Fusiform aneurysms in the posterior inferior cerebellar artery (PICA) are rare and challenging to treat. Surgical treatment options for a fusiform aneurysm in the PICA include trapping with/without bypass and wrap-clipping, when elimination of the pathological wall from the systemic circulation and prevention of perforator injury are important. In addition, lower cranial nerve impairment due to surgical manipulation should also be avoided.A fusiform-shaped aneurysm was found in a proximal part of the PICA by magnetic resonance angiography undertaken for evaluation of repeated vertigo in a 36-year-old man. The patient underwent direct surgery via a lateral suboccipital transcondylar fossa approach. The entrance of the pseudolumen was the only part to be wrapped and obstructed by clip application, through the corridor between the acoustic and glossopharyngeal nerves to avoid lower cranial nerve injury.Indocyanine green (ICG) videoangiography demonstrated obliteration of pseudolumen and patency of peripheral PICA and perforator contributing to the medulla oblongata. The postoperative course was uneventful without periprocedural complications, including dysphagia and hoarseness.Partial wrap-clipping technique for obstruction of the entrance into a pseudolumen is one of alternatives for dissecting fusiform-shaped aneurysm in the PICA. ICG videoangiography was helpful to confirm the obliteration of the pseudolumen and patency of parent vessel and perforators. Aneurysms located in the peripheral posterior inferior cerebellar artery (PICA) are rare and account for less than 1% of all intracranial aneurysms . FusiforHerein we present the case of a dissecting fusiform aneurysm in the PICA with tendency for growing. In this case, only the entrance of the pseudolumen separated by the intimal flap was obstructed with the partial wrap-clipping technique in order to avoid lower cranial nerve injury. Indocyanine green (ICG) videoangiography was helpful to confirm the obliteration of the pseudolumen and patency of the parent vessel and perforators.A 36-year-old man presented with a 1-year history of repeated vertigo, when magnetic resonance angiography (MRA) demonstrated an aneurysm originating from the proximal part of right PICA. Follow-up MRA showed an increase in size of the aneurysm after 6 months. Besides MRA, digital subtraction angiography and three-dimensional computed tomographic angiography (3DCTA) indicated that the aneurysm appeared to have an ellipsoid shape with an 8-mm maximum diameter and a distinct neck, and was diagnosed as a congenital berry aneurysm Fig. , which wProximal ligation, trapping, and resection have been reported as curative treatment options for intracranial arterial dissecting or fusiform aneurysms, as well as endovascular internal trapping. Even though these treatments are reasonable for the concept of elimination of pathological vascular walls from the systemic flow, normal blood flow would be consequently sacrificed to require vascular reconstruction. Even if that were the case, the danger of perforating arterial injury would remain.The wrap-clipping method has been established as one of treatment options for arterial dissection and blood blister aneurysm (BBA), which can provide reinforcement of holding the applied clip to result in prevention of re-rupture and enlargement of the pathological wall –3. This A variety of materials have been reported for wrapping, including muscle fascia, cotton, Goatex, and Dacron , 3. For A dissecting aneurysm occasionally has a fusiform shape besides a tapered occlusion and pearl and string signs. On cerebral angiography, some fusiform aneurysms show the intimal flap itself, like in our case, as well as a double lumen, pooling, and/or slow filling along the vessel, as an evidence of dissection. However, many of them do not reveal any apparent findings supporting a dissecting nature. For precise diagnosis of fusiform aneurysm, not only catheter angiography and 3DCTA that mainly provide information regarding the intra-vascular space but also MRI are important for depicting the character of the wall and thrombus inside the aneurysm. Basi-parallel anatomical scanning (BPAS) MRI has been reported to be useful for diagnosis of vertebral artery dissection . MRI-BPAIn this paper, only the entrance of the pseudolumen was obliterated with partial clip application Fig. . MeticulThe wrap-clip technique for obliteration of only the entrance into a pseudolumen might be one of the alternatives for treating a dissecting fusiform-shaped aneurysm, which was effective for avoidance of lower cranial nerve injury in the case of the proximal part of the PICA. ICG videoangiography played an extremely important role in confirmation of obliteration of the pseudolumen and patency of the parent vessel and perforators.


Primary orbital peripheral T-cell lymphoma, not otherwise specified is an exceedingly rare disorder with a very poor outcome, and to the best of our knowledge only a few cases have been reported in the English literature. We present the youngest reported case describing the successful outcome after management with a thorough review of the English literature of all the reported cases of primary peripheral T-cell lymphoma, not otherwise specified.Our patient is a 3-year-old Syrian boy who presented with gradual progressive orbital swelling. A physical examination showed a left orbital dystopia and a superior medial displacement of the globe. Extraocular motility was limited in upward elevation of his left eye. A computed tomography scan and magnetic resonance imaging of his orbit showed a mass involving the lateral and inferior walls of his left orbit and extending intraconally. A diagnosis of peripheral T-cell lymphoma, not otherwise specified was made by careful histopathological examination and Berlin-Frankfurt-Munster protocol was initiated. A 6-month follow up with orbital magnetic resonance imaging showed no sign of orbital or brain involvement.Through this report we emphasize two takeaway lessons: (1) always have a high level of suspicion of this entity regardless of the age of the patient; and (2) careful histopathological examination is very important for prompt confirmation of the diagnosis and early commencement of proper treatment.The online version of this article (10.1186/s13256-018-1630-2) contains supplementary material, which is available to authorized users. In this case report we describe the youngest patient to have a rare type of lymphoma occurring in the orbit recorded in the English literature. We discuss the challenging histopathological diagnosis, the importance of ophthalmologists’ awareness to include this entity in their differential diagnosis, and the promising resolution with proper treatment.et al. involving a 6-year-old boy [et al. (CARE = case report) [Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a subtype of the aggressive group of peripheral T-cell lymphomas (PTCLs), which are derived from various types of mature T-cells, that do not meet the criteria for the other specifically defined subtypes of PTCL . Primary-old boy –6. We pr report) .The parents of a 3-year-old Syrian boy noticed that he had gradual progressive painless swelling of his left lower eyelid and ptosis over the course of 2 months that significantly increased over the last 2 weeks. There was no significant loss of vision. Medically, the parents recalled loss of appetite of 1-week duration, with no history of trauma, other systemic manifestations, or past surgery. He had an unremarkable family history.An ophthalmological examination revealed left orbital dystopia and superomedial displacement of the globe. Extraocular motility was limited in upward elevation of his left eye. His left upper lid seemed to be drooping; however, it proved to be pseudoptosis, which was caused by the upward displacement of the globe due to the mass effect. Correspondingly, the palpebral fissure measurement in his left eye was 5 mm, while it measured 9 mm in his right eye Fig. . FurtherA computed tomography (CT) scan revealed a 9.5 mm osseous destruction at the left inferior lateral orbital margin that was associated with a large soft tissue mass measuring 1.9 × 2.1 × 1.9 cm. The mass extended into his left orbital cavity. A small soft tissue component measuring 7.8 × 7.8 × 9.7 mm was also seen extending toward the subcutaneous zygomatic region. Magnetic resonance imaging (MRI) of his orbits showed a solid enhancing mass lesion, involving the lateral and inferior walls of his left orbit, and extending into the intraconal compartment. The mass was displacing the globe superiorly and medially with medial displacement of the left inferior rectus muscle Fig. . His optAn incisional biopsy under general anesthesia was performed and a 2.1 × 1.5 × 1.0 cm tissue sample was obtained. Histological slides showed diffuse effacement atypical cells of variable size infiltrating the bone Fig. . The proSerology for Epstein–Barr virus (EBV), cytomegalovirus, and human immunodeficiency virus were negative. A systemic evaluation including: CT of his chest, abdomen, and pelvis; bone marrow biopsy; and physical examination of the ear, nose, and throat, were normal. A cardiac function assessment as well as a complete body gallium scan were performed and were negative for metastasis. His peripheral blood workup was within normal limits. He was a high risk and was started on Berlin-Frankfurt-Munster protocol, with 2 weeks on and 2 