Extraneural metastases are relatively rare manifestations of medulloblastoma.MYCN-amplified medulloblastoma. He received multimodal chemotherapy consisting of gross total resection followed by postoperative craniospinal radiation and adjuvant chemotherapy. The patient developed extraneural metastases 4 months after the end of therapy. Literature review identifies the poor prognosis of MYCN-amplified medulloblastomas as well as extraneural metastases; we review the current limitations and future directions of medulloblastoma treatment options.We present the case of a young boy with group three To the best of our knowledge, this is the first molecularly characterized report of extraneural metastases of medulloblastoma in a child. Historically, medulloblastoma is the most frequent brain tumor in children accounting for more than 20% of pediatric brain tumor cases. Medulloblastoma can metastasize outside the nervous system and by direct extension via leptomeningeal seeding. The most common sites of metastasis are bone and bone marrow, followed by lymph nodes and, to a lesser extent, liver, lung, and peritoneum. Although only 1–2% of medulloblastoma patients present with extraneural metastasis at the time of initial diagnosis, the cumulative incidence during follow-up reaches values of up to 5–10%. To date, 282 patients with extraneural metastases have been reported. Of these reported cases, 40.4% have occurred in children [A previously healthy 7-year-old boy was hospitalized in March 2015 with a 1-week history of progressively worsening symptoms of nausea, vomiting and decreased levels of consciousness. Upon admission, his examination was significant for diffuse hypotonia  and papilledema. A head CT scan revealed a posterior fossa tumor and obstructive hydrocephalus [Fig. The case was discussed at our institution’s multidisciplinary Neuro-Oncology tumor board. Plans were made to start multimodal therapy, as per the KFMC high-risk medulloblastoma protocol. This protocol consists of 6 weeks of craniospinal axis radiotherapy CSI 36 Gy and a boost to the posterior fossa to a dose of 55.8 Gy/ 31F concurrently with 2 cycles of oral Etoposide, beginning day 1 of radiation, oral Etoposide daily for 3 weeks every 4 weeks .Repeat MRI was performed post radiation therapy and showed post-operative changes and interval improvement of leptomeningeal enhancement over the spinal cord. Adjuvant chemotherapy based on The COG ACNS0332 was started on week 11 and consisted of 3 cycles of Cisplatin and oral Etoposide (cycle A), and 3 cycles of cyclophosphamide and Vincristine (cycle B) for a total of 6 alternating cycles of A and then B, followed by 6 cycles of maintenance with oral Isotretinoin.Our patient tolerated chemotherapy very well apart from one admission for febrile neutropenia following cycle B chemotherapy; he finished his chemotherapy in February 2016. Following treatment, the patient was in good condition, without symptoms suggestive of persisting disease or recurrence. End of therapy follow-up brain and spine magnetic resonance imaging (MRI) revealed no signs of recurrence.However, 2 months after the completion of therapy, the patient began to complain of a hard and non-tender right mandibular swelling that gradually increased in size.Brain and spine MRI was performed and showed no signs of tumor recurrence in the surgical bed of the posterior fossa and no evidence of CSF seeding metastases along the spine. However, MRI of the mandibular area revealed a large necrotic and heterogeneous right mandibular soft tissue mass with extension into the infratemporal fossa Fig. .Fig. 3MRThe patient was re hospitalized in June 2016 for mandibular mass incisional biopsy. Histological examination of the biopsy revealed small, round, blue tumor cells, consistent with metastasis of medulloblastoma. Immunohistochemistry was performed using commercially available antibodies according to standard protocols for paraffin-embedded formalin-fixed tissue. The slides were incubated with antibodies against GFAP, INI1, synaptophysin and P53. Histopathology confirmed the diagnosis of medulloblastoma with classic morphology. No anaplasia or excessive nodularity noted in both samples. Immunohistochemistry for the primary and metastatic tumors were positive diffusely for synaptophysin and scattered positive glial staining for GFAP. Staining for P53 was negative. Intact nuclear staining for INI1, which confirm the diagnosis of classic medulloblastoma and rule out atypical teratoid rhabdoid tumor diagnosis.Oncopanel assayWe used two methods in our molecular testing for the primary specimen to better classify the genomic subgrouping because the next generation sequencing method alone classified the tumor as either group 3 or 4. The metastatic specimen didn’t have enough materials for testing.Oncopanel) of 300 cancer-related genes was performed using Illumina-based methods as previously described [Using a QIAamp DNA FFPE Tissue Kit , we obtained DNA from approximately 5–10 × 0.6 mm punches containing at least 80% tumor  from a formalin-fixed paraffin-embedded (FFPE) tissue block. The concentration of double-stranded DNA was quantified using a Quant-iT™ PicoGreen® dsDNA Assay Kit . Next-generation targeted exon sequencing  reviewed the tumour specimen and estimated the number of neoplastic cells in the submitted sample (> 80% viable tumour cells). Oncopanel assay showed 9,564,899 unique, aligned, high-quality reads of this specimen, with a mean of 182 reads across all targeted exons and 98% of all exons having more than 30 reads. No notable somatic mutations were detected. Copy number analysis showed MYCN high amplification red arrow ; monosomies in chromosomes 3, 8, 10, 11, 16 and 21; and polysomy in chromosome 18. Breakmer analysis identified rearrangements involving (1) MYCN and a null area  and (2) MYCN and SMC6 . This molecular pattern suggests either group 3 or 4 medulloblastoma.2.Methylation assayMethylation data generated by the Illumina Infinium HumanMethylation 850 BeadChip array at the molecular pathology department in NYU. We used the previously described by the German Cancer Research Center (DKFZ), in-depth analysis of the DNA methylation , 5.MYCN amplification  reveled methylation class medulloblastoma, subclass group 3 with calibrated match score to the database of 0.99. The methylation class “medulloblastoma, subclass group 3” is comprised of tumors with the diagnosis medulloblastoma, genetically defined, group 3. Histologically most cases fall into the classical (like our case) and large cell/anaplastic groups. Copy number variation showed also The case was discussed in multidisciplinary Neuro-Oncology tumor board. Given his diagnosis of extraneural metastases, no further medical interventions were offered. Initially, his general condition was stable, and he continued palliative care at his home. After a couple of months, he was admitted again due to a rapid increase in the size of the mandibular mass. The mass showed extension into the oral cavity, with erosion and ulceration of the mucosa. The patient developed feeding difficulties, recurrent massive epistaxis and oral bleeding, he required frequent blood product transfusion, including recombinant factor VIIa, to control bleeding. Unfortunately, with further deterioration of his condition, he died 18 months after the initial diagnosis.Historically despite the heterogeneity of medulloblastoma, all patients receive the same therapy. The current standard of care for children with medulloblastoma involves maximal surgical resection and adjuvant therapy including both radiotherapy and chemotherapy. Approximately 80% of children with medulloblastoma are cured, but current treatment strategies leave medulloblastoma survivors with significant long-term morbidity and debilitating side effects. Frequently, cure of high-risk patients is difficult to achieve.Medulloblastoma represent approximately 20% of all pediatric brain tumors and can be subdivided into 4 distinct molecular subgroups. These four distinct subgroups of medulloblastoma differ in demographics, somatic genetic events, & clinical outcomes. Current WHO 2016 classify four distinct subgroups of medulloblastoma with distinct demographics, genetics and clinical outcomes. These groups have been designated as Wnt-positive medulloblastoma, SHH-positive medulloblastoma, group 3 and group 4 [MYC amplification, aneuploidy, isochromosome 17q and GFI1/1B activation by enhancer hijacking are recurrent features, but a fraction lack an obvious driving genetic change [Group 4 tumors comprise over 30% of all medulloblastoma and occur mainly in older children, though they can occur in adults and similar to group 3, more frequently occur in males. Group 4 medulloblastoma have a similar prognosis to SHH-positive tumors most groups 4 medulloblastoma have a classic histology. Group 3. Histologically most cases fall into the classical and large cell/anaplastic groups. Tumors are located in the cerebellum, typically in the vermis. Median age is 4 years (range 1 to 17). Group 3 medulloblastomas are more common in males than females. c change .Medulloblastoma metastases usually occur along the route of the cerebrospinal flow . The extraneural metastases of primary brain tumors are relatively rare, accounting for 5–10% of all medulloblastoma cases.Mechanisms of extraneural spread of medulloblastoma are still unclear. Craniotomy and surgical interventions mechanically disrupt the blood-brain barrier and allow migration of the tumor cells. Both hematogenic and lymphatic  spread of primary brain tumor cells have been suggested , 8. AnotThe majority of extraneural metastases of Medulloblastoma occur relatively early after initial diagnosis. While leptomeningeal and posterior fossa metastases are diagnosed in the first 5 years in 80–85% of the pediatric cases, nearly 80% of the extraneural metastases are discovered within the first 3 years after initial diagnosis , 8.Concurrent central nervous system involvement, pulmonary or liver metastasis, early development of extraneural metastasis , and patient age of < 16 years at the time of extraneural metastasis diagnosis are all associated with poorer prognosis [SHH-positive tumors recur mostly in the local tumor bed, while group 3 and group 4 tumors recur mostly with extraneural metastases and do not relapse in the primary site, Group 4 tumors also have an increased time to death following recurrence.Recurrence patterns across medulloblastoma subgroups have been described, and it was found that medulloblastoma does not typically change subgroup at the time of recurrence, further highlighting the stability of the four-principle medulloblastoma subgroups , 8, 9. AThese findings suggest that microscopic leptomeningeal metastases in group 3 and group 4 tumors not visible by neuroimaging or CSF examination are likely to be resistant to current therapy. Thus, additional local therapies targeting the primary site in the posterior fossa are unlikely to increase the cure rate for patients with group 3 or group 4 medulloblastoma, further reinforcing the importance of generating novel approaches to therapy in the metastatic compartment , 9.The identification of molecular subgroups of medulloblastoma has potential to aid in understanding when it is possible to reduce standard chemotherapy doses and/or radiation therapies for children with favorable molecular medulloblastoma subgroups. Also intensify the therapy and /or use of maintenance-targeted therapies for high-risk medulloblastoma .Recent study used highly integrative genomic analysis of medulloblastoma from 579 medulloblastoma samples, which led to identification of new subgroup-specific driver genes, epigenetic subtypes, and candidate targets for therapy .The epigenetic defining group 3 and Group 4 MB subgroups are not as clearly demarcated as their WNT and SHH counterparts. This molecular heterogeneity could further help in predicting different clinical behavior in groups 3 and 4 and design better-targeted therapy. However, more clinical validation to this study is needed [C-MYC (MYC), oncogene as well as its paralogs MYCN and MYCL1, has been shown to play essential roles in cycling progenitor cells born from proliferating zones during embryonic development. After birth, MYC plays important roles in the proliferation of all cell types. MYC, MYCN, and MYCL1 amplifications have all been described in medulloblastomas and associated with poor prognosis [rognosis –13.MYC/MYCN amplification who will receive high dose craniospinal radiation with boost to the primary tumor and standard chemotherapy  for 4 cycles intermixed with an additional 3 cycles of chemotherapy with pemetrexed and gemcitabine .Ongoing St. Jude MB protocol study (SJMB12) intensify therapy for stratum N3 High Risk patients with BRD4), a bromodomain. The preclinical evaluation of BET bromodomain inhibitors in MYCN-amplified medulloblastoma is also ongoing [Novel agents are being tested as potential therapeutics for medulloblastoma. These agents include drugs that suppress expression of MYC isoforms by inhibiting bromodomain containing 4 ( ongoing .In summary, routine molecular profiling of medulloblastoma may give oncologists a more thorough understanding of tumors of unfavorable molecular subgroups that are at increased risk of extraneural metastases. This approach may help modify therapeutic approaches for children with unfavorable medulloblastoma molecular subgroups and have real implications for precision medicine in our oncology practice.


