH is a rare disorder and it is considered to be a precursor lesion for pulmonary carcinoid tumors. Information on the disease is still limited, especially with regard to management and prognosis. This case is the first report of a patient with DIPNECH in association with a pulmonary adenocarcinoma. Since an increasing incidence of DIPNECH cases has been noted in the past few years, we report this case to contribute to a better understanding of the disorder and its associated pathologies.DIPNECH = diffuse idiopathic neuroendocrine cell hyperplasia; CK7 = cytokeratin; CK18 = cytokeratin 18; TTF1 = thyroid transcription factor 1; SPA = surfactant protein A; CEA = carcinoembryonic antigen; NSE = neuron specific enolase.The author(s) declare that they have no competing interests.AW wrote the manuscript. EH diagnosed the specimens and collected data. AS performed and diagnosed the CT scans. KS performed the operation, pre- and post-operative patient management. PAS diagnosed the specimens and made final corrections of the manuscript. All authors read and approved the final manuscript.Written consent was obtained from the patient for the publication of the report.


Primary mediastinal synovial sarcoma is a rare malignancy with only a few cases reported so far. A 56-year-old woman was admitted to our hospital for an investigation of a nodule in the left middle lung on chest radiography. Computed tomography revealed a mediastinal mass first described as a solitary fibrous tumor. The diagnosis of synovial sarcoma was established by computed tomography-guided percutaneous needle biopsy. Work up showed no metastasis to distant organs or contralateral pleural cavity. The mass was surgically resected; pathological and immunohistochemical analyses confirmed the diagnosis of a monophasic spindle cell synovial sarcoma probably originating from phrenic nerve. The patient received adjuvant chemotherapy and radiation and is free of recurrence after a follow up of 16 months. Soft tissue sarcomas (STS) are a heterogeneous group of neoplasms. They account for less than 1% of all adult malignancies , is unreA 56-year-old Caucasian woman was referred to our hospital after a chest X-ray showed a dense mass in the left middle lung . The patBronchoscopic examination revealed a constriction of the left upper lobe but no endobronchial tumor. CT-guided percutaneous needle biopsy was performed and histological H&E examination at the Institute of Pathology Medical University Innsbruck showed a monomorphic cell rich spindle cell proliferation with mild nuclear atypia. The characteristic H&E morphology of the core biopsy together with homogenous vimentin positivity, bcl-2 positivity and focal keratin  as well as EMA positivity together with the absence of S-100 Protein , calretinin  and CD34 (rules out solitary fibrous tumor) led to the diagnosis of a monophasic synovial sarcoma.Discussion at the multi-disciplinary bone and soft tissue tumor board (MUI) as well as in the lung and mediastinal tumor board (MUI) decided to do primary surgery as there was no spread of disease, no metastases on X-ray and CT. The tumor was surgically resected by thoracotomy . The medSynovial sarcoma originates in the deep soft tissue and commonly presents as an asymptomatic slow growing mass. In almost 60% of cases the neoplasm is located in an extremity; less than 20% occur in the trunk .In a study by Spillane et al. the overall 5-year survival rate for STS was 57%. They also demonstrated that an age greater than 20 years at diagnosis and the trend in size (≥ 5 cm) were associated with a significantly worse prognosis. Their survey assessed one hundred and fifty patients . In our Only a few other cases of synovial sarcoma of the mediastinum have been reported -13. All Immunohistochemically the tumor cells were positive for CAM 5.2, cytokeratin 7 (CK), bcl-2 and vimentin and negative for S100, CD34, CD99, desmin and SMA. With this findings  it was possible to exclude two entities that can most closely resemble monophasic synovial sarcoma in this location: Solitary fibrous tumor (CD34 positive) and malignant peripheral nerve sheath tumor  .CT scan of the tumor may demonstrate a heterogenous soft-tissue mass that occasionally contains calcium with attenuation slightly higher than that of muscle . InvasioSurgical resection with adequate safety margins represents the therapy of choice potentially being curative for STSs. Because of the atypical location and advanced size of > 5.0 cm it was decided to treat our patient also with adjuvant Ifosfamid/Adriamycin (doxorubicin) chemotherapy . This is