weeks off chemotherapy. The protocol includes the following agents: cytarabine (ARA-C), L-asparaginase (ASP), cyclophosphamide (CTX), dexamethasone (DEX), doxorubicin (DXR), mercaptopurine (6-MP), methotrexate (MTX), prednisone (PDN), 6-thioguanine (6-TP), and vincristine (VCR). However, the doses and details of administration are beyond the scope of this case report since we are highlighting the challenge and the importance of accurately diagnosing such rare cases. He was followed up every month for 6 months. He tolerated the chemotherapy well according to his parents and his oncologist. He showed remarkable response to chemotherapy with resolution of his signs and symptoms Fig. . MRI of Orbital and ocular adnexal lymphoid neoplasms are not scarce, representing 6 to 8% of all orbital tumors, whereas out of the extranodal non-Hodgkin’s lymphomas, primary non-Hodgkin’s lymphoma of the orbit represented 8 to 10% . Based oet al. published an extensive review of 340 cases to better understand PTCL-NOS [According to the World Health Organization (WHO) classification of lymphoid neoplasms, the diagnosis of PTCL-NOS requires the integration of morphology, aberrant T-cell phenotype, and clonally rearranged TCR genes ; having PTCL-NOS . The 340PTCL-NOS . To obsePTCL-NOS . The medPTCL-NOS . If we aPTCL-NOS . The celPTCL-NOS . In EuroPTCL-NOS , 14.In the review of the five reports, not all the cases seem to have a detailed immunohistochemistry. Having said that, based on the previous five authors’ reports, the IHC staining in most of the cases proved T-cell type of lymphoma where tumor cells expressed T-cell markers CD3, CD4, and CD8. One case showed a drop-off of CD5 and CD7. On the other hand, in almost all cases reported, the cells failed to express CD30 and CD56 markers that aid in the diagnosis of ALCL and NK cell lymphoma, respectively. Only four cases had PCR showing TCR rearrangement.Our case of a 3-year-old child diagnosed with primary orbital PTCL is unusual in the sense that he is the youngest case of primary PTCL-NOS of the orbit ever reported to the best of our knowledge. The presentation of our patient with pseudoptosis was also unusual. The consistency of a hard mass clinically and the appearance of the lesion by imaging gave an impression of a bony origin, such as Ewing sarcoma. However, histopathology revealed PTCL-NOS features. The IHC stains showed the expression of the tumor cells for CD30, CD56, and ALK were negative excluding the diagnosis of ALCL and NK cell lymphoma. In addition, the findings of TCR rearrangements on PCR did confirm our diagnosis of PTCL-NOS despite being challenging and confusing. The overall 5-year survival has been reported to be as low as 32%. Significant numbers of EBV-positive B cells, CD56 expression, and CD30 expression by more than 20% of the tumor cells are bad prognostic factors . FortunaIn conclusion, we have presented the youngest case of proven PTCL-NOS ever reported in the English literature. Our case was unusual in presentation and imaging features; thus, the diagnosis was totally unexpected. The early accurate diagnosis allowed for proper promising treatment. Therefore, we strongly recommend the following: (1) a high level of suspicion of such entities regardless of the age or presentation; and (2) careful histopathological examination and proper tissue diagnosis by an experienced pathologist, which is critical in confirming the diagnosis for early intervention.Additional file 1:Timeline of the patient's clinical history starting from the date of presentation to the date of last follow up. (JPEG 172 kb)


Complete small bowel obstruction (SBO) is a common surgical emergency often resulting from adhesive bands that form following iatrogenic peritoneal injury. Rarely, adhesive SBO may arise without previous intra-abdominal surgery through other modes of peritoneal trauma. We present the case of a male evaluated in the emergency department for a closed-loop small bowel obstruction due to an adhesive band that likely formed after blunt abdominal trauma over two decades earlier. We review the epidemiology, pathophysiology, and treatment options for similar cases of adhesive SBO. Small bowel obstruction (SBO) is a common clinical entity in emergency medicine and globally remains an important source of morbidity and mortality. Approximately 65–75% of SBOs are due to peritoneal adhesions, aberrant fibrous bands within the abdominal cavity that constrict the intestine and disrupt its luminal flow.A 37-year-old male was evaluated in our emergency department for a 16-hour history of constant, cramping epigastric abdominal pain and nausea. His past medical history was significant for a pelvic fracture, suffered during a motor vehicle collision (MVC) more than 20 years earlier, which required open reduction and internal fixation at the pubic symphysis. The patient reported no previous abdominal surgeries, recent abdominal injury, or history of acute or chronic intra-abdominal inflammation other than that likely associated with his remote abdominal trauma. He was otherwise in good health and did not use any regular medications.9/liter, normal 4.0–10.0×109/liter) and neutrophil fraction . An abdomino-pelvic computed tomography (CT) with intravenous (IV) contrast revealed a distended stomach and several dilated loops of small bowel in the mid-abdomen, with multiple air-fluid levels and gradual decompression proximally and distally  is a common surgical emergency often caused by adhesive bands that form after abdominal surgery or inflammatory disease.What makes this presentation of disease reportable?Our patient had no such history, and therefore the adhesive SBO likely resulted from blunt abdominal trauma sustained several decades prior.What is the major learning point?It is critical to obtain a thorough history of abdominal trauma from patients presenting with symptoms of obstruction yet lacking these common risk factors.How might this improve emergency medicine practice?Delaying surgery for complicated SBO increases its mortality; thus, recognizing unusual causes of obstruction may expedite intervention and improve patient outcomes.There was no evidence of ischemia, but several bowel loops had inflammatory stranding and surrounding free fluid. The patient received IV isotonic fluid resuscitation and, following admission to the hospital, subsequently developed peritoneal signs. Due to concern for acute SBO with peritonitis, the patient underwent exploratory laparotomy later that day. He was found to have a fibrous band extending from the greater omentum to the jejunum, through which a 20 centimeter segment of decompressed bowel formed a closed-loop obstruction around which the jejunum was wrapped. After the entire length of the intestine was inspected and its viability was confirmed, the omental adhesion was divided and the bowel was released.The patient’s post-operative course was complicated by mild colonic ileus and a cellulitic incisional infection that resolved following a course of cephalexin. At follow-up on post-operative day 12, he was asymptomatic and had a normal physical examination.Adhesions are the most common cause of SBO and thus create a substantial burden for patients and healthcare systems.4Our patient’s omental band adhesion was not likely the result of his prior pelvic surgery. The reduction and internal fixation of his pubic symphysis was entirely extraperitoneal and there was no evidence of screws transecting the peritoneum. Instead, we attribute his adhesive SBO to blunt abdominal trauma sustained during a remote MVC. Approximately 3–5% of patients treated for blunt abdominal trauma, commonly related to seatbelt use during MVCs, receive hollow viscus and mesenteric injuries.9Adhesions that are not broken down will mature within 10–14 days., with symptoms including colicky abdominal pain, nausea, vomiting, abdominal distension, and obstipation.11Adhesive SBO often presents similarly to other acute abdominal diseasesHowever, CT of the abdomen is more useful for determining the location and etiology of a SBO, and therefore may be used instead of radiographs when this diagnosis is strongly suspected.14Historically, patients suspected of having SBO secondary to adhesions underwent surgery immediately due to the uncertainty of strangulation.16Early identification and treatment is particularly important in the case of a closed-loop obstruction, which can quickly progress to strangulation, ischemia, and necrosis.Our patient’s clinical presentation was consistent with a SBO, including symptoms of nausea, abdominal tenderness, and dehydration, yet his medical history contained no commonly identified risk factors for adhesions. We followed our institution’s standardized protocol by performing volume resuscitation and open surgery immediately after obtaining CT imaging findings consistent with obstruction and with the development of peritoneal signs consistent with bowel compromise.16Complete adhesive SBO is a common surgical emergency that requires rapid diagnosis to minimize complications. Patients presenting with adhesive SBO may rarely have no history of abdominal surgery or inflammatory disease, and thus may pose diagnostic uncertainty. Blunt trauma can create intra-abdominal adhesions, yet when old or trivial, may not be recognized as the cause. It is therefore imperative to take a thorough history of abdominal trauma from patients who present with symptoms consistent with obstruction.