Hepatic portal venous gas with pneumatosis intestinalis are radiological clues of intestinal ischemia. Prompt evaluation and a high index of clinical suspicion for the diagnosis of acute mesenteric ischemia are necessary given the high mortality of this condition. We present a case of mesenteric ischemia, radiological clues to diagnosis, and associated mechanisms. A 77 year old male with the past medical history of diabetes mellitus type 2 complicated by a right BKA, hypertension, hypercholesterolemia, and chronic kidney disease presents to the ED for shortness of breath and abdominal pain. Three days prior to admission he noted a sudden onset of crampy and aching pain across his lower abdomen, constant in nature, relieved somewhat with over-the-counter Maalox and Pepcid, but never completely subsiding. The same evening the pain started, he experienced nausea, but no vomiting. He had no history of biliary colic, or abdominal angina. The patient also noted increasing dyspnea, and was found sitting on the edge of his bed trying to catch his breath. He denied any recent fever or chills, hematochezia, or melena. In the ED, he was afebrile with a BP 101/50 mmHg, pulse of 93/min, and oxygen saturation of 92% on 2 liters nasal cannula, and respirations of 24/min. His cardiac exam was regular. His lungs revealed bibasilar crackles. His abdomen exam revealed an obese abdomen with some pain to deep palpation in the lower quadrants, but overall fairly benign. Bowel sounds were normoactive, and there was no hepatosplenomegaly. Rectal exam was guaic negative without any masses.Laboratory data was significant for a creatinine 3.6 mg/dl, with normal liver function tests and coagulation tests. A V/Q scan was read as high probability for pulmonary embolism, later revised to intermediate probability. He was admitted to the hospital for treatment with IV unfractionated heparin, with the diagnosis of pulmonary embolism. His abdominal pain was never clarified on admission, but was still concerning and a CT scan of the abdomen without contrast because of renal insufficiency was ordered in the morning.Hepatic portal venous gas (HPVG), as well as pneumatosis intestinalis (PI), are radiological clues and not diagnoses. Two groups should be distinguished at the time of diagnosis, those who have HPVG with associated PI, and those who have HPVG without associated PI. When associated with PI, the cause is usually an intestinal ischemia event, and in a symptomatic patient it justifies performing systematic exploratory surgery . In a reGas in the portal venous system is carried by the centrifugal flow of blood in the periphery of the liver, appearing to extend within 2 cm of the hepatic capsule, while gas in the biliary tract moves within the centripetal flow of bile, thus appearing more centrally in the liver. Therefore, a history of biliodigestive anastomosis, endoscopic papillotomy, biliary endoprosthesis, or choledocointestinal fistula are important in the differential diagnosis .Speculative mechanisms proposed for this finding include bowel distension, presence of mucosal defects, and sepsis. HPVG can be visualized by conventional radiography, ultrasound, and CT. Radiographic criteria for diagnosis include a branching radio-lucency extending to 2 cm within the liver capsule on abdominal x-ray or CT . X-rays In the absence of a clinical indication for emergency laparotomy, mesenteric angiography remains the investigation of choice in suspected acute mesenteric ischemia. Early angiography has been shown to improve survival rates. Mesenteric angiography can usually differentiate embolic from thrombotic arterial occlusions. Emboli usually lodge where the artery tapers, which is just after the first major branch of the SMA-the middle colic artery. In contrast, thrombotic disease usually involves the origin of the SMA .Acute mesenteric ischemia can be categorized into 4 specific types: arterial embolic, acute mesenteric thrombosis, non-occlusive mesenteric ischemia, and mesenteric venous thrombosis. Arterial emboli are the predominant cause of acute mesenteric ischemia accounting for approximately 40% to 50% of cases. The majority of emboli originate from a cardiac source. Acute mesenteric thrombosis accounts for 25% to 30% of all events. Mesenteric ischemia due to arterial thrombosis occurs in the setting of severe atherosclerosis, with the most common site near the origin of the SMA. Patients with SMA embolism or thrombosis typically have an acute onset of symptoms and rapid deterioration. With an embolism, the onset of symptoms is usually dramatic because of lack of collateral circulation, and it manifests as severe and unrelenting abdominal pain, nausea, vomiting, and urgent bowel evacuation. This is the classical abdominal pain out of proportion to physical findings. Patients with SMA thrombosis frequently report a prodromal symptom complex of postprandial pain, nausea, and weight loss associated with chronic intestinal insufficiency .Approximately 20% of patients with mesenteric ischemia have nonocclusive disease, a poorly understood entity that often includes low cardiac output and diffuse mesenteric vasoconstriction. It most frequently occurs in the setting of an acute hemodynamic insult in elderly and critically ill patients, and in those with severe mesenteric atherosclerosis. Mesenteric venous thrombosis is the least common cause of mesenteric ischemia, representing up to 10% of all patients. Most cases are thought to be secondary to other intra-abdominal pathologic conditions , or clotting disorders .In regards to mortality, the etiology of mesenteric ischemia does make a difference. In a review of over 3000 patients, the mortality rate following surgical treatment of arterial embolism and venous thrombosis (54.1% and 32.1%) was less than that after surgery for arterial thrombosis and non-occlusive ischemia (77.4% and 72.7%) [


Timely diagnosis of meningeal carcinomatosis is often difficult even with the assistant of magnetic resonance imaging examination, cerebrospinal fluid analysis, or both. To the best of our knowledge, gadobutrol-enhanced MRI has not been reported in the diagnosis of meningeal carcinomatosis. Here we present two cases where meningeal carcinomatosis was identified on gadobutrol-enhanced magnetic resonance imaging.We identified two cases of meningeal carcinomatosis who had been diagnosed with malignant tumors several years ago. Both patients presented with progressive headache and seizures. Gadopentetate dimeglumine-enhanced magnetic resonance imaging of the brain was performed and did not detect any abnormality of meninges. Lumbar puncture was performed repeatedly, but cerebrospinal fluid cytology showed no evidence of malignant cells. Finally the gadobutrol-enhanced magnetic resonance imaging detected the meningeal metastasis, and supported the diagnosis of meningeal carcinomatosis.Gadobutrol provides higher lesion conspicuity and enhances lesion detection in meningeal metastasis compared with gadopentetate dimeglumine. Our observation is a cue to analyze the accuracy in the diagnosis of meningeal carcinomatosis, and presents a choice that may facilitate early diagnosis. Meningeal carcinomatosis (MC) which results from the multifocal seeding of meninges by malignant cells is diagnosed in 1–5 % of patients with malignant tumors . However2O, but CSF cytology showed no evidence of malignant cells. Her CSF carbohydrate antigen (CA) 12-5, carbohydrate antigen 19-9 (CA19-9) were 976.7U/mL, 126.7U/mL compared to 1087.2U/mL and 130.9U/mL in serum. Chest and abdomen computed tomography (CT) scan showed no evidence of abnormality. Two weeks after admission, the patient had frequent tonic-clonic seizures, and complained of severe headache. Multidisciplinary teamwork recommended repeated CSF cytology examination, and brain MRI scan using gadobutrol  as contrast agent. Gadobutrol is not covered by patient’s medical insurance in our hospital and patients need to pay for it. As a result, we chose the gadopentetate dimeglumine which is in the range of medical insurance as the initial contrast agent for MRI scan. Finally the gadobutrol-enhanced MRI  detected the meningeal metastasis  was performed and did not detect any solid tumor Fig , b, c. Lasis Fig , and supA 53-year-old female was referred to our department from a local hospital for further diagnosis and treatment after being diagnosed with a space-occupying lesion in the left occipital lobe. The patient presented with headache accompanied by nausea and vomiting for 10 days. In 2010, she had been diagnosed with and resected for gastric adenocarcinoma. Gadopentetate dimeglumine-enhanced MRI scan  was performed to identify the occipital lesion. On the MRI scan, an ovoid mass with the diameter of 1.6 centimeter can be seen in the left occipital lobe. The mass was hyperintense on T1-weighted images, and iso- to hypointense on T2-weighted images Fig , b. AfteMeningeal carcinomatosis is the last stage of patients with malignant tumors. Basically, CSF dissemination may happen in all patients with a malignant tumor but is most common in patients affected by lung cancer, breast carcinoma, melanoma or hematologic neoplasms such as lymphoma and leukemia . The two2O was consistent with her headache. The headache progresses quickly and is usually insufferable in the last stage until to cerebral herniation [The clinical presentations associated with MC are symptoms mainly due to increased intracranial pressure such as headache, nausea and vomiting, neck pain as well as confusion. The two patients in this study all presented progressive headache from the early stage to the end and the opening pressure of the first patient increasing from 200 to 350 mmHrniation . TherefoBrain gdMRI plays a growing role in the diagnosis of MC especially when CSF cytology is negative . PostconThe gold diagnosis standard is CSF cytology with the specificity of about 95 % for MC . HoweverMC is usually fatal for patients with advanced cancer and the median survival ranges from 4 to 6 weeks. Considering the poor prognosis early intervention may prolong survival and relieve symptoms for patients. Radiotherapy and chemotherapy are the main therapeutic approaches, and the supportive care is also very important for patients. Because the studies of MC are small clinical series, retrospective analyses or clinical experience, there are no clear guidelines which treatments fits best for each patient.Although rare, MC should be considered a possible diagnosis when the patient presents with progressive headache and further examination such as CSF cytology and brain MRI should be arranged to identify the disease. Brain gdMRI plays a growing role in the diagnosis of MC especially when CSF cytology is negative. Gadobutrol provides higher lesion conspicuity and enhances lesion detection in meningeal metastasis compared with gadopentetate dimeglumine. These findings may be a cue to analyze the accuracy in the diagnosis of MC, and present a choice that may facilitate early diagnosis.