Basal cell adenocarcinoma of a minor salivary gland is extremely rare. The goal of this report is to increase awareness of this rare disease and to review and discuss the differential diagnosis and important considerations in treatment.Case report and review of the literature.Case report of a basal cell adenocarcinoma of a hard palate minor salivary gland and review of the literature of basal cell adenocarcinoma.Basal cell adenocarcinomas are slow-growing tumours that most commonly involve the parotid gland and very rarely involve minor salivary glands. Although recurrence rates for these tumours are high, mortality rates are low. Histological diagnosis is important to distinguish this tumour from adenoid cystic carcinoma given the significant difference in disease prognosis.Diagnosis of these tumours must be made histologically. Recommended treatment options include wide local excision with radiotherapy reserved for close surgical margins or for local recurrence. Minor salivary gland neoplasms constitute up to approximately 25% of all salivary gland tumours ,2. The iA 69 year-old woman presented with a one-year history of a persistent ulcer on her hard palate and a poorly fitting upper denture plate. During this period, the patient had no weight loss, symptoms of nasal obstruction, nasal discharge, or dysesthesias. A midline 2 × 2.5 cm mass was present on her central hard palate with raised surfaces, an area of central ulceration, and soft palate extension. No lymphadenopathy was detected. An axial computed tomography (CT) scan image revealed a 2-cm heterogeneously enhancing mass at the nasal septum extending into the hard palate Figure  and biopThe patient was taken to the operating room for a combined transnasal endoscopic septectomy and a transoral maxillectomy. Septal incisions were made around the mass and carried down to the maxillary crest. The mouth was opened, and a McIvor mouth gag was placed to maintain maximal exposure. Wide local excision of the mass included a portion of the anterior soft palate and the oral incision was connected to the septal incision to permit en bloc resection. On gross pathologic examination, the tumour had a cystic hemorrhagic granular tan brown appearance and bone invasion Figure . Low powBCAC typically arise in adults older than 60 years and have no gender predominance . PatientBCAC is believed to arise from pluripotent ductal reserve cells. Grossly, these tumours appear solid grey-tan and grow as large as 7 cm in diameter . BCAC haThe major pathologic differential diagnostic considerations for BCAC are basal cell adenoma and adenoid cystic carcinoma. As its malignant counterpart, BCAC shares many histologic characteristics with basal cell adenoma but is distinguished from basal cell adenomas often only by invasion of local structures or by perineural or angiolymphatic invasion . DistingBCAC of a minor salivary gland is an extremely rare tumour with 22 reported cases in the literature. In recent series' of minor salivary gland tumours, BCAC account for <1% of all minor salivary gland tumours -16. OverBasal cell adenocarcinoma (BCAC) is a rare salivary gland tumour that carries a favourable prognosis. Although there are few reported cases of BCAC of minor salivary gland origin, BCAC should remain a diagnostic consideration. Since these tumours typically require only local excision, they must be distinguished histologically from adenoid cystic carcinoma, which has a poorer prognosis and often necessitates a more aggressive clinical approach.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.BKW performed the literature research, participated in the clinical care of the patient and drafted the manuscript. RRS and ELB participated in drafting the manuscript and in the patient's diagnosis. SYL performed the patient's surgery and participated in drafting the manuscript. All authors read and approved the final manuscript.