Clear cell papillary renal cell carcinoma (CCPRCC) is a newly recognized entity in the 2016 WHO classification and usually presents as a small, circumscribed, solitary mass of indolent nature. CCPRCCs could seldom occur in conjunction with other synchronous or metachronous kidney tumors and even less frequently as bilateral masses. To our knowledge, multiple bilateral CCPRCCs have never been described with the existence of a synchronous well-differentiated neuroendocrine tumor of the kidney and hence reported here as a unique case. This case report highlights the importance in considering this entity and its unusual presentation in the differential diagnosis as a possible mimicker of Von Hippel-Lindau syndrome. A 55-year-old male with a notable history of diabetes, hypertension, obesity, and chronic kidney disease was found to have bilateral kidney masses while undergoing ultrasound for evaluation of recurrent kidney infections. He was eventually referred to our institution. Computed Tomography (CT) at that time showed a left upper pole 3.2 cm mass, left lower pole 1.4 cm mass, a right mid-kidney 6.6 cm mass, and an upper pole 2.1 cm mass  which weResections from the left kidney were performed. The upper pole, lower pole, and the large mid-portion to lower pole masses were removed. All resections showed a histologically typical CCPRCC with a background of simple renal cysts and moderate global glomerular sclerosis, hyperplastic arteriolosclerosis, and lymphoplasmacytic interstitial nephritis. Von Hippel-Lindau (VHL) gene, including mutations, methylation abnormalities, and LOH3p, were identified by genetic testing at an outside institution. Just adjacent to the CCPRCC, a microscopically separate 1.5 cm well-differentiated neuroendocrine tumor was identified focally invading the hilar fat  and does not require dialysis.β-catenin and may play a role in CCPRCCs indolent nature [CCPRCC was described less than a decade ago  as a dist nature . Interest nature . In addit nature –14. The Though first described in patients with end stage renal disease , the maj VHL mutations and the classification of such CCPRCC cases with VHL mutations is questionable [Awareness of this newly described combination of CCPRCC and neuroendocrine tumors is critical to consider in the differential diagnosis of VHL syndrome. VHL syndrome can present with multiple clear cell renal cell carcinomas which can mimic CCPRCC morphologically and grossly. However, these tumors differ in their molecular and immunohistochemical profiles . In additionable , 25–27.Primary renal carcinoid tumors are extremely rare. Peak incidence is the fifth and sixth decades of life with a slight female predominance. Renal carcinoids have been associated with horseshoe kidneys and polycystic kidney disease and have been described arising from teratomas, including one case report of a reported adenocarcinoma and a carcinoid arising within a teratoma in a horseshoe kidney , 29. TheCCPRCC has never before been reported to coexist with a primary renal carcinoid tumor much less with bilateral multiple CCPRCCs. Though CCPRCCs have an indolent course, multifocality and synchronous tumors of various types, including well-differentiated neuroendocrine tumors, as described here, must be considered in the prognosis and raise the concern of possible syndromic associations. Further studies are needed to better understand these neoplasms, their interaction, and their underlying mechanisms.This is an unprecedented case of a primary renal carcinoid with simultaneous multifocal bilateral CCPRCCs. The presence of concurrent multiple tumors, besides their negative impact on residual kidney function, should be considered in the diagnosis in the otherwise indolent prognosis of CCPRCC. This case report highlights the importance in considering this entity and its unusual presentation in the differential diagnosis as a possible mimicker of Von Hippel-Lindau syndrome.