Cut sections of the mass revealed mainly yellow fatty tissue surrounded by a thin fibrous capsule with numerous interlacing thin lamellar bony structures. The definitive pathological diagnosis was osteolipoma without evidence of malignancy. No recurrence was observed at eight months follow-up.A lipoma containing mature osseous elements is called osteolipoma. This article describes a giant osteolipoma fixed to the periosteum of the greater trochanter of the femur. A seventy-year-old woman presented with a large subcutaneous mass in the right buttock which had been present for six years. On local examination, a giant mass that was ovoid, firm, non-tender, well demarcated, subcutaneous, and relatively fixed to the greater trochanter was palpated in the right buttock. A medical imaging and fluoro-deoxy-glucose (FDG) bone scan revealed a large lipomatous and metabolically active lesion arising from the periosteum of the greater trochanter of femur. The excisional mass of 17 × 8 × 7 cmOsteolipoma with an osseous basal portion is rare. Surgical excision is the treatment of choice and the prognosis is good. To the best of our knowledge, this is the first report of an unusual giant osteolipoma fixed to the periosteum of the greater trochanter of the femur. Lipomas are the most common benign soft tissue tumors and can occur anywhere in the body . When a Written informed consent was obtained from the patient for the publication of this case report and accompanying images.A seventy-year-old woman presented to the authors with a painful mass in the right buttock. The patient noted the swelling six years back, which was insidious in onset and gradually progressive in nature, but she did not bring it to the attention of a physician because it caused only slight discomfort while sitting. She had no family history and had not suffered any severe trauma. Two weeks prior to presentation, her discomfort became obvious and she began experiencing pain while sitting and walking. The pain was dull-aching in nature and did not radiate to other regions. On routine physical examination, she appeared healthy with no known medical comorbidity. Her gait was normal.On local examination, a giant mass that was ovoid, firm, non-tender, well demarcated, subcutaneous, and relatively fixed to the greater trochanter was palpated in the right buttock. The hip and knee movements were normal. The neurological examination was within normal limits, and no lymphadenopathy was present.Her laboratory parameters were normal except for a raised erythrocyte sedimentation rate (36 mm/hour). A plain X-ray revealed dispersed calcification close to the periosteum of the greater trochanter of the femur Figure .An MRI revealed a well-circumscribed oval, lobular lipomatous mass that had high signal intensity on T1-weighted images Figure  and low The dense osseous tissue within the fatty core appeared as a hypointense cortical line on magnetic resonance imaging. A fluoro-deoxy-glucose (FDG) bone scan revealed a metabolically active lesion arising from the posterior aspect of the greater trochanter, showing chondroid type of calcifications within and low grade FDG uptake associated with a large soft tissue component showing fat density Figure .The preoperative differential diagnoses were primarily a lipoma with tumoral calcinosis, myositis ossificans, or an osteochondroma.An ultrasound-guided (USG) needle biopsy was performed revealing only benign mature fat cells and was evaluated as non-diagnostic. An excisional biopsy was undertaken. During the operation, a well encapsulated tumour mass was found to be located in the gluteal region underneath the gluteus maximus muscle Figure .At the base of the tumour, a basal osseous part was found arising from the periosteum of the greater trochanter of the femur. The tumour mass and the basal osseous part were removed surgically Figure , and the3 with a smooth surface  is rare.The only previously reported case of osteolipoma in relation to the proximal femur was an intramuscular ossifying lipoma without evidence of malignancy in a middle-aged man . In thisThe pathogenesis of osteolipoma remains unclear, although two main theories exist. First theory: the tumour may originate directly from multipotent mesenchymal cells -3. SeconImaging features of osteolipoma are pathognomonic -7. A plaA definitive diagnosis is established with a histopathological examination. Microscopic appearance of mature adipose tissue with interlacing mature lamellar and woven bone in areas of ossification is the mainstay of the diagnosis . The adiOsteolipoma with an osseous basal portion is extremely rare. Surgical excision is the treatment of choice and the prognosis is good. Although osteolipomas are rare, it is necessary to have a high index of suspicion when encountering a lesion with adipose tissue in combination with ossification.


Gossypiboma is a serious and potentially dangerous medico-legal problem.We present a case of lower abdominal gossypiboma that presented as an abdominal cystic mass mimicking ovarian teratoma. The mass and the adhesive intestine loop were en blocly resected. The cut section confirmed gossypiboma diagnosis.The present experience and related literature results indicate that gossypiboma should always be kept in mind for the differential diagnosis of cystic soft-tissue mass detected in patients with a prior operation history despite its rarity and diagnosis difficulty. Once detected or suspected, appropriate surgical intervention should be performed promptly. Most importantly, preventing is much more crucial than curing in order to avoid this highly undesired potential complication. Gossypiboma is referred to as a surgical gauze or towel retained inadvertently in the human body during surgery and the resulting reactions. Although it is a rare and preventable occurrence, this challenging medical situation which may induce considerable morbidity and at times even mortality, is a serious and potentially dangerous medico-legal problem , 2. HereA 32-year-old woman was admitted to our hospital Obstetrics and Gynecology Department with intermittent left lower abdominal pain about half month. She had undergone cesarean section through abdominal incision at a suburban maternity hospital 8 years earlier. The results were uneventful. In the follow-up, the patient suffered sometimes from mild intermittent left lower abdominal pain, but eased 1–2 days without any treatment, the problem was explained with postoperative adhesion, and not performed further advanced examinations. She denied any history of fever, vomiting, constipation, or body weight loss at that stage. On physical examination, a lower midline laparotomy scar was noted; a smooth, round, mobile, nontender mass was palpable in the left hypochondrium. On bimanual pelvic examination, a 5 cm × 8 cm mass was felt in the left adnexa region. Blood tests including tumor marker carcino-embryonic antigen (CEA), carbohydrate antigen 199 (CA199), and carbohydrate antigen 125 (CA125) were within normal values. Ultrasound detected a well-encapsulated cystic mass in the left lower quadrant. The lesion showed echogenic foci and multiple linear reticular echogenic structures within it, without blood flow Fig. . NoncontThe patient was optimized and taken up for elective laparoscopic exploratory operation. Upon operation, a well-circumscribed firm mass was found, which was about 6 cm × 5 cm, densely adhered to the adjacent greater omentum, sigmoid colon, and intestine, and could not be removed by laparoscopic operation. The uterine and bilateral ovaries were normal except a 3.7 cm × 3.5 cm right ovary cyst. After enucleating the right ovary cyst and consulting the general department surgeon, the operation stopped. The patient was transferred to general department, and laparotomy via a prior surgical incision line under general anesthesia after bowel preparation was performed. At operation, adhesive omentum and sigmoid colon were separated, and the small bowel was too dense to separate, so the densely adhesive intestine loop and the mass were en blocly resected Fig. . The cutGossypiboma, otherwise known as textiloma, is a rare incidents caused by retained postoperative foreign bodies. The reported incidence varies between 1 in 1000 and 1500 of all intraperitoneal operations. Probably, the condition is underestimated owing to diagnostic difficulties and medico-legal implications associated with it , 4. GossGossypiboma clinical manifestation varies and is strongly related to the body reaction as well as the characters of the retained sponge. The patient may present acutely, within months of the original surgery, or may have a delayed presentation years after previous operation. Gossypiboma triggers two types of biological reactions, aseptic fibrotic reaction or exudative inflammatory reaction . The forGossypiboma usually creates a diagnostic dilemma since clinical symptoms are always not characteristic, and the imaging methods are often uncertain , 15, 16.On plan X-ray, the radiopaque marker attached to the sponge may be easily detected; however, due to the possibility of folding, twisting, or disintegrating over a period of time, the surgical material and marker may be difficult to be identified on a radiograph.On CT scan, which is the preferred modality, gossypiboma containing gas bubbles and a whorl-like appearance is characteristic. The lesion may appear as a cystic lump with internal spongy appearance mimicking teratoma or dermoid cyst. Occasionally, it may manifest as a hypodense mass, which has a thick peripheral rim and usually misinterpreted as a new-onset tumor or a recurrent tumor , 17, 18.The typical ultrasonic performance usually presents as a well-defined mass including internal wavy hyperechoic focus, encompassing a hypoechoic rim and having a strong posterior shadow. However, owing to the clinical rarity, this performance is often misinterpreted.To prevent severe gastrointestinal complications or to overcome the accompanied medico-legal problems, appropriate surgical treatment should be performed as early as possible when gossypiboma is detected or suspected. The most commonly adopted approach is surgical removal through the previous operative site, but treatments like percutaneous, endoscopic, or laparoscopic approaches were also be attempted and reported .Precautions are much more crucial than cure in order to avoid this serious detrimental problem. Strict adherence to surgical material count prior to closing the surgical wound is imperative to avoid the occurrence of this highly undesired potential complication. Surgical materials with radiopaque markers which are useful in reducing the incidence of this condition and making diagnosis in suspected cases should be adopted widely. Although human mistakes cannot be completely eradicated, continuous healthcare staff medical training and vigilant adherence to rules of the operation theaters should reduce the incidence of gossypiboma to a minimum . GawandeGossypiboma is a rare and preventable challenging medical situation. If the diagnosis is neglected and not intervene timely, it may cause detrimental impact on patient and the healthcare staff. Once detected or suspected, appropriate surgical intervention should be performed promptly. Most importantly, preventing is much more crucial than curing in order to avoid this highly undesired potential complication.