Creutzfeldt-Jacob Disease is the most frequently seen type of prion diseases. Its clinical findings consist of predominantly progressive dementia with a rapid onset, myoclonus, and also cerebellar, pyramidal, extrapyramidal and visual signs. Definitive diagnosis is established with histological examination of brain biopsy or autopsy materials. Occurrence of periodical spikes in EEG, observation of cortical signal alterations during diffusion weighted (DW) MRI studies, and detection of protein 14-3-3 in cerebrospinal fluid (CSF) substantiate the diagnosis.Seventy year-old male patient referred with complaints of weakness and involuntary movements in left arm, changes in behavior, and forgetfulness. He also developed akinetic mutism after nearly three months. In EEG periodic triphasic waves were seen. Despite the absence of any apparent pathological finding in T2 and FLAIR MRI, excluding signs of atrophy, on DW MRI hyperintense signal changes in cortical regions  were observed. Protein 14-3-3 in CSF was detected.Patients who have progressive dementia and associated atypical features should be investigated especially with DW MRI. Cortical ribboning is a very useful diagnostic sign for CJD. Creutzfeldt-Jacob Disease (CJD) is a rarely seen neurodegenerative disease among the infectious spongioform encephalopathies. It has four subtypes as sporadic, familial, iatrogenic and variant forms thought to be transmitted with ingestion of infected meat products . Mean agIn this article a case with a probable sporadic CJD, in which diagnosis was established based on medical history, clinical presentation, findings of diffusion weighted (DW) MRI, EEG and CSF in accordance with clinical diagnostic criteria of World Health Organization (WHO) is presented.rd day of his hospitalization akinetic mutism developed. EEG showed 4–5 cps teta waves in background activity and also slow triphasic waves with higher amplitude on frontal regions were detected. In differential diagnosis Hashimoto encephalitis was contemplated secondary to lower levels of TSH. Laboratory findings of the patient were interpreted as subclinical hyperthyrodism. Marked cerebral atrophy in frontoparietal regions and several ischemic-gliotic foci were seen on cranial MRI, T2 weighted and FLAIR imaging, while hyperintense areas  all over the cortex was noted in DW MRI  and a prolonged course (22–24 months). Typical EEG findings are not seen, and genetic studies revealed nearly 20 genes mutations. Iatrogenic form can be occur secondary to duramater graft implantation, corneal transplantation, surgical operations performed with contaminated instruments, and hormonal therapies using hypophyseal growth hormones ,5.Symptoms of sporadic CJD can appear at 50–70 years of age. It constitutes 90% of all cases with CJD and it is characterized with rapidly progressive dementia and myoclonus. Personality changes accompany cerebellar and visual symptoms. Ataxia is more marked in advanced cases and most patients have myoclonus manifesting as a response to auditory and tactile stimuli. In late stages patient develops akinetic mutism and myoclonus can disappear. Eighty percent of the patients die from infection, cardiac and respiratory failure within the first year . CSF proIn sporadic CJD cerebral atrophy, increase in signal intensity in putamen, caudate nucleus and cerebral cortex can be detected in imaging studies. Increased signal intensity in the cortex is called ribboning. Shiga et al. revealed 92.3% sensitivity and 93% specificity for DW MRI in their patients with definitive (n = 9) and probable n = 36) diagnoses of CJD [ diagnoseAccording to diagnostic criteria of World Health Organization (WHO) for the probable diagnosis of CDJ, the presence of at least one criterion among typical EEG findings and 14-3-3 positivity for CSF samples or at least 2 criteria among myoclonus, visual disturbances, cerebellar, pyramidal or extrapyramidal findings and akinetic mutism together with progressive dementia are required. . Among tWritten 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.EG analyzed and interpreted the patient data. NE and MU performed examination and clinical observation of the patient, and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