Ovarian immature teratoma is a germ cell tumor that comprises less than 1% of ovarian cancers and is treated with surgical debulking and chemotherapy depending on stage. Growing teratoma syndrome (GTS) is the phenomenon of the growth of mature teratoma elements with normal tumor markers during or following chemotherapy for treatment of a malignant germ cell tumor. These tumors are associated with significant morbidity and mortality due to invasive and compressive growth as well as potential for malignant transformation. Current treatment modality is surgical resection. We discuss a 12-year-old female who presented following resection of a pure ovarian immature teratoma . Following chemotherapy and resection of a pelvic/liver recurrence demonstrating mature teratoma, she underwent molecular genetics based chemotherapeutic treatment. No standardized management protocol has been established for the treatment of GTS. The effect of chemotherapeutic agents for decreasing the volume of and prevention of expansion is unknown. We review in detail the history, diagnostic algorithm, and previous reported pediatric cases as well as treatment options for pediatric patients with GTS. The incidence of GTS is 12% of ovarian germ cell tumors (GCT) occurring in young adults and adolescents [Growing teratoma syndrome (GTS) is thought to be the progression of immature to mature teratoma during or following chemotherapy. GTS requires three criteria: normalization of previously elevated tumor markers . Repeat CT of chest, abdomen, and pelvis revealed that, in the abdomen and pelvis, she now had enlarged peritoneal implants, as large as 3.5 × 1.5 cm, but no other masses, and, in the chest, she had a 1.3 × 2 × 0.3 cm left anterior pleural nodule, as well as bilateral diaphragmatic lesions, 2.5 × 2.1 × 1.8 cm on right and 2.1 × 3 × 1.5 cm on left. She subsequently underwent chemotherapy with bleomycin, etoposide, and cisplatin (BEP) between February and May 2016 for high risk malignant ovarian germ cell tumor. After 4 cycles, her AFP was 0.84 ng/mL.A 12-year-old female patient presented from a referring institution in November 2015 for evaluation of 1-month history of an enlarging abdominal mass. The patient reported a 4.5 kg weight loss with increased lethargy. The physical examination was normal except for a large abdominal solid mass. Pelvic CT  revealedteratoma . She wasAfter 4 cycles, she underwent interval radiologic evaluation in preparation for second look surgery. The CT scan of the chest, abdomen, and pelvis in May 2016 revealed a significant increase in the size of the thoracic masses, as well as the size and a number of abdominal and pelvic masses . The lar2 PO daily (days 1–28), tretinoin 25 mg/m2/day PO BID (days 1–14), and sorafenib 150 mg/m2 PO BID (days 1–28). Thalidomide was started during cycle 2. This protocol was chosen as this study tests the feasibility of experimental technologies to determine a tumor's molecular makeup. This technology includes a genomic report based on DNA exomes and RNA sequencing that will be used to discover new ways to understand cancers and potentially predict the best treatments for patients with cancer in the future. Prior to the experimental therapeutics, a CT scan was obtained to evaluate disease progression. She is currently without new tumor growths.Due to the massive growth of tumor in 4 months, as well as recently undergoing 2 major surgical debulkings, and still being unable to completely resect her tumors, medical treatment was discussed with the patient and her parents. In an attempt to prevent regrowth of her tumors, in August 2016, the patient was started on experimental protocol #009 of the Neuroblastoma and Medulloblastoma Translational Research Consortium (NMTRC). This treatment regimen consists of temozolomide 40 mg/mp = 0.005) [Immature ovarian teratoma is a rare germ cell neoplasm that comprises <1% of ovarian teratomas . The imm= 0.005) . The ove= 0.005) . Similar= 0.005) , 76 patiGTS was first introduced by DiSia et al. in 1977 and was called “chemotherapeutic retroconversion” (CR)  while Lon = 56) [3/month [Ovarian GTS has been described in 101 published English literature cases , 16. Mosn = 56) . Many can = 56) , 17, 18.[3/month , 20.Recurrent tumors following chemotherapy for germ cell tumors should be resected to confirm diagnosis, relieve impending/possible obstruction, and prevent future malignant transformation . GTS hasGTS histology is comprised of mature teratoma elements, but morbidity and mortality arise from its local expansion and the potential for malignant degeneration , 22, 23.A rare form of tumor spread can be associated with GTS known as Gliomatosis Peritonei (GP). This tissue is composed of mature glial tissue and can be located in the peritoneal cavity and omentum in patients with ovarian teratoma. GP is often mixed with GTS series despite this being a separate entity  because No standardized management protocol has been established, but one has been set out by Byrd et al. , 37 Fig. A key pAdjuvant chemotherapy with bleomycin, etopside, and cisplatin is recommended for patients when diagnosed with immature teratoma following primary surgery. Palbociclib, a CDK4/6 inhibitor (PD0332991), is reported that it can stabilize the vascularization of the tumor in pediatric patients with an intracranial teratoma . It is aSurgical approaches stress fertility sparing surgery as recommended for women of childbearing age. Successful pregnancy following development of GTS has been reported indicating necessity of fertility sparing approaches . The treIn the setting of immature ovarian teratoma treated with chemotherapy, a rapid growth rate in the presence of normalized serum tumor markers should raise suspicion of GTS . No effe


We have discussed a unique presentation of primary diffuse large cell B-cell non-Hodgkin (DLBC NHL) hepatic lymphoma involving the porta hepatis and biliary confluence causing obstructive jaundice with contiguous soft tissue involvement of the right lobe of liver extending up to the right renal cortex. This appears to be the only case in literature where primary hepatic lymphoma has shown contiguous localized intra- and extrahepatic tumor infiltration. A 67-year-old gentleman presented with history of significant loss of appetite and weight in 2 months with associated progressive painless cholestatic jaundice. Physical evaluation revealed normal vitals with pallor, deep icterus, scratch marks over the abdomen, generalized muscle wasting, grade II clubbing and a palpable non-tender liver with a globular, firm mass beneath the liver. He had a total serum bilirubin of 15.9 mg/dL and direct bilirubin of 9.24 mg/dL. His liver enzymes were moderately elevated with raised serum creatinine and dyselectrolytemia. Serology for enterohepatic viruses was negative. Contrast-enhanced magnetic resonance imaging (CEMRI) showed poorly enhancing multiple soft tissue masses in both lobes of liver with the largest mass involving, biliary confluence and porta hepatis causing right bile duct and portal vein encasement. The mass occupied the posterior right lobe and extended to the inferior surface of liver with contiguous invasion of the right renal upper pole cortex. The mass was associated with a retracted liver capsule in the involved segments and delayed enhancement, mimicking a cholangiocarcinoma. Tissue biopsy revealed hepatic DLBC type NHL and patient was subsequently treated with a CHOP-R (cyclophosphamide-doxorubicin-vincristine-prednisolone/rituximab) regimen, on which he has shown non-progressive disease at 1-year follow-up. DLBC NHL of the liver is a very rare tumor with propensity for isolated involvement of the liver and minimal extrahepatic spread. This case shows many interesting features such as obstructive jaundice for 2 months, porta hepatis involvement and tumor infiltration up to the right renal parenchyma. We have illustrated various imaging findings which should be considered when evaluating such a lesion to help differentiate it from cholangiocarcinoma. The literature is extensively reviewed. The case demonstrates relevant diagnostic parameters for physicians, radiologists and oncologists who are likely to encounter patients with tumor-induced obstructive jaundice in their daily practice. Lymphoma is a systemic lymphoproliferative malignancy and is classified into the Hodgkin and non-Hodgkin lymphoma (NHL) subtypes. NHL originates from nodal and extra nodal sites. Primary hepatic lymphoma (PHL) is a very rare subgroup of extra-nodal NHL because of a natural lack of abundant lymphoid tissue in the liver. It is seen in