Echinococcus multiloculariscauses human alveolar echinococcosis, commonly affecting the liver. However, in ∼1% of cases, systematic spread of the disease involves the brain as well. A patient had a 6-year history of liver and lung alveolar echinococcosis that was considered not suitable for surgery, and treatment with albendazole was introduced. After the appearance of neurologic disturbances, an intracranial mass lesion was demonstrated by radiologic imaging. The lesion was surgically removed, and histologic analysis revealed metacestode tissue ofE. multilocularis. Despite the surgical resection of the lesion, the patient died of progression of systemic alveolar echinococcosis. The authors highly recommend implementing neurologic monitoring to the follow-up algorithm for patients with systemically disseminated alveolar echinococcosis. When neurologic symptoms occur, radiologic imaging of the brain should be obtained immediately. Surgery should be considered for all intracranial echinococcal lesions, unless the lesion is located in the eloquent brain area.The fox tapeworm A 63-year-old woman residing in northern Lithuania was admitted to the Department of Neurosurgery at Republican Vilnius University Hospital with severe headache, progressive left-sided hemiparesis, and seizures. These symptoms, along with ataxia and left-sided facial nerve palsy, were present for several weeks and gradually worsened. The patient was diagnosed 6 years before with liver alveolar echinococcosis (AE) stage IV P4N4M1 with transdiaphragmatic bilateral pulmonary dissemination ,2.1Magnetic resonance imaging (MRI) of the brain demonstrated an irregular, contrast-enhancing, 40 × 34 × 25-mm nodular lesion in the postcentral gyrus surrounded by extensive perifocal edema .During surgery, a solid lesion of the right motor cortex was identified and totally removed. The lesion was white to gray, multilocular, limited, and easily distinguishable from the normal brain tissue .The histopathologic examination of the resected lesion revealed multiple cysts with periodic acid-Schiff-positive cuticular layers surrounded by necrosis, heavy infiltration of inflammatory cells, and granulomatous reaction characterized by epithelioid cells and multinucleated giant cells. These morphologic findings confirmed the suspected diagnosis of AE .The patient had a full neurologic recovery postoperatively. On the second postoperative day, she experienced abdominal pain. Clinical and radiologic investigation  demonstrated no signs of peritonitis or intestinal obstruction (ileus), but ascites was noted, which increased progressively to produce respiration insufficiency. On the fifth postoperative day, the patient died of cardiopulmonary insufficiency.Echinococcus multilocularisis of increasing public health concern, with the highest incidences recorded in Europe, varying from 0.03 per 100,000 inhabitants in 2004 to 0.57 in 2009 and 0.74 in 2012. AE primarily affects the liver but in a quarter of the cases in Lithuania extrahepatic disseminations were observed, and in 35.4% of the cases, the survival was less than 1 year from diagnosis due to late diagnosis at stage IV of the disease. For the control of this zoonosis, timely diagnosis and treatment in humans as well as the development of local control programs present major challenges.In Lithuania, AE caused by the fox tapewormAE with resultant brain involvement is rare (1%).Cerebral lesions can be solitary and multifocal. In general, they are supratentorial and distributed within the middle cerebral artery.Currently, there is no consensus on treatment of brain AE. Radical resection is the treatment of choice, except for the cases when a surgical approach may cause mortality or considerable morbidity . The patients undergo chemotherapy with benzimidazole derivates  for 2 years after the radical surgery. Albendazole is a first-choice drug for the treatment of cerebral AE, because it penetrates the blood–brain barrier better than mebendazole. If resection was incomplete or if the case is nonsurgical, long-term or even lifelong treatment with albendazole is initiated.Echinococcus-endemic region) when examining the patient. The appropriate diagnostic tools help to establish the correct diagnosis quickly. Specific medical therapy prevents the progression and systemic spread of echinococcosis.We report our experience of surgical treatment of brain AE. Although extremely rare (<1%), cerebral echinococcosis should be considered as a differential diagnosis of intracranial mass lesions. In general, clinicians must be well aware of the echinococcosis problem and must carefully consider the clinical  and epidemiologic details (i.e., direct contact with the definitive hosts, residence in anThe AE lesion in the brain may have been a consequence of metastatic spread of the disease from the primary lesion in the liver, which could have happened during a time of inappropriate medical control of the disease. Such a moment might have occurred when the patient experienced adverse effects from the medical treatment, which could have led to the patient taking the drugs irregularly or in inadequate doses.Another explanation may be that the multiple oncospheres' invasion resulted in all three primary lesions in the liver, lung, and brain, but for some reasons the brain lesion became clinically evident the latest. Aoki et al reported a case of a patient infected with at least two oncospheres that developed into two small primary separate cysts in different liver segments.Our patient had her first brain MRI scan when neurologic symptoms appeared and not at the time of diagnosis of the disease, so the primary brain lesion/metastatic brain lesion cannot be proved or denied.If we presume that the brain lesion was a primary lesion along with the liver and lung lesions, why then was it clinically silent for more than 13 years? Is it possible that liver echinococcosis progressed while the brain lesion was occult despite the poor drug penetration into the brain tissue and fewer intracranial mass compensatory mechanisms? This question could only be answered if neurologic and neuroradiologic investigations had been initiated at the time of diagnosis of disseminated AE.Difficulties in controlling the dangerous progression of AE led to the death of the patient. At the beginning, the patient was diagnosed as being in the terminal stage of the disease. We believe that the asymptomatic period may have lasted for many years. Multidisciplinary screening and follow-up along with the albendazole therapy and palliative surgery increased the survival for 7 years after the establishment of the diagnosis.The authors highly recommend introducing neurologic screening into the treatment and follow-up algorithm for the patients with disseminated AE even though no neurologic disturbances are evident. This recommendation is especially important when the patient has the disease for a long time or does not get adequate treatment. When neurologic symptoms occur, brain MRI scan should be obtained immediately. Surgery should be considered for all intracranial echinococcocal lesions, unless the lesion is located in the eloquent brain area. In summary, clinicians must be aware of this condition to allow early diagnosis, facilitate referral, and ensure prompt treatment. Proper medical treatment, regular long-term multidisciplinary follow-up, and early surgical interventions may improve the clinical outcome and survival.


To report the association between thoracic duct ligation and acute pancreatitis. The association between sudden stop of lymphatic flow and pancreatitis has been established in experimental models.A 57-year-old woman operated for thymoma presented a iatrogenic chylothorax. After thoracic duct ligation, she presented an acute pancreatitis which resolved after conservative treatment. The chylothorax disappeared within 4 days of thoracic duct ligation.This is the first report of acute pancreatitis following thoracic duct ligation. The pancreas and digestive tract should be assessed in symptomatic patients after thoracic duct ligation.The online version of this article (doi:10.1186/s12893-017-0204-3) contains supplementary material, which is available to authorized users. Chylothorax is a rare disease defined as a leakage of chyle into the pleural space, and can be classified as traumatic or non traumatic. Esophageal surgery is the major cause of traumatic iatrogenic chylothorax. Other iatrogenic causes include lymph node dissection, lung resection and mediastinal tumor resection , 2. A laWe report herein the first case of an abdominal complication associated with thoracic duct ligation, such as acute pancreatitis.A 57-year-old woman with a history of back pain underwent a thoracic CT-scan in 2016 with the incidental discovery of an anterior mediastinal mass. The mass had a size of 4x3cm, was round and was well delimited, compatible with a teratoma or a thymoma. The patient had no symptom or clinical manifestations of myasthenia gravis, with a Quantitative Myasthenic Gravis Score grade 0. A thymomectomy was performed using a right single-port video-assisted thoracoscopic surgery (VATS). A chest drain was left after the procedure. Histopathology confirmed a thymoma, type B1, Masaoka-Koga stage 1. Two days after surgery, the patient developed a high output chylothorax (>1000 mL/day), without any signs of infection or inflammation in the blood tests. We introduced a low-fat diet for 6 days and then she was fasting for two more days. However, the chyle continued to flow with an output of 750 mL/day without other complications. No somatostatin or analog was introduced. A lymphangio-magnetic resonance imaging (MRI) didn’t show aberrant thoracic duct anatomy nor chyle-leak (Fig. This case presentation was conducted in accordance with the CARE guidelines and methodology.The occurrence of chylothorax after thymomectomy is a rare complication and usually associated with extensive dissection . In thisFor chyle leak of traumatic iatrogenic etiology, such as mediastinal tumor resection, management is debated and to date no consensus exists. Initial conservative treatment involving chest drainage and a low-fat diet except for the medium-chain triglycerides or fasting with parenteral nutrition has shown excellent outcome. Octreotide or somatostatin therapy can be efficient for the medical approach . HoweverThe consequences of sudden disturbed lymph drainage of the abdominal viscera, which constitutes 80% of the lymphatic flow in the thoracic duct, are not known. It is assumed that with time collateral lymphatic vessels overcome the thoracic duct obstruction. Reports in literature show that thoracic duct ligation is safe and has no known associated abdominal or lymphatic complications, except two cases of leg edema associated with thoracic duct embolization . HoweverIn our case, common etiologies of acute pancreatitis were excluded, including hypotension and medications. Auto-immune-like pancreatitis was previously described in a patient with myasthenia gravis and autoantibodies . HoweverThe recurrence of chylothorax 2 days after surgery evokes aberrant collateral thoracic duct, which has not been seen on MRI nor during surgery. Probably this duct was small with a low chyle flow that could explain a rapid healing after a conservative treatment and a persistant disturbed lymph drainage of the abdominal viscera with abdominal pain.In conclusion, this is the first report of a patient developing acute pancreatitis after thoracic duct clipping. Although our knowledge relies on experimental models, edema of the abdominal viscera should be assessed in symptomatic patients with a CT-scan and a pancreatic enzymes analysis.