Pneumatosis cystoides intestinalis is a rare disease characterized by presence of multilocular cysts in the gastrointestinal wall. Rarely, patients may experience symptoms secondary to the cysts. The pathogenesis of pneumatosis cystoides intestinalis is still unclear and many theories have been advocated to explain the exact origin. Complications occur in about 3% of cases and include obstruction, intussusception, volvulus, haemorrhage and intestinal perforation.The author reported a male patient aged 56 years presented to the emergency department with acute upper abdominal pain. Widespread variable sized serosal intestinal air cysts were seen at the first look involving long segment of jejunum and ileum. Perforated duodenal ulcer, as the cause of generalized peritonitis, was repaired with direct closure and omental patch. A second laparotomy, was done and exploration was systematically performed and denoted hugely distended stomach with cicatrisation at the site of previous closure of perforated duodenal ulcer and the whole length of small gut was completely free from the already described pneumatosis cystoides intestinalis.The pneumatosis cystoides intestinalis is a rare disease and suspicion of this disease process should be based on imaging and clinical finding. The therapy can be conservative or surgical in restricted situations. Pneumatosis cystoides intestinalis is a rare disease characterized by presence of multilocular cysts in the gastrointestinal wall. Idiopathic and secondary forms of the disease can be distinguished . In the Primary pneumatosis is often asymptomatic. Rarely, patients may experience symptoms secondary to the cysts. Signs and symptoms include diarrhea, bloody stools, abdominal pain, abdominal distention, and constipation. The physical findings are usually unremarkable .Pneumatosis cystoides intestinalis may be associated with bowel ischemia, perforation, and a high mortality rate. As a result, many authorities advocate an aggressive surgical approach in those patients .The pathogenesis of pneumatosis cystoides intestinalis is still unclear and many theories have been advocated to explain the exact origin -7]. The. The7]. Second, there is the bacterial theory. In animal experiments, introduction of bacteria into the bowel wall by injection has been shown to cause PCI . The pulComplications occur in about 3% of cases and include obstruction, intussusception, volvulus, haemorrhage and intestinal perforation ,6.A fit and otherwise healthy male patient aged 56 years from the Asian part of Egypt, presented to the emergency department with acute upper abdominal pain with three hours duration. The patient was in agony with anxious look. Due to religious background, he did not drink alcoholic beverages. His body temperature was 37.7°C and pulse rate was 88 beats per minute. Abdominal examination revealed tenderness over the epigasrtic region and the right side of the abdomen with rigidity maximally detected all over the right abdomen.Plain x-ray films detected free gas under the diaphragm and abdomino-pelvic ultrasonography demonstrated free fluid in the peritoneal cavity.After the routine work up and proper resuscitation, laparotomy was performed. Widespread variable sized serosal intestinal air cysts were seen at the first look involving long segment of jejunum and ileum. All cysts were intact and were not incriminated as a cause of pneumoperitoneun or peritonitis Figure . PerforaThe patient passed very smooth postoperative course and was followed up for the next eight months until the complaint of repeated vomiting was evident and food particles of eaten diets since more than two days were recognized in the vomitus.Upper gastro-duodenal endoscope and barium meal study were performed and revealed gastric outlet obstruction. Computed axial tomography also confirmed the diagnosis and denied any other pathology.Laparotomy, through the previous midline incision, was done and exploration was systematically performed and denoted hugely distended stomach with cicatrisation at the site of previous closure of perforated duodenal ulcer and the whole length of small gut was completely free from the already described pneumatosis cystoides intestinalis Figure . Gastro-Pneumatosis cystoides intestinalis is a rare disease and the exact cause is probably a combination of associated diseases causing elevated pressure and mucosal damage allowing gas-forming microorganisms to enter the bowel wall, thus forming the cysts .The author in this case report put the question whether pneumatosis cystoides intestinalis is the direct cause of perforated duodenal ulcer or the result of this disease process as advocated in other pervious reports ,11.Previous data stated that the cysts may be located in the subserosa, submucosa, and, rarely, the muscularis layer ,4. They Usually, no treatment is necessary for 85% of patients who are asymptomatic. The resolution of gas collections has been reported after inhalation of 70% of oxygen and after hyperbaric oxygen therapy ,9,13 GooIn the post-operative period, the patient of the present case was given metronidazole 500 mg / 12 hour as intravenous infusion together with cefotaxim 1 gm/ 8 hour. We noticed that the cysts showed complete resolution during the second operation with no adhesion formation or amalgamation between the intestinal loops. This finding came in agreement with those data reporting that therapy with metronidazole up to 1500 mg daily was effective to cause resolution of pneumatosis cystoides intestinalis .The pneumatosis cystoides intestinalis is a rare disease and suspicion of this disease process should be based on imaging and clinical finding. The therapy can be conservative or surgical in restricted situations.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 author declares that he has no competing interests.All cysts were intact.Click here for file