less than 1% of NHL . PHL wasThe diagnostic criterion for PHL is a lesion confined to the liver without spleen, bone marrow or hematological involvement and lack of superficial lymphadenopathy at the time of presentation as well as up to the next 6 months . The criDue to the stringent and well-defined diagnostic guidelines of this group of lymphomas, only few hundred isolated case reports have been described in literature so far. Most of these tumors present as insidious, asymptomatic lesions which are rarely suspected at the time of evaluation and are a histological surprise . The speThe etio-pathogenesis of PHL is unclear; however, few associated factors have been suggested. These include: prior infection with hepatitis B virus (HBV), hepatitis C virus (HCV), Epstein-Barr virus (EBV), cirrhosis due to primary biliary or secondary to viral infections, auto-immune diseases and immunosuppressive therapy , 9. It hAlthough there are few case reports of surgically treated solitary focal liver lesions in PHL, the universally accepted non-surgical treatment consists of cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab (CHOP-R) regimen, which is the mainstay of therapy for PHL patients.We present a unique case of NHL with obstructive jaundice involving the liver as the primary site. We believe that the soft tissue extension of the tumor to the right kidney along with the other imaging and clinical features makes this a rare case report which is worthy of attention to all physicians and clinicians who observe similar cases in their daily practice.A 67-year-old gentleman presented with history of significant loss of appetite and weight (approximately 42 pounds) in 2 months with associated progressive painless cholestatic jaundice. He also complained of extreme generalized weakness and fatigue. The patient denied any febrile episodes or bleeding manifestations during this period. There was no history of abdominal pain, distension or fullness. No symptoms of altered sensorium leg and face swelling or decreased urine output were seen. Patient denied chronic, addictive substance abuse or use of any indigenous medication during and prior to his illness period. The patient had never undergone surgery or blood transfusions in the past. Physical evaluation revealed the patient was conscious, oriented and afebrile with normal vitals. Pallor was evident and the patient was deeply icteric with evidence of scratch marks over the abdomen. There was generalized muscle wasting and loss of subcutaneous fat over the face and hollowing of the temporal regions. There was presence of grade II clubbing. No evidence of cyanosis or peripheral edema was seen. No peripheral, mediastinal or abdominal adenopathy was found. The abdomen was non-distended without visible venous prominences or visible pulsations. Palpation revealed a firm, rounded, smooth non-tender liver palpable up to 6 cm below the right costal margin. A large globular non-tender firm mass of about 8 cm was palpable beneath the liver. There was no splenomegaly or free fluid. The rest of the systemic examination was normal and non-contributory. His laboratory investigations are shown in Serology for hepatitis A, E, B, and C viruses as well as for HIV was negative. Tumor markers such as carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) were normal. Serum CA19-9 was elevated. Contrast-enhanced magnetic resonance imaging (CEMRI) using hepatocyte specific contrast gadobenate dimeglumine (gadolinium BOPTA) was performed at our institute which revealed the presence of multiple T1-hypointense and T2-hyperintense intrahepatic (entire right lobe and segment-IV of the left lobe) lesions showing restriction on diffusion weighted images . On contFig. 7-Differential diagnosis on imaging as well as on pathology which was ruled out at tissue biopsy is as follows: 1) hepatocellular carcinoma; 2) cholangiocarcinoma; 3) metastatic carcinoma; 4) metastatic neuroendocrine carcinoma; 5) malignant lymphoma .He was subsequently treated with a CHOP-R regimen, on which he is doing well at 1-year follow-up.PHL is defined as tumor confined to the liver with no evidence of lymphomatous or hematological involvement of other organs in the body. It is rare to find NHL presenting with obstructive jaundice. The patient in our study is of interest because of the rarity of combination of PHL presenting as malignant biliary obstruction. This particular tumor combined multiple interesting features such as multiple hepatic lesions, involvement of porta hepatis, portal vein encasement and biliary obstruction. However the most noteworthy feature that was confounding in its diagnosis was, the fact that the tumor extended to the right renal parenchyma directly from the right lobe of liver crossing the hepatic capsule and Morrison’s pouch fat. This is probably the first case in literature which exhibited such findings. Literature review of published case reports of DLBCL of the liver reveals that presentation of these tumors is an amalgam of various entities . The medLymphomatous liver lesions may be incidentally diagnosed on imaging of clinically asymptomatic patients or on evaluation of terminally sick and morbid cases of fulminant hepatic failure . AbdominOur report extends the range of clinical manifestations from solitary, multiple or diffuse lesions to a locally infiltrating tumor extending beyond the liver capsule. Recognition of such an entity is important for the sake of both diagnosis and management of PHL presenting as obstructive jaundice with extent beyond the liver. Prognosis remains favorable in appropriately treated cases.DLBC PHL of the liver is a rare diagnosis on imaging. This case illustrates the importance of imaging and histopathology correlation in patients presenting with malignant obstructive jaundice. The above case has demonstrated a unique presentation of PHL with biliary, portal vein, perihepatic fat planes involvement as well as contiguous extension of tumor tissue into the right kidney. This report will help clinicians, radiologists, oncologists and pathologists to consider this rare tumor as a differential diagnosis for cholangiocarcinoma as well as a possible cause for malignant obstructive jaundice which can be effectively treated with non-surgical means, offering a favorable prognosis.


She underwent a total laparoscopic hysterectomy with bilateral salpingectomy. The histologic evaluation of the specimen showed a follicular lymphoma with diffuse pattern in the endometrium. This report illustrates the difficulty in the diagnosis of primary lymphomas of the female genital tract.Primary non-Hodgkin's lymphoma (NHL) can arise from lymphatic cells located in solid organs  and it accounts for 25 to 35% of all NHL. Primary lymphoma on the female genital tract (PLFGT) is a rare disease, comprising 0.2 to 1.1% of all extranodal lymphomas in the female population. In this paper, the authors report an extremely rare case of a 48-year-old woman who exhibited an abnormal uterine bleeding, pelvic pain, and dysmenorrhea history. The transvaginal ultrasound showed an anteverted uterus measuring 153 cm Primary non-Hodgkin's lymphoma (NHL) may arise from lymphatic cells located in solid organs  and it accounts for 25 to 35% of all NHL –4. Most The main histological subtypes of non-Hodgkin's lymphoma are the diffuse large B-cell lymphoma, follicular lymphoma, and Burkitt lymphoma –11. ImmuThe ovaries are the most common site of PLFGT, followed by the uterine cervix and the uterine body, which is very rare , 15. UsuA 48-year-old woman, G4 P2 C2, came to our service complaining about abnormal uterine bleeding, pelvic pain, and dysmenorrhea for the last 6 months. She was under clinical treatment for the uterine bleeding with continuous use of hormonal contraceptive (Gestodene 75 mcg + Ethinylestradiol 30 mcg). She had a past surgical history of cholecystectomy, cesarean section, abdominoplasty, and bilateral tubal ligation. The gynecological examination was unremarkable.3, with a heterogeneous pattern, with intramural leiomyomas at the posterior uterine wall. Pap smear was negative for neoplasia.On bimanual examination, the uterus was mobile and had a normal size. There was no increase in the volume of the ovaries. Transvaginal ultrasound showed an anteverted uterus, measuring 153 cmShe underwent a laparoscopic hysterectomy with bilateral salpingectomy for a presumed abnormal uterine bleeding and leiomyomas not responding to clinical treatment. The laparoscopic hysterectomy was performed according to the previously reported surgical technique . The surAfter clinical staging, the disease