A 48-year-old woman with a history of voiding difficulty visited our hospital. Magnetic resonance imaging revealed a periurethral tumor, which was pathologically diagnosed as an adenocarcinoma via transperineal needle biopsy. Radical cystectomy and urethrectomy were performed, and the urinary tract was reconstructed using an ileal conduit. Pathological examination of a resected specimen confirmed adenocarcinoma of the urethral diverticulum. The patient received adjuvant gemcitabine and cisplatin chemotherapy. She is alive at 5 months since the operation. Urethral malignancies are rare in the female population. We encountered a case of urethral diverticulum cancer in a woman, which was difficult to diagnose and was treated with surgery and chemotherapy. Herein, we describe our experience with this case and review the relevant English literature.A 48-year-old woman with a history of voiding difficulty visited a nearby hospital, where she was prescribed urapidil. However, her symptom worsened, and, therefore, clean intermittent catheterization was initiated. For further examination, magnetic resonance imaging (MRI) was performed, which revealed a periurethral diverticulum . The divThree months later, transvaginal ultrasonography showed that the size of the diverticulum had not reduced. Subsequently, she was transferred to our department for further examination and treatment. In order to assess the diverticulum, MRI was performed, which revealed a periurethral tumor . The tumThe patient underwent transperineal needle biopsy of the tumor using transvaginal ultrasonography. The pathologic diagnosis was adenocarcinoma, and immunohistochemistry revealed CK7 positivity and CK20 negativity. The tumor cells did not express the estrogen receptor or progesterone receptor. Owing to lack of CDX2 expression, the gastrointestinal region was excluded as a possible site of origin of the tumor. The tumor had smooth muscle tissue and prostate-like tissue and was therefore diagnosed as a urethral cancer.We conducted radical cystectomy and urethrectomy with combined resection of the uterus and vagina. The patient's urinary tract was reconstructed using an ileal conduit. She was discharged from our hospital after 3 weeks without any severe complications. Pathological examination of a resected specimen revealed adenocarcinoma of the urethral diverticulum . The ureThe patient received 2 courses of adjuvant chemotherapy, comprising gemcitabine and cisplatin, as multidisciplinary therapy. Five months after surgery, she was free of recurrence and metastasis.Urethral malignancies are rarely reported in the female population, accounting for only 0.02% of all malignancies . Accordin = 8], T2 [n = 5], T3 [n = 22], and T4 [n = 9]) [ Grigsby examined the records of 44 women with carcinoma of the urethra  [Based on the factors in The patient presented with the chief complaint of voiding difficulty. In general, voiding difficulty among the female population is typically associated with a neurogenic disorder such as spinal cord injury, a neurogenic bladder due to diabetes mellitus, or impaired activity of daily living. Some cases are caused by a gynecological tumor or pelvic organ prolapse. Urethral tumors are rare and are thus likely to be overlooked. Therefore, we recommend imaging analysis via MRI or CT for further examination of voiding difficulty in relatively young female patients.


Oral lichen planus (OLP) is one of the common chronic inflammatory, noninfectious, and precancerous oral mucosal diseases that affect the stratified squamous epithelium in adults. Grinspan et al. reported an association of OLP with diabetes mellitus and vascular hypertension and called that Grinspan's syndrome in 1966. We aim to present a case of Grinspan's syndrome with malignant transformation.  A 60-year-old man who presented with a ten-year history of OLP diagnosed clinically and histologically was referred to our otolaryngology department with a painless swallowing in the left buccal mucosa for 3 months. Clinical examination revealed several plaques, striated white lesions in the buccal mucosa bilaterally, and an exophytic tumor in the left buccal mucosa. Histopathological examination showed lichen planus bilaterally and oral squamous cell carcinoma in the left buccal mucosa. The tumor had been developed on the preexisting areas of lichen planus which had been histologically proven before. The tumor was removed completely, and the tissue defect on the buccal mucosa was repaired with a split-thickness skin graft.  Patients with OLP should be followed up periodically in a long term at close intervals for early diagnosis of malignant transformation. Oral lichen planus (OLP) is one of the common chronic inflammatory, noninfectious, and precancerous oral mucosal diseases that affect the stratified squamous epithelium in adults , 2. In gAlthough the etiopathogenesis is still unknown, immunological mechanisms are blamed , 6. AutoOLP diagnosis can be made with clinical features like classic oral white lesions, but to confirm diagnosis and exclude the dysplasia or malignancy, the histopathological examination is recommended , 6.OLP has been associated with different systemic diseases such as primary biliary cirrhosis, thymoma, ulcerative colitis, chronic active hepatitis, and myasthenia gravis and with several viruses such as hepatitis C virus (HCV), human papillomavirus (HPV), human herpes virus 6 (HHV-6), and Epstein-Barr virus (EBV) . Some deAnti-inflammatory agents such as the topical corticosteroids are mainly preferred for OLP treatment; if lesions are widespread, systemic agents may be required , 5, 6.Patients with OLP should be followed up periodically in a long term at close intervals for early diagnosis of malignant transformation. Malignant transformation after 40 years from the initial diagnosis of OLP has been reported , 10.Grinspan et al. reported an association of OLP with diabetes mellitus (DM) and vascular hypertension (VHT) and called that Grinspan's syndrome . We presA 60-year-old man who presented with a 10-year history of OLP diagnosed clinically and histopathologically was referred to our otolaryngology department from dermatology department. The complaints of the patient were burning sensation after hot or spicy foods in the left buccal mucosa and painless swallowing in the left buccal mucosa for 3 months . There wClinical examination revealed several plaques and striated white lesions on the tongue and in the left and right buccal mucosae  and an eThe tumor had been developed on the preexisting areas of lichen planus which had been histologically proven before. The tumor was removed completely, and the tissue defect on the buccal mucosa was repaired with a split-thickness skin graft. OLP may clinically demonstrate various forms such as reticular, erosive and ulcerated, atrophic, hypertrophic, bullous, and pigmentous forms , 6. RetiThe incidence of oral mucosa malignancies is reported about 0.004% per year with an 80% rate of OSCC in Europe . OSCC maNowadays, the malignant transformation of OLP is still a controversial subject , 5, 8. TBoth OLP and lichenoid reactions can cause OSCC. In a recent meta-analysis of 57 studies with the data on 19,676 OLP and 419 oral lichenoid reaction cases, Aghbari et al.  reportedKrutchkoff et al. had established the criteria of malignant transformation of OLP as follows: there must be a 2-year follow-up period after the initial clinical and histological diagnosis of OLP and the absence of carcinogenic factors such as exposure to tobacco and alcohol history . Our patDiabetes mellitus (DM) is a clinical entity with hyperglycemia due to insulin defect. Disregulation of insulin, glucose, and lipids can lead to skin lesions in some DM patients . GrinspaIn our case, the patient was followed up for lichen planus earlier by dermatologists; after recognition of mass in the left buccal mucosa, he was referred to us. Hematological and histopathological investigations showed that the patient has Grinspan's syndrome with malignant transformation. The patient underwent surgical treatment and followed up monthly for the first year after the operation. Now, no relapse or any sign of metastasis was observed in the patient 33 months after the operation.Malignant transformation is the most important complication of long-standing and nonhealing lichen planus. Close follow-up and repeat biopsies are mandatory for prevention and early recognition of malignant transformation. It is still controversial about Grinspan's syndrome that DM and VHT are accompanying OLP or medication of DM and VHT leads to oral lichenoid lesions. We presented a case with Grinspan's syndrome which appeared before DM and VHT medication and with malignant transformation.


Idiopathic pyloric stenosis in adults is a rare condition of unknown etiology, caused by hypertrophy and hyperplasia of the pyloric musculature with gastric outlet obstruction and delayed gastric emptying. It should be differentiated from the secondary form, caused by recurrent peptic ulcers, malignancy or hypertrophic gastritis. Symptoms include epigastric pain, early satiety and postprandial nausea and vomiting, however many cases remain asymptomatic for years. Although pyloroplasty is an acceptable option, distal gastric resection is the recommended approach, to avoid recurrence. We report the case of 44-year-old man who presented at the emergency department, with a 5-day history of protracted vomiting, abdominal pain and constipation. He appeared severely malnourished, complaining of malaise and generalized weakness. Clinical examination revealed hypotension (90/60 mmHg) and tachycardia (100 bpm), abdominal distention with hyper-resonance during percussion and diffuse tenderness on palpation. Other than leukocytosis , laboratory tests were within normal range and the nasogastric tube produced 200ml of clear, non-bilious fluid. CT scans showed a markedly dilated stomach (A), causing compression of the inferior vena cava and the solid organs of the upper abdomen . He was transferred to the operating theater for exploratory laparotomy, however he collapsed hemodynamically upon induction to anesthesia, and succumbed despite attempts to CPR.