Neonatal hemochromatosis is the most common cause of liver failure and liver transplantation in the newborn. The size of the infant determines the liver volume that can be transplanted safely without incurring complications arising from a large graft. Transplantation of monosegments II or III is a standard method for the newborns with liver failure.A three-week old African-American male neonate was diagnosed with acute liver failure secondary to neonatal hemochromatosis. Living-related liver transplantation was considered after the failure of intensive medical therapy. Intra-operatively a non-anatomical resection and transplantation of segments II and III was performed successfully. The boy is growing normally two years after the transplantation.Non-anatomical resection and transplantation of liver segments II and III is preferred to the transplantation of anatomically resected monosegements, especially when the left lobe is thin and flat. It allows the use of a reduced-size donor liver with intact hilar structures and outflow veins. In an emergency, living-related liver transplantation should be offered to infants with liver failure secondary to neonatal hemochromatosis who fail to respond to medical treatment. Neonatal hemochromatosis (NH), although rare, is the most common cause of liver failure and liver transplantation in neonates. Liver transplantation is the main therapy for infants who fail to respond to medical treatment . Liver tA three-week old African-American male newborn, weighing 2.5 kg, was admitted to our unit with jaundice, abdominal distension and hepatomegaly. The pregnancy had been uncomplicated and there was no family history of metabolic or liver disease.Laboratory studies for liver failure revealed a total serum bilirubin of 22.5 mg/dL, an international normalized ratio 4.9, aspartate aminotransferase 45 U/L and alanine aminotransferase 23 U/L. The boy had a serum iron of 157 μg/dL , serum ferritin 994 ng/mL , serum transferrin 103 mg/dL  and transferrin saturation 109% (range 16%-60%). He had an elevated alpha-fetoprotein level (3289 ng/mL). Investigations for infectious and inherited metabolic pathologies were negative. Magnetic resonance imaging (MRI) of the abdomen was suggestive of iron deposition in the liver, with conventional, patent arterial and venous anatomy. NH was suspected and confirmed by minor salivary gland biopsy from the lower lip.3. We therefore decided to do a left lateral resection with back-table monosegmentectomy followed by transplantation.Medical therapy consisting of anti-oxidants and chelation with desferroxamine was initiated. As there was a progressive worsening of the boy's condition on medical therapy, his mother volunteered to be a living liver donor. After a standard expedited two-day donor-workup, the related living donor liver transplantation was planned. Pre-operative MRI showed that the mother's left lateral segment volume was approximately 200 ccIntra-operatively, after isolation of the mother's left hepatic artery, hepatic duct and portal branch, the hepatic parenchyma of segment IV was transected 5 mm to the right of the falciform ligament without blood inflow occlusion or graft manipulation. The segment II and III ducts united, just lateral to the umbilical portion of the left portal vein and the segment IV duct, then joined medial to umbilical portion. This confluence of segment II and III ducts was divided and used for anastomosis in the recipient. This was a thin 'pancake' left lateral segment that was transected using ultrasound guidance. On the back-table, a 2.5 mm endostapler with two, triple-staggered rows of titanium staples  was used to staple and divide across the mid-portion of the left lateral segment just to the left of the secondary portal vasculature branching Figure . The finNeonatal hemochromatosis is a syndrome with an aggressive course and a poor prognosis. The etiopathogenesis is not very clear although siderosis resulting from infections, genetics and auto-immunity may play a role . The prein situ in the donor or on the back-table. Despite these surgical innovations, neonatal liver transplantation still poses challenges because of the size of the recipients who usually weigh less than 10 kg [Liver transplantation using either monosegment II or III is a useful option for small infants in whom a whole left lateral segment is large-for-size . Monosegan 10 kg . DependiUrgent living-related liver transplantation should be offered to infants with acute liver failure secondary to neonatal hemochromatosis who are