was diagnosed as follicular lymphoma of the endometrium stage IV, due to the involvement of the bone marrow. The patient was submitted to chemotherapy with six cycles of R-CHOP , after surgery. Follow-up PET/CT was made sixteen months after surgery and demonstrated increased activity in the retroperitoneum, and it was decided to maintain rituximab for 2 more years.Lymphomas are malignant tumors that affect the immune system, most commonly, the lymph nodes. However, 40% of the lymphomas are extra nodal –20. WhenIt usually occurs in women during the fifth decade of life. Nevertheless, it depends on the histological subtype; as an example, diffuse large B-cell lymphoma is more common between 35 and 45 years old, whereas follicular lymphoma is more frequent in people aged over 50, and Burkitt lymphoma affects children from five to ten years old .The diagnosis is very difficult because of the rarity of this entity. In addition, there is no typical presentation of the symptoms; usually, it depends on the site where the cancer is confined . Some ofSince clinical evaluation and imaging studies cannot give a definitive diagnosis, most PLFGT is initially treated as being any other common gynecologic malignancy. The definitive diagnosis is obtained after surgery during the pathological examination of the surgical specimen . ImmunohThe main histological subtype of non-Hodgkin's lymphoma is the diffuse large B-cell lymphoma , 11. TheIn this case, the patient presented no specific symptoms. She underwent a total laparoscopic hysterectomy for a presumed benign disease (leiomyoma and increased uterine bleeding). The diagnosis of follicular lymphoma with diffuse pattern coming from the endometrium was confirmed after anatomopathological and immunohistochemical evaluations.The treatment of PLFGT consists of a multimodal approach, including the gynecologist, the clinical oncologist, and the radiation oncologist, trying to individualize each treatment. As this type of lymphoma often compromises premenopausal women, it is very important to consider the patient's fertility. Chemotherapy induces irreversible damage to the ovarian tissue, which leads to premature ovarian failure. This is an important issue that must be discussed with the patient, and the possibility of oocyte or embryo cryopreservation must be remembered in those patients who desire future childbearing . TemporaR-CHOP regimen is the standard treatment for follicular lymphoma and includes rituximab plus cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine), and prednisone or prednisolone. The cyclophosphamide is considered a high-risk gonadotoxic drug, oncovin a medium risk .In the posttreatment follow-up, tumor remission is usually evaluated by PET-CT (positron emission tomography-computed tomography) .PLFGT is a rare condition. Different clinical presentations may lead to a difficult diagnosis, frequently misunderstood during the preoperative setting. Due to the scarce number of cases, there is no formal protocol for the management of PLFGT. Although rare, PLFGT should be considered in the differential diagnosis of organic diseases, leading to increased uterine bleeding and pelvic pain.


Hepatic cystadenoma is a rare benign cystic tumor; it tends to recur after incomplete surgical resection and has malignant potential. We report the case of a patient with a ruptured biliary cystadenoma in the common bile duct that caused diagnostic and therapeutic problems.A 34-year-old North African woman, admitted for angiocholitis, was operated 2 months before for a hepatic cystic lesion taken for a hydatid cyst compressing her common bile duct. The clinical and the complementary examinations converged toward recurrence of the hydatid cyst for which a surgical resection was decided. Intraoperative findings as well as the histological study of the “membranes” extracted from her common bile duct indicated a hepatic cystadenoma.The rarity of hepatic cystadenoma and the non-specificity of clinical and imaging signs make diagnosis of hepatic cystadenoma difficult, especially when it is complicated by rupture in the bile ducts; this contributes to a delay in diagnosis and an inadequate therapeutic approach. Hepatic cystadenoma is a rare benign cystic tumor, which most often arises from the intrahepatic biliary system; it is characterized by the risk of recurrence, in cases of incomplete resection, and malignant transformation. A lack of clinical and biological specificities makes preoperative diagnosis difficult, posing a differential diagnosis problem with other cystic lesions. Its evolution can be marked by complications; the most serious complication is a malignant transformation into cystadenocarcinoma, the risk of which is estimated to be 10 to 20% [A 34-year-old North African woman with no specific past medical history was hospitalized, initially in another hospital, for the management of obstructive jaundice with a hepatic cystic lesion of segment I taken for a hydatid cyst that compressed the biliary convergence. She had undergone a resection of the protruding dome by laparotomy. Two months later, she was admitted for angiocholitis due to a probable recurrence of the hydatid cyst complicated by compression or rupture in the bile ducts. She was febrile 38.5 °C with mucocutaneous jaundice, dark urine, and discolored stools. A clinical examination found neither defense nor hepatomegaly or palpable mass.Biological examinations revealed moderate cytolysis and cholestasis. Hydatid serology and serum tumor markers were negative: carcinoembryonic antigen (CEA) and carbohydrate antigen (CA).A computed tomography (CT) scan showed a hepatic cystic lesion of segment I, with parietal and septal enhancement after agent contrast injection; this lesion was ruptured in the biliary convergence.Magnetic resonance imaging (MRI) showed a partitioned lesion, hypointense in T1 and hyperintense in T2, ruptured in the biliary bifurcation and causing dilatation of intrahepatic bile ducts, especially on the left side; the common bile duct (CBD) was also dilated with heterogeneous signal content Figs.  and 4. AThree months after this second surgery, we decided to perform radical excision after regression of jaundice and amelioration of blood tests. In fact, she underwent a left hepatectomy enlarged to segment I by laparotomy, because the cyst was ruptured in the posterior wall of the left hepatic canal, with large fistula, near the biliary convergence; a simple enucleation was impossible due to posterior location of the cyst and the adhesions of anterior surgeries. Her postoperative follow-up was simple and a histological study of the specimen confirmed the diagnosis of biliary cystadenoma without signs of malignancy.Hepatic cystadenomas are extremely rare, accounting for less than 5% of non-parasitic hepatic cystic lesions . The meaCystadenomas occur in the liver in 90% of cases; they are much less frequent in the extrahepatic bile ducts. Concurrent intrahepatic and extrahepatic cases have also been reported . They arThey are generally asymptomatic, in the patent forms, and apart from the complications the clinical signs are not specific: hepatic colic or vague abdominal pain. In addition, a large tumor can cause a mass effect on the stomach or duodenum causing nausea, vomiting, dyspepsia, and anorexia. Abdominal palpation may reveal a moving mass with respiration.Many complications can occur during evolution, including biliary obstruction that may be due to external biliary compression or internal obstruction by a protrusion of the cyst within the bile duct, but more rarely secondary to mucous plugs hindering bile flow; secondary biliary cirrhosis is likely to be the consequence of prolonged obstruction of the CBD.Biology is usually normal; an increase in the activity of gamma-glutamyltransferase (GGT) and alkaline phosphatases can occur in cases of compression or obstruction of the biliary tract, leukocytosis can also be found in cases of cyst’s superinfection or angiocholitis.In our patient, biology noted cholestasis and moderate cytolysis; the latter can be explained by the association with biliary cirrhosis.Serum CA 19-9 analysis may be useful, but does not allow the diagnosis of cystadenoma. In fact, it can be elevated in cases of cholestasis and is therefore less reliable for the diagnosis. Analysis of CEA and CA 19-9 in the cyst fluid is more useful than serum . High leRadiology imaging is important in diagnosis and characterization of hepatic cystic lesions. However, the radiological aspects of benign and malignant lesions overlap considerably. On ultrasound, a cystadenoma