Introduction: Bilateral Wilms’ tumors with an unfavorable histology require a combined treatment .Objective: Presentation of the first renal transplant performed in Romania in a child with bilateral Wilms’ tumor, at 3 years and 4 months after the end of a multimodal treatment.Material and methods: Patient C. N., born on 30.04.1998, was diagnosed in 04.2001 with right parenchymal renal tumor, polycystic kidney, left cystic renal tumor. 25.04.2001 - right radical nephrectomy and partial left upper pole nephrectomy; histopathology examination: triphasic bilateral nephroblastoma, reactive lymph nodes, negative resection edges in the left kidney. 30.04-19.11.2001- polychemotherapy according to the NWTS-5 stages 2-4 focal anaplasia and radiotherapy of the right kidney bed (29.06.2001). 02.2002- a nephrotic syndrome on the remnant kidney which requires its excision and peritoneal dialysis. Abdominal control CT was normal in 03.2005. 11.03.2005- renal transplant from living related donor. Results: Favorable post-transplant course with normal renal clearance values; at 2 months, normal urography control.Conclusions: The tumor pathology does not represent an absolute contraindication for renal transplantation. For the cases with extensive surgery, polychimiotherapy and radiotherapy correctly applied, a pre-transplant “tumor-free” period of at least 2 years is compulsory.  The nephroblastoma [Wilms’ tumor] is the most frequent solid tumor in childhood, representing 5% of all cancers of the child. Concomitant bilateral Wilms’ tumors occur in 5% of cases. Histologically, they are characterized by tissue and cell polymorphism, with various rates of “nephrogenic” , epithelial and stromal structures. The most important negative prognostic factors remain the unfavorable histological subtypes . Bilateral Wilms’ tumors (stage 5) with an unfavorable histology require a combined treatment . Approximately 15% of children with Wilms’ tumors have recognized congenital abnormalities: aniridia, Beckwith-Wiedermann syndrome, genitourinary abnormalities .Up to 15% of patients with Wilms tumors and congenital malformations with favorable post-therapeutical outcome, will develop a second malignancy due to radiation therapy and/or inherited susceptibility.The article’s objective is the presentation of the first renal transplant performed in Romania in a child with bilateral Wilms tumor, at 3 years and 4 months after the end of a multimodal treatment.Male patient, C. N., born on the 30th of April 1998, was investigated in April 2001 for fever (38.5°C) and for the occurrence of an abdominal tumor, the diagnosis  being right parenchymatous renal tumor, polycystic kidney, left cystic renal tumor. On the 25th of April 2001, a right radical nephrectomy and partial left upper pole nephrectomy were performed; results of the histopathology examination: triphasic bilateral nephroblastoma, reactive lymph nodes, without any tumor invasion at the level of resection edges in the left kidney. During the period 30.04-19.11.2001, the patient underwent a PCT treatment according to the NWTS-5 stages 2-4 focal anaplasia, as well as radiotherapy at the level of right kidney bed (29.06.2001). The treatment was additionally burdened by the occurrence of arterial hypertension requiring treatment with Captopril and by a moderate elevation of proteinuria values. Abdominal and thoracic CT after 2 months was normal. In February 2002, a nephrotic syndrome develops on the remnant kidney, which does not respond to immunosuppressive treatment and which, associated with uncontrollable arterial hypertension and proteinuria, requires excision of the remnant kidney. A hemodialysis program is attempted, but it is interrupted because of arterial hypotension. Patient enters a peritoneal dialysis program, the course of which is complicated by multiple and recurrent abdominal hernias, as well as by two peritonitis episodes (April 2003 and June 2004). Abdominal control CT was normal in March 2005. On the 11th of March 2005, kidney transplantation from living related donor (mother) is performed.Favorable post-transplant course without surgical or immunological complications, with normal renal clearance values; at 2 months, normal urographic control; at 12 months, abdominal CT control is normal. Three years after renal transplantation, serum creatinine level is normal, the renal graft is morphologically normal and the child is living with normal parameters according to age.The most complete therapeutical option for patients with end stage disease is kidney transplantation.The tumoral pathology does not represent an absolute contraindication for the renal transplant. There are though two major reasons for excluding patients with malignant disease: the first is that immunosuppressive drugs may unfavorably influence the natural history of the malignancy; the second is that it is not reasonable for someone whose life expectancy is significantly curtailed by the presence of malignant disease to undergo transplantation.But, the multimodal approach of the treatment of children with nephroblastoma has significantly improved outcomes. The IIIrd National Wilms’ Tumor Study (NTWS) reported an overall survival rate without tumoral recurrence of 85-92, 4% and for patients with bilateral Wilms’ tumor revealed a 3-year survival rate of 82%.For the cases with extensive surgery, polychemotherapy and radiotherapy correctly treated patients with bilateral Wilms’ tumors, a pre-transplant “tumor-free” period of at least 2 years is compulsory.Also, long-term follow-up is mandatory for patients with pretransplantation nephroblastoma (because of immunosuppressive drugs), especially for children with Wilms’ tumors and associated congenital abnormalities.


Spontaneous perforation of the colon is a well documented but rare clinical entity commonly found to occur in the elderly and associated with chronic constipation. Hypothyroidism is known to be associated with intestinal motility disorders ranging from chronic constipation to more serious conditions like mega colon and pseudo obstruction. The case described here is that of a 35 years old hypothyroid male who presented with perforation peritonitis due to spontaneous perforation of the rectosigmoid. A thorough search of literature shows only one report of spontaneous perforation of the colon associated with hypothyroidism, to date. This case gives an opportunity to review the literature and discuss such unusual and dangerous presentations of hypothyroidism associated colonic motility disorders. Spontaneous perforation of the colon (SPC) is a rare condition that occurs in the absence primary bowel pathology such as tumours, diverticulosis, chronic inflammatory disease or trauma . This di3. Thyroid hormone profile was T3 = 55 ng/dl , T4 = 3.5 ugm% , TSH = 22µ IU/ml . Plain abdominal X-ray revealed free intraperitoneal air under the right hemi diaphragm. Hence based on a provisional diagnosis of hollow viscus perforation, an emergency exploratory laparotomy was performed. At laparotomy the upper abdomen appeared relatively normal; however there was purulent fluid and multiple small fecoliths in the pelvis. A thorough search revealed a small circular perforation of about 1 cm diameter over the antimesenteric border of recto-sigmoid. The sigmoid colon was found to be thinned out and dilated with hard faecal matter within it. No other gross abnormality could be detected. The mesenteric artery pulsations were well felt. Hence a Hartmann’s procedure was performed using a linear stapling device, and a segment of about 10 cm of thinned out distal colon including the ulcer was resected. The post operative period was uneventful. Manual faecal evacuation and multiple enemas were given through the colostomy to evacuate impacted faeces. He was started on thyroid hormones 200 ug/day. Histopathology report was suggestive of a non specific ulcer with focal mucosal and sub mucosal neutrophilic infiltration  treatment since one year. He gave a history of chronic constipation which worsened since the last four months. He was also on treatment for acid peptic disease. On examination he was tachycardic (pulse rate: 100/min), blood pressure was 110/70 mm Hg. The abdomen was distended with maximal guarding and tenderness in the epigastrium. Bowel sounds were diminished. Laboratory investigations showed a total count of 14,900/mmltration .In 1827, Brodie described cases of middle-aged women whose rectums spontaneously ruptured . In 1919The exact aetiology of SPC is unclear. Stercoral ulcers of the colon, cortisone therapy, hypothyroidism, rectal prolapse, and psychiatric disorders have been suggested as contributory factors . SPC canSPC most frequently occurs at antimesenteric border of the rectosigmoid, which is an area of physiological ischemia. The lower water content of stool and also narrow intraluminal diameter are contributory factors. Maurer et al  reported-/HCO3- anion exchange with a subsequent effect on intestinal motility [Hypothyroidism is well associated with intestinal hypomotility, chronic constipation, faecal impaction, atony, distension, and pseudo-obstruction . Reductimotility .In this case it could be assumed that hypothyroidism was the cause of chronic constipation which in turn led to faecal impaction. The hard stools would have caused a solitary pressure ulcer which later perforated spontaneously. Furthermore, SPC commonly occurs in the elderly, unlike in this case where hypothyroidism could be the most likely cause for occurrence at a young age. There was no other obvious pathology such as a tumour, diverticulitis or chronic inflammatory disease, in this case. Histopathological findings are usually non-specific, consisting of acute inflammatory reaction surrounding the perforation site . In the The treatment for spontaneous perforation of the rectosigmoid is based on the same principles as other perforations of the rectosigmoid. Surgical options include primary closure, with or without proximal diverting colostomy and Hartmann surgery. In this case a Hartmann’s procedure was performed as a segment of relatively thinned wall unprepared faecal loaded colon had to be removed. Serpell et al  found thIn conclusion, SPC is a rare but noteworthy condition. It can be rationally assumed that SPC is a dangerous complication of the colonic motility disorders associated with hypothyroidism. Surgical options are same as for other causes of rectosigmoid perforations. A high index of suspicion is needed to correctly diagnose this condition. Symptoms of gastrointestinal dyskinesia due to hypothyroidism should not be taken unconscientiously.


Herniation of small bowel through the peritoneal defect of the Pouch of Douglas is extremely rare type of internal hernia, and this type of internal hernia has been described as an entity of perineal hernia. Here, we describe a case of a 26-year-old female without history of abdominal surgery presenting with incarcerated small bowel hernia through a peritoneal defect of the Pouch of Douglas. She visited an emergency department presenting with abdominal pain and distension. Without improvement symptom by conservative management, an operation was performed. During the operation, the distal ileum had been herniated through a peritoneal defect of the Pouch of Douglas, and there were no specific findings on gynecological examination. Reduction of the herniated bowel and primary repair of the peritoneal defect were performed. The case represents a very rare type of internal hernia and provides published cases of hernia through a peritoneal defect of the Pouch of Douglas.  Intestinal obstruction caused by internal hernia is rare, with an incidence of between 0.2% and 0.9% . DiverseA 26-year-old woman was admitted to the emergency department of Ilsan Paik Hospital with abdominal pain and distension lasting for 3 days. She had no history of abdominal surgery. Abdominal examination revealed mild distension and hyperactive bowel sounds. Signs of peritoneal irritation were not apparent and there were no remarkable laboratory data. Plain abdominal X-ray showed a dilated small bowel loop without free air. An emergency computed tomography (CT) revealed diffuse dilatation in proximal to mid ileal loop with abrupt luminal narrowing at distal ileum level without definite evidence of bowel ischemia . During Without improvement of symptoms and physical findings during conservative management, the patient underwent an exploratory laparotomy on 3rd hospital stay. During the operation, approximately 5 cm length of the distal ileum  had been herniated into a defect of the Pouch of Douglas, and there were no specific findings of pelvic examination . After rIn patients without history of abdominal surgery, causes of bowel obstruction such as colorectal cancer, inguinal hernia, intussusception, volvulus, and inflammatory bowel disease might be diagnosed based on physical examination and imaging studies preoperatively. However, the preoperative diagnosis of internal hernia may be difficult although preoperative diagnosis through CT has been reported , 8.Congenitally created peritoneal defect and foramen may lead to internal hernias . Among tThe Pouch of Douglas is an anterior peritoneal reflection between the uterus and the rectum which is so-called rectouterine pouch. Multiparity, old age, and history of pelvic surgery may lead to weakening or defect of the pelvic floor resulting in herniation of bowel though the Pouch of Douglas . This tyThe definitive diagnosis usually has been made during the surgery, although CT findings of hernia through a peritoneal defect of the Pouch of Douglas have been reported, in which a cluster of collapsed small bowel loops in the peritoneal defect between the rectum and the uterine cervix may be detected . In the The surgical treatments of perineal hernia consist of reduction of hernia sac, ligation, excision, and approximation of the uterosacral ligament, or obliteration of hernia sac using continuous sutures through the posterior wall of the cervix and the anterior wall of the rectum . HoweverThe preoperative diagnosis of internal hernia may be elusive because of the rarity of the disease entity, especially unusual type of internal hernia. Delayed diagnosis and treatment could result in morbidity and mortality. Thus, even though hernia through a peritoneal defect of the Pouch of Douglas is an extremely rare type of internal hernia and its preoperative diagnosis is difficult, a high degree of suspicion based on CT findings and history of abdominal surgery may be necessary for prompt management.