non-responsive to medical therapy. The left lateral segment can be reduced in size, especially when it is flat (like a pancake), by splitting it along a non-anatomical plane. This simple technique allows the use of the confluence of donor segment II and III bile ducts that are less prone to stricturing due to their larger caliber. However, this advantage may be lost in cases where the segment II and III bile ducts join separately, medial to the umbilical portion of the portal vein.IUGR: intrauterine growth restriction; MRI: magnetic resonance imaging; NH: neonatal hemochromatosis.The authors declare that they have no competing interests.AS collected data, designed and wrote the manuscript. AHC was a major contributor to the manuscript. DGM assisted in the critical revisions of the manuscript. MPP reanalyzed the surgical facts and provided comments on the critical intellectual content of the manuscript. RAF helped to conceive, critically revise and write the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patient's mother for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We report a case of intra-articular angiolipoma of the knee. This case report describes our experience in excising an intra-articular angiolipoma of the knee joint. Complete resection under arthroscopy was performed in a 30-year-old man. Two years after the surgery, no evidence of recurrence was seen. Intra-articular angiolipomas should be considered in the differential diagnosis of intra-articular masses in adolescents with recurrent hemarthrosis without trauma. Angiolipomas are uncommon benign soft-tissue tumors that typically arise in the subcutaneous tissues of young patients . The forIntra-articular angiolipoma of the knee is a very rare cause of knee pain, hemarthrosis and dysfunction. To our knowledge, there has been no reported case of intra-articular angiolipoma of the knee in English. We report on this exceedingly rare case and review the previous angiolipoma reports.A 30-year-old man presented to our clinic complaining of a recurrent swollen and painful knee over a period of 6 months without significant trauma. Examination revealed swelling and ballottement of the patella in the right knee joint, and aspiration of bloody joint fluid suggested a hemorrhage. The range of motion of the knee was limited to between 0 and 130 degrees with terminal flexion pain. There was no point of tenderness and instability of the knee. A plain x-ray showed no atrophic or degenerative change of the knee. Magnetic resonance imaging (MRI) showed a 2.5 cm polylobulated tumor localized in the dorsal area of the posterior cruciate ligament (PCL). MRI of the tumor exhibited a slightly high signal intensity, in contrast to muscle on T1-weighted images, a fluctuating high and low signal intensity with low signal septa around the tumor on T2-weighted images and an inhomogeneous signal, enhanced moderately on T1-weighted images with gadolinium (Gd)-enhancement , idiopathic hemorrhage and hemophilic arthropathy. The differential diagnosis, of synovial hemangioma or localized PVS for intra-articular bleeding could be reached after detecting an intra-articular tumor-like mass on MRI.Regarding the differential diagnosis of these entities on MRI, the characteristics of synovial hemangioma on MRI are that iso signal intensity contrasted to muscle on T1-weighted images, a strong high signal intensity with low signal intensity septa on T2-weighted images and inhomogeneous enhancement of internal lesions on T1-weighted images with Gd-enhancement . In contIn this case, the tumor exhibited slightly high signal intensity in contrast to muscle with low intensity area on T1-weighted images, a fluctuating high and low signal intensity with low signal septa around the tumor on T2-weighted images and inhomogeneous enhancement on T1-weighted images with Gd-enhancement. From differences of these characteristics on MRI, we might be able to distinguish this case from the other tumors. However, MRI is useful, albeit imperfect, in distinguishing these tumors with accuracy. Therefore, diagnosis could be made arthroscopically and accurately with histopathological evaluation.Surgical excision is curative, and there is no evidence of malignant transformation . We depiIn conclusion, an intra-articular angiolipoma of the knee can be the cause of knee pain and recurrent hemarthrosis. Diagnosis can be made arthroscopically, but histopathological evaluation is more accurate. Excision should be performed either arthroscopically if the tumor is completely visible, or using arthrotomy.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.MO, MD, NA and MN carried out the surgical treatment and MD, AK and MN contributed to the follow-up examinations in an outpatient clinic. MD, MN and AN co-wrote the paper, discussed the results and commented on the manuscript. All authors have read and approved the final manuscript.