usually presents as a limited and large mass, with a thick and irregular wall that is rarely fine and smooth with internal echogenic septa, which delineate vesicles of different sizes and shapes; Wall nodules and papillary projections in the interior of the cyst can also be seen . The conA CT scan determines size, morphology, and relationships with neighboring structures; sometimes it may show the extent of the cyst in the bile ducts. A CT scan shows a hypodense cystic mass, well limited, multilocular with vesicles of variable density due to a different content of mucin. The wall is thick with internal partitions and sometimes wall nodules Fig. . After iMRI is very specific for diagnosis and differentiation of cystadenoma from other lesions; in addition, the combination of MRI with bili-MRI is even more useful. On T1 sequences, MRI reveals a multilocular mass, partitioned with a liquid content in hyposignal. On T2 sequences, the liquid is hyperintense but heterogeneous due to a variable protein content from one vesicle to another, whereas the wall is hypointense. The intracystic septa are also hypointense in T2. After an injection of gadolinium, an enhancement of the capsule and septa, the existence of nodules, and local invasion of the liver, bile ducts, and portal vessels are suggestive of carcinomatous transformation .A bili-MRI provides information on the state of the biliary tract, and the site and nature of a possible biliary obstacle: mucin, cyst’s protrusion in the bile ducts, or extrinsic compression.In our patient, this examination made it possible to demonstrate the biliary communication and the lesion’s extension in the bile duct.Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTHC) may show a heterogeneous aspect in the bile duct that may be related to the tumor or to intraluminal mucin  Fig. 7)7); sometPercutaneous aspiration of the cyst with a fine needle under echographic control allows a bacteriological, cytological, and tumor markers study of the suction liquid. The cystic fluid may be hemorrhagic, bilious, mucinous, clear, or mixed; hemorrhagic fluid leads to a cystadenocarcinoma, on the other hand, a mucinous content is mostly a characteristic of biliary cystadenoma . This exThe differential diagnosis of cystadenoma occurs mainly with other hepatic cystic lesions, especially with the simple cyst. Also, hepatorenal polycystic disease is a frequent hereditary disease where lesions have the same characteristics as simple cysts; the association with a polycystic kidney is constant which makes it possible to pose the diagnosis . In our In the anatomopathologic stage, the differential diagnosis of a cystadenoma is cystadenocarcinoma. In fact, cystadenocarcinoma shares most of the morphological and radiological characteristics of cystadenoma, so the distinction is almost exclusively anatomopathologic; therefore, distinction requires search for the criteria of malignancy in multiple histologic sections .The treatment of choice of hepatic cystadenomas is surgical resection. The objective is to avoid local recurrence and malignant transformation; this can be achieved by widespread surgery removing the whole cyst with a healthy tissue margin around the lesion, and making an extemporaneous examination if necessary. Enucleation may be an alternative for superficial lesions and large central lesions that are closely related to the main vascular and biliary structures.The procedure can be performed by laparotomy or laparoscopy. The procedure is carefully selected according to the size and the location of the tumor; laparoscopic treatment may lead to results that are similar to those of open surgery .Intraoperative cholangiography should be performed to search for possible biliary communication. In this case, resection of the tumor must be completed by a suture of the fistula or even a resection of the affected bile duct with a biliodigestive reconstruction if necessary.Hepatic transplantation may be necessary in the rare cases of bilobar tumoral extension making it impossible to perform a resection .In our patient, the endemic context and the radiological images led to the mistaken diagnosis of a ruptured hydatid cyst in the biliary tract; consequently, her former two operations were inadequate and it was only after histologic study of the “membranes” that the correct diagnosis was made.The prognosis of hepatic cystadenomas is extremely good after complete resection. Inappropriate treatment, in particular aspiration or partial resection, is associated with a recurrence rate greater than 90% and a malignant degeneration risk of 30% .Hepatic cystadenoma is a rare tumor, which is often confused with other cystic tumors of the liver and the confusion can be increased when a hepatic cystadenoma is complicated by rupture in the bile ducts. Our observation is a concrete illustration of this confusion: a cystadenoma was twice taken for a hydatid cyst ruptured in the biliary tract, which is a much more frequent situation given our endemic context.


Handlebar hernias are very rare and arise following a sudden force from a handle-like object impacting a focal area of the abdomen, which results in a disruption of the underlying abdominal muscle and fascia without necessarily disrupting the overlying skin. Other than a reducible swelling on the abdominal wall, the physical examination of such patients is usually unremarkable and the diagnosis could easily be missed.An 8-year-old Cameroonian boy with no significant past history presented to our emergency service with a tender left flank swelling following a road traffic accident. He was knocked down by a motorbike with resulting impact of the handlebar on his abdomen. A handlebar hernia was diagnosed on the basis of a reducible abdominal swelling with a positive cough impulse. A herniorrhaphy was done the following day after resuscitation and his postoperative period was uneventful.Handlebar hernias, although rare, should be suspected when patients present with an abdominal swelling following blunt abdominal trauma involving a handlebar-like object. A good history and physical examination are usually enough to pose an early diagnosis of handlebar hernia. Management typically involves surgical intervention to prevent complications. The timing and surgical approach should be decided on a case-by-case basis. Hand in 1956  and are  in 1956 . We repoAn 8-year-old Cameroonian boy with no significant past history was brought to our emergency service following a road traffic accident. On crossing a road, he was knocked down by an oncoming motorbike weighing approximately 125 kg, medium sized, and traveling at moderate speed. The motorbike fell on the boy with the handlebar catching him in the abdomen as its rider lost control. He sustained a lesion to the head with no initial loss of consciousness. On arrival, he complained of pain in his head and a swelling in the left upper quadrant of his abdomen.On examination, he was conscious, with a Glasgow coma score of 15/15, blood pressure of 119/83 mmHg, pulse of 90 beats per minute, respiratory rate of 18 breaths per minute, temperature of 37.3 °C; he was 96 cm tall and weighed 26 kg. There was a 2 cm laceration in the occipital region of his head, and a swelling in his left lumbar region with mild bruising of the overlying skin  occurred in children aged 4 to 14 years , 20. In The diagnosis of handlebar hernia is mostly clinical , 18. A hThe management of handlebar hernias involves prompt surgical repair to prevent incarceration or strangulation , 23. If The timing of the surgery could be emergent or delayed. The most important factor for the timing of the surgery is the probability of intra-abdominal injury, with immediate operation being preferred as it allows the ruling out of any intra-abdominal injury and prevents strangulation of any incarcerated bowel . HoweverIt is important to always rule out intra-abdominal injury when managing traumatic abdominal wall hernias. There exists a debate on whether a midline exploratory laparotomy or local wound exploration is best to rule out intra-abdominal injury . In the Handlebar hernias, although rare, should be suspected when patients present with an abdominal swelling following blunt abdominal trauma involving a handlebar-like object. In the absence of an ultrasound and computed tomography scan, a good history with a clear mechanism of injury and a physical examination are imperative for an early diagnosis. Management typically involves surgical intervention to prevent complications, but the timing and the surgical approach should be decided on a case-by-case basis.