Hernioscopy is essentially hernia sac laparoscopy. Hernia repair has evolved over the years with better outcomes; however, strangulated inguinal hernias are acute surgical emergencies which require emergent operative intervention. During anesthesia induction and/or after incision, hernia self-reduction is possible, with or without compromised bowel, back into the abdominal cavity. It is pivotal to examine the bowel to decide on further operative course. A simple alternative to unnecessary laparotomy or standard laparoscopy is hernioscopy, which is quite uncommon. We present a case of an acute symptomatic strangulated left-sided inguinal hernia which got self-reduced during anesthesia induction and was successfully repaired after hernioscopy was used to evaluate the incarcerated hernia content. We provide a brief review of literature about hernioscopy and an algorithm to guide surgeons in emergent cases. Progressive advances in surgical techniques have made it possible to utilize the laparoscopic approach in a great variety of elective and emergency surgical cases. The number of patients undergoing laparoscopic surgery, and the physicians trained to perform it, are constantly increasing. It is well documented that minimally invasive surgery has its own perks and advantages over open surgery like reducing surgical site infection, postoperative pain, and morbidity, in addition to shorter hospital stay and better cosmetic outcomes.Inguinal hernia repair remains one of the most common surgeries performed in the US . When paSpontaneous reduction of an inguinal hernia is always a possibility and could represent a dilemma whether to proceed with exploratory laparotomy or laparoscopically evaluate intraabdominal contents. As no clear guidelines are currently present on the best method to rule out bowel necrosis and to assess its extent, exploratory laparotomy is usually performed at the surgeon's discretion and is associated with a higher rate of occurrence of major complications compared with the laparoscopic approach.In our limited review of the literature, we noted innovative practices of utilizing minimally invasive approaches for specimen extraction via natural and pathological orifices . A similA 57-year-old man with past surgical history of exploratory laparotomy after a motor vehicle accident 35 years ago and past medical history of asymptomatic left inguinal hernia for the past one year presented to the emergency department with complaints of left scrotal swelling, nausea, vomiting, and obstipation for six hours. On examination, he had a large, tender, non-reducible left inguinal hernia associated with abdominal distension. Vital signs were within normal limits.Laboratory workup showed that his lactate level was elevated to 3.3 mmol/L and the rest of the tests were unremarkable. White blood cell count was 9.5 k/uL, hemoglobin 15.8 g/dL, and hematocrit 49.4%. A computed tomography (CT) scan with oral and intravenous contrast was obtained, which suggested ascites and a large left inguinal hernia with multiple dilated loops of small bowel within the hernia sac and collapsed loops distally with a transition point at the neck of the hernia Figures .The patient was aggressively resuscitated with intravenous fluids and was taken to the operating room emergently for left incarcerated inguinal hernia exploration and repair. Following induction of anesthesia and groin incision, the patient’s hernia spontaneously reduced, thus precluding assessment of the viability of the bowel. For a complete bowel assessment and to rule out any ischemic changes, we decided to proceed with diagnostic laparoscopy through the hernia sac (hernioscopy) using a 12 mm trocar and a 30-degree angled laparoscope. The 12 mm trocar was placed through the inguinal hernia sac and through the deep inguinal ring into the peritoneal cavity and secured with umbilical tape (Figure A pneumo-peritoneum was successfully created and maintained. Two 5 mm trocars were then placed under direct vision in the left lower quadrant, and an assistant port in the right lower quadrant. The peritoneal cavity, including the small intestines and viscera, was examined in its entirety using laparoscopy. Findings included a small area of small mid-jejunum and corresponding mesenteric contusion, but no evidence of ischemia or necrosis. After this, a tension-free tissue repair of the inguinal hernia was done. The patient subsequently did well and was discharged home on postoperative Day 2 following adequate pain control, diet tolerance, and return of bowel function.  He was seen in the clinic two weeks later with minimal pain, no evidence of hernia recurrence, and continued bowel function.The practice of hernia repair has increased tremendously owing to the number of patient surgically treated annually. Hernia surgeries are one of the most commonly performed operations worldwide, with more than 800,000 inguinal hernias alone repaired annually in the US . The majAn incarcerated inguinal hernia is always an emergency and an indication for immediate surgery . Once inSpontaneous reduction of an incarcerated inguinal hernia without definitive evidence of viable content is a well-known problem surgeons may encounter. This could happen spontaneously in the operating room during induction of anesthesia or when an initial groin incision is already performed, and it is important to evaluate abdominal contents to prevent a disaster. However, there are no guidelines on the most appropriate intervention in case of hernia reduction and possible bowel ischemia/necrosis . When boExploratory laparotomy was the gold standard for evaluating strangulated content in case of hernia reduction; however, it may contribute to a longer hospital stay, later return to full ordinary activities , and incAs we mentioned before, with the advent of the laparoscopic approach, new techniques are emerging. Hernioscopy is a simple laparoscopy technique performed through the hernia sac. Laparoscopic assessment of bowel viability following reduction through the hernia sac is an easy and safe approach that can be employed in surgical practice especially in case of previous abdominal surgeries. The explorative potential of the technique with the current advances in laparoscopic instruments allows for a reliable assessment of the intestinal wall. Additional trocar placement could be done under direct visualization with minimized iatrogenic injury. In case of spontaneous inguinal hernia reduction, the content could be reliably observed until gradual reversal of the ischemic bowel discoloration, venous congestion, or the recovery of bowel peristalsis is confirmed, and groin incision repair is performed later in consistency with current guidelines. In case of bowel necrosis, resection could be done using the original groin incision; extension of subsequent port incisions under direct visualization could be safely done to deliver the necrotic segment away from the clean groin incision. Minilaparotomy and laparoscopic-assisted bowel resection, as well as laparoscopic resection, can relieve the patient from having a full laparotomy performed and the related complications as well as contamination of the hernia repair. Although there are no guidelines for spontaneously reduced incarcerated inguinal hernia management, we created an algorithm to help guide surgeons in emergency cases Figure .Hernioscopy demonstrates a safe and simple approach for incarcerated inguinal hernia management and reduced hernia content assessment with the perspective to prevent unnecessary exploratory laparotomies and related complications with possible potential decrease in perioperative morbidity. Further management could be titrated accordingly and depends on intraoperative findings and surgeon’s experience and preference. Hernioscopy could be an accurate and reliable adjunctive diagnostic tool to expand current practices, and our algorithm could be utilized as a helpful strategy for the surgical community with the objective of minimizing the number of unnecessary exploratory laparotomies and related complications in patients with incarcerated inguinal hernia.


Sclerosing encapsulating peritonitis (SEP) is a rare disorder that is characterized by encapsulation of bowel loops by thick fibrinogenous case. Most patients present with vague abdominal symptoms. It is challenging to diagnose the condition preoperatively. Surgical management is preserved for patients with small bowel obstruction with no improvement on conservative measures or for those with signs of bowel ischemia . Herein, we discuss the clinical signs and symptoms, the radiological features, the surgical management, and outcome of SEP based on a patient who underwent surgery in our hospital.  Sclerosing encapsulating peritonitis, or cocoon syndrome, is a rare disorder, which is usually seen in patients on continuous ambulatory peritoneal dialysis (CAPD). The exact known of the condition is still unknown. The gross pathology resembles a cocoon case encapsulating the bowel loops, hence the name. Patients with SEP often present with vague abdominal symptoms. A computed tomography in a patient with intestinal obstruction is likely to raise the suspicion of SEP; however, the case is usually diagnosed intraoperatively. Many patients would resolve with only conservative medical managements, but some may require surgical intervention. It is still unclear whether early surgical intervention has an advantage over conservative management, but, in most reviewed case reports, surgeons preferred to preserve the surgical management for those who do not respond to conservative measures , 2. HereA 50-year-old Pakistani gentleman, who was previously healthy, was admitted to our surgical department with generalized abdominal pain, absolute constipation, and abdominal distention. He had multiple admissions during the previous eighteen months with similar episodes, which were diagnosed as small bowel obstruction, by the clinical picture, and multiple air-fluid levels on abdominal X-rays, and always resolved on conservative measures. He was afebrile and hemodynamically stable. His abdomen was distended with no scars. He had generalized abdominal tenderness and exaggerated bowel sounds. His rectum had remnant of stools on PR examination. He had neutrophilic leukocytosis, but the rest of his labs were within normal limits. His axial abdominal X-ray showed multiple air-fluid levels. A computed tomographic scan of his abdomen, which was performed on the same day of admission, showed evidence of multiple bowel loops dilated in the left side of the abdomen with distal collapse of bowel (transition zone) with swirling sign of the vessels. Additionally, an encasement of the small bowel within a membrane-like sac was observed, with a few calcifications Figures  and 2. NSclerosing encapsulating peritonitis, also known as cocoon syndrome, is a poorly understood and rarely described condition. The pathogenesis is thought to be by the release of fibrin-like material by fibrinogenic cytokines . It is sThe clinical presentation of patients with SEP is usually vague, with nonspecific abdominal symptoms, including bloating, nausea, abdominal discomfort, constipation, or vomiting , 12, whiThe preoperative diagnosis of SEP is usually challenging. It takes high experience and knowledge of the disease to suspect its presence. To properly diagnose SEP preoperatively, imaging studies are of crucial importance. These include erect abdominal X-ray films, barium passage radiography, ultrasonography (USG), and computed tomography (CT). Abdominal X-rays would show air-fluid levels and dilated bowel loops in a patient with symptoms of intestinal obstruction. A barium swallow would show an accordion pattern and cauliflower appearance , 9, 12. The management of SEP depends on the presentation of the patient. In asymptomatic patients with idiopathic SEP, regular follow-up is all that is required . PatientIn conclusion, SEP is a rare, poorly understood condition in which a fibrous membrane encapsulates the bowel, possibly leading to intestinal obstruction and its consequences. Preoperative diagnosis is usually challenging. The use of the CT scans along with other imaging techniques may assist in reaching a correct diagnosis and subsequently the proper management. Although conservative management remains preferred in mildly symptomatic patients, surgical intervention is often required to prevent or deal with the complications of intestinal obstruction. A minimally invasive approach should be attempted to avoid troublesome complications.


Chondrosarcoma is one of the most common malignant bone tumors in adults. It use to affect upper arm, pelvis and thigh bone. A wide surgical extirpation represent the gold standard to treat this disorder. In fact, radiotherapy and chemotherapy are no useful. Interestingly, chondrosarcoma is rare in head and neck (HNCS) and extremely uncommon during pregnancy. Thus, there is a lack of evidence about the proper treatment in these cases. A wide surgical extirpation is also considered the most effective procedure in HNCS. There are no consistent evidences about the he role of radiation and chemotherapy. In view of that, the present study describes a case of juxtacortical mandibular chondrosarcoma affecting a 28-year-old pregnant woman. After a multidisciplinary analysis of the case, we decided to treat the patient with a wide surgical resection and and immediately reconstruction. Key words:Mandibular chondrosarcoma, pregnancy, surgical extirpation, radiotherapy, chemoteraphy. Chondrosarcoma is a malignant cartilaginous tumor characterized by the formation of cartilage, but not of bone, by tumor cells . The mosIn this line, chondrosarcoma is also uncommon during pregnancy. In fact, only 10 cases of gestational chondrosarcomas was described in the literature during the last 25 years. Only one of these cases affected craniofacial area . The effDue to the rarity of head and neck chondrosarcoma (HNCS) there are no established treatment protocols. Many of the current treatment approaches stem from protocols developed and tested for chondrosarcomas of other more frequent locations. However, the adequate surgical resection remains the gold standard for the treatment of the jaw chondrosarcoma. In fact, the real role of chemotherapy and radiotherapy is still controversial .The article describes a case of a juxtacortical chondrosarcoma of the mandible affecting a 28-year-old woman. Interestingly, she was 31 weeks pregnant.More in detail, the patient referred the onset of a painless lump in the mandibular symphysis at the emergency department of our hospital. The lesion appeared spontaneously a few weeks earlier. No trismus, paresthesia or other symptoms were reported by the patient. At the exploration, the mass was fixed to the jaw without mobility. Moreover, the patient related a rapid growth Fig. . AgainstThus, after the presentation of all the evidence we classified the cancer as cT3N0M0. In the oncological committee of our hospi-tal, we decided to treat the case with a wide surgical resection and inmediately reconstruction. Surgery was performed three weeks after delivery. The extension of the injury obliged to perform a mandibulectomy from the tooth 44 to 37, including the skin of mental and submental region. The reconstruction was performed by using a microsurgical scapular flap . The clinical behavior is variable. However, the majority 80%-90%) are low-grade malignant tumors. The median age of presentation varies between 17 to 75 years and it occurs more often in females . Close or positive margins are related with an increased risk of recurrence. Local recurrence is the main cause of death. Other factors that influence the global survival are represented by stage at diagnosis, histopathological grade and location. In general, lymph node metastases and distant metastases are infrequent. Survival rates for HNCS range between 44 and 88 % .Postoperative radiotherapy and systematic chemotherapy might be useful to improve disease free survival and overall survival. Notwithstanding, there are no consistent evidences about the effectiveness of these treatment. Chondrosarcoma appears to be resistant to radiation treatment. Postoperative radiotherapy is recommended only for high-grade tumors and for unresectable lesions. On the other hand, an effective chemotherapy regimen does not exist at the moment .A wide surgical resection is also the first choice of treatment in skeletal chondrosarcoma of during pregnancy . In our Resuming, this case stresses the importance of wide surgical resection. However, no evidences exist and more research is needed in this field. This particularly applies to HNCS during pregnancy.


Background: Persistent Müllerian duct syndrome (PMDS) is not a common form of disorder of sex development in which Müllerian duct derivatives  are present in an otherwise normally differentiated 46 XY male. In most of cases, the challenge comes in the procedure of orchiopexy.Case Presentation: We report a case of a 26-year-old man with PMDS. It was accidentally discovered when the patient presented to our outpatient clinic concerning about his empty scrotum as a premarital check. Diagnostic laparoscopy discovered Mullerian remnants in the form of uterus, cervix, and fallopian tubes with two attached testes to the fallopian tubes. Staged laparoscopic orchiopexy was done. We discuss the presentation, the management of this case in the literature, and our intervention.Conclusion: PMDS is not a common condition. Several concerns present in the management of these cases. Malignant transformation of the testis is the main risk facing those patients. Few literature studies discussed the risk of changing of these remnants into malignant tissue. Thus discussion with the patient, tissue histopathology, expert opinions, and literature review are the main clues in management of such cases. In addition, it is usually associated with bilateral undescended testes or more seriously testicular tumors of these abdominal testes as the first presentation. It is so uncommon condition as only 260 cases are published in the literature.1 A case of PMDS in an adult male with bilateral undescended testis was encountered by our team and is discussed in this report.PA 26-year-old male patient was referred to our outpatient clinic in Ain Shams University Hospitals concerning about his empty scrotum since birth. The patient had no erectile problems. This was also not associated with any urologic symptoms. There was no family history of relevant condition. There was no other medical or surgical history of relevant importance.No abnormalities were detected except for his hypoplastic scrotum. Otherwise, he had an average build with good secondary male sexual characters. He had normal penile length as regard the age. Urethral meatus showed no abnormalities as regard the site. Neither of the testes could be felt in the scrotum or in the inguinal canal. Laboratory work-up showed mildly elevated FSH 14 mIU/mL , LH 12 nmol/L , testosterone 400 ng/dL , and prolactin 4 ng/dL . Abdominopelvic ultrasonography detected bilateral abdominal testes in the iliac region. Karyotyping was 46 XY.Diagnostic laparoscopy revealed midline abnormal structures in the form of uterus and cervix with two gonads attached to fallopian tube on each side . ConsequThe condition was discussed with the patient. He consented to orchiectomy and excision of all the Müllerian remnants or orchiopexy for early detection of the testicular tumors and keeping parts of the remnants with unsupported evidence for malignant transformation of these remnants. Accordingly, he opted for orchiopexy. The abdominal position of the testes required staged Stephen Fowler operation for the orchiopexy as the gonadal vessels were tethering the testes in their position.In the first stage, with two 10 mm ports and one 5 mm laparoscopic ports, any dissection around the testis was avoided. Clipping of the gonadal vessels was done using two Hem-o-lok polymer ligating clips in each side and testes were left in the abdominal position .Three months later, pelvic duplex ultrasonography was formed before the procedure, which confirmed the adequate vascularity of the testes. The same abdominal access was used as in the first stage. Blunt and cold knife lateral dissection of the testis and the fallopian tubes was done without any distal dissection of the fallopian tube. Dissection of the vas from the fallopian tubes was avoided.Adequate length was gained by this dissection without any manipulation or incision in the uterus.Additional length was obtained with the Prentiss maneuver, in which the testis was passed behind the inferior epigastric vessels, allowing for a more medial position of the cord. A 2-cm external incision in the scrotum was done and another incision in the peritoneum between the bladder and the medial umbilical ligament just lateral to the median umbilical ligament was done. The testis was grasped and replaced in the scrotum without any tension on the cord . The proThe patient was discharged 24 hours later and scrotal duplex 1, 2, and 4 weeks later showed adequate blood supply for both testes in the scrotum. The patient was scheduled for annual monitoring of the testes in the scrotum and the remnants of the Müllerian duct for any malignant transformation1Embryologically, the disappearance of the Müllerian ducts in male in the 7th week of gestational age is closely related to the testicular descent. Thus, the absence of the anti-Müllerian hormone or resistance of one of the receptors will prevent the downward migration of the testis. However, in some cases the testis could be found in other places rather than its abdominal position and that is why Clarnette and coworkers in 1997 divided PMDS into three categories according to testicular position: the majority (60% to 70%) with bilateral intra-abdominal testes in a position analogous to ovaries, a smaller group (20% to 30%) in which one testis is found in a hernial sac, and the smallest group (10%) in which both testes are located in the same hernia sac along with the fallopian tubes and uterus.Consequently, the main challenge that arises in these cases is the orchiopexy procedure owing to the presence of the usual oncologic risk associated with cryptorchidism in this syndrome in addition to the reported cases that had Müllerian remnant tumors. Another concern arising during orchiopexy is the testicular viability through securing adequate blood supply to the testis and preserving the vasa deferentia. Nevertheless, this is not feasible in the PMDS as the vasa deferentia are closely adherent to the uterus and proximal vagina in these cases and the testes are usually tethered by the Müllerian remnants, thus dissecting the vasa from the fallopian tubes may damage it and affect the fertility. By the same concept, when orchiopexy is done only for early detection of testicular tumors in older males, those cases may also require meticulous preservation of the vas especially after the Stephen Fowler procedure as the testis will depend on the artery of the vas for its blood supply. Therefore, preserving the vas is of importance in PMDS cases either for infertility or for vascularity purposes.2Consequently, some authors advocate that midline splitting of the Müllerian remnants and mucosal excision will give more length and allow feasible orchiopexy. Excision of the remnants and effective orchiopexy with delicate dissection of the vasa was reported by few authors. However, testicular atrophy was reported in some of those cases.3According to the limited amount of literature, there is not a standard practice for orchiopexy in PMDS. It could be managed by a single-stage procedure or a two-stage procedure including gonadal biopsies in first stage followed by Müllerian remnants excision or dissection and orchidopexy in the second stage. In contrast, the persistent Müllerian structures can be divided minimally to allow placement of the testis in the scrotum or incised in the midline or even excised totally. At this point, a staged procedure could be considered if manipulation of the Müllerian structures puts the vas in jeopardy, so Fowler Stephens procedure or microvascular auto transplantation will be helpful.PMDS is not a common condition and most urologists will not encounter it during their career. However, a few points could be learned from our case. The release of the fallopian tubes added marvelously to the length of the cord, and midline splitting of the uterus was not required although it was planned based on reviewing the literature. Gonadal biopsies, intraoperative photographs, senior staff consultation, and patient counseling were the corner stones in effective management of this condition.