Urothelial cell carcinoma (UCC) metastases to skeletal muscle are extremely rare and usually found in patients with advanced stage cancer. The most common sites of bladder cancer metastases are lymph nodes, lung, liver, and bones. Muscle is an unusual site of metastases from a distant primary cancer, due to several protective factors. We present a rare case of 76-year-old patient with metastases in the flexor digitorum superficialis (FDS) and flexor digitorum profundus (FDP) muscles, 2 years after a radical cystectomy for invasive UCC of the bladder. This case is the first description of a forearm lesion, with an extensive infiltration of the volar compartments of the forearm, and the first one with a clear functional impairment. UCC is the most common primary malignancy of the urinary tract, accounting for more than 90% of all bladder cancers, and may affect its entire length, from renal pelvis to bladder . UCC is Skeletal muscle metastases from bladder cancer are very rare. In the English literature, only five studies reported –6 on skeThe case that we present is the first report of metastases involving the FDS and FDP muscles after an invasive UCC of the bladder.The authors have obtained the patient's informed written consent for print and electronic publication of the case report.A 76-year-old male presented to the general practitioner with gross hematuria and urinary frequency. The ultrasound (US) imaging and the cystoscopy revealed a mass in the bladder. A transurethral resection was undertaken and an invasive carcinoma  was found at the histological examination. Three cycles of neoadjuvant chemotherapy were performed with cisplatin and gemcitabine.Four months later, a radical cystectomy and an ileal conduit urinary diversion were performed. 1 year after, a complete thoracoabdominal computed tomography (CT) was negative for metastases.18 months later, the patient presented a persistent anterior right forearm pain, resistant to analgesic, at first underestimated by the general practitioner and treated as tendonitis.The patient was referred to our hand surgery unit six months later for persistent pain of the right forearm and a painful flexion contracture of the proximal interphalangeal joints of the fingers  with theThe consequent clenched fist position was not passively reducible. Spasticity was also supposed, but there was no evidence of hyperactive response to quick stretch of the long fingers .The Magnetic Resonance Imaging (MRI) revealed an irregular ring-enhancing lesion of 7.5 cm × 2.7 cm × 2.1 cm in the superficial and deep volar compartment of the forearm . The lesA surgical explorative excision  of the lThe pathology reported a skeletal muscle metastasis compatible with a high grade UCC positive to the CK7, p63, GATA3, and AE1/AE3 markers and negative to the CK20 Figures  and 5.A Pet/CT whole body  showed aFor a local pain control, a palliative chemotherapy was administered with carboplatin and gemcitabine. Then, 5 cycles of 4 Gy radiotherapy were performed in the forearm (20 Gy).At 4 months postoperatively, the patient showed a recalcitrant flexion contracture of the long fingers without local pain in the right forearm.Five months after the surgical excision, the patient died due to the progressive deterioration of the general conditions.Metastases to the skeletal muscle from distant primary lesions are rare and usually found in case of advanced stage of cancer. Muscles are an unusual site of metastases even though they represent almost 50% of the total body mass  and are Several studies from autopsies report rates of muscle metastases oscillating from 6% to 17,5% , 11. TheSkeletal muscles metastases from UCC are, however, very rare and to our best knowledge, only 9 cases have been reported in the English literature –6.The great muscles, such as the erector spinae, psoas, and gluteals, are the most common sites of metastatic involvement . Nabi etEkici et al.  describeUsually, the clinical presentation of all the reported cases in literature –6 is a l Ekici et al.  emphasizSkeletal muscle metastases are frequently incidental findings on CT of the chest or abdomen. The CT scans show an increase in ring-shaped enhancement with hypoattenuation of the inner part. MRI findings, although not specific, show low to intermediate signal intensity in the T1-weighted image and uniform high-signal intensity in the T2-weighted image .Fine-needle aspiration biopsy with ultrasonographic guidance should be advisable to determine skeletal muscle metastases from UCC.In our case, we removed the lesion en bloc for diagnosis and treatment, according to Ekici et al.  and KataNabi et al.  reportedChemotherapy with or without the addition of local radiotherapy has been reported as an efficient treatment in skeletal muscle metastases –5. In thNabi et al.  used in Chemotherapy and radiotherapy are usually the preferred palliative treatment method. Surgical resection should be proposed in case of compressive effect of the metastasis, even though en bloc excision can improve pain control in painful muscle metastases , 5.Muscular localized swelling and lumps in patients with positive clinical history for UCC need to be taken as possible skeletal metastases. Radiological findings and a biopsy may help to study the lesion and to determine the most suitable treatment.


Systemic chemotherapy for stage IV colorectal cancer has advanced markedly in the recent years. We report an unusual case of 13 synchronous liver metastases for which a pathological complete response was achieved with neoadjuvant chemotherapy (NAC) consisting of a combination of 5-fluorouracil (5-FU), oxaliplatin, leucovorin (mFOLFOX6), and bevacizumab.A 44-year-old man was diagnosed with colorectal cancer with synchronous liver metastases. We resected the primary rectal tumor first. Further, after providing NAC for hepatic metastases, lateral segmentectomy and partial resection of the liver were performed. The subsequent result was compatible with a complete pathological response. The postoperative course was uneventful, and the patient is currently alive 5 years after the first surgery without evidence of recurrence and without adjuvant chemotherapy.For patients with initially resectable colorectal liver metastases, the survival benefits of NAC are still unclear. We report a rare case of 13 synchronous liver metastatic lesions from rectal cancer with a complete pathological response after neoadjuvant bevacizumab-containing chemotherapy. Systemic chemotherapy for stage IV colorectal cancer has advanced markedly in recent years following the clinical introduction of two powerful cytotoxic anticancer drugs  and moleTwo distinct strategies for preoperative chemotherapy for CRLM have emerged: neoadjuvant and conversion chemotherapy. Neoadjuvant chemotherapy (NAC) refers to the administration of preoperative chemotherapy for initially resectable CRLM. Although the role of NAC in patients with initially resectable CRLM is still controversial, objectives of this approach include reducing the tumor size, controlling any micrometastases, and avoiding liver surgery in those with rapidly progressive disease.We report herein an unusual case with 13 liver metastases for which a pathological complete response was achieved with NAC consisting of a combination of 5-fluorouracil (5-FU), oxaliplatin, leucovorin (mFOLFOX6), and bevacizumab, although the patient was considered to have a progressive disease during preoperative chemotherapy.A 44-year-old man was accidentally found to have positive fecal occult blood tests on a regular checkup; therefore, he visited a local hospital for further examination. Colonoscopy showed a type 2 tumor, 34 × 27 mm in size, in the rectum Fig. a. Then, Contrast-enhanced computed tomography (CT) showed two hepatic metastases in segments II and IV. We planned to resect the primary rectal tumor first, followed by liver resection. The patient underwent low anterior resection of the rectum, and the resected specimen was a type 2 tumor of the rectum Fig. b. HistolAfter 6 cycles of mFOLFOX6 plus bevacizumab, a major response of the liver metastases was documented by using CT. All metastases in the liver decreased in size, and the tumor morphology changed from heterogeneous attenuation, a variable degree of enhancement, and ill-defined borders before treatment to homogeneous, hypoattenuation with well-defined borders Figs.  and 3. HHe underwent lateral segmentectomy  and partial resection of segments IV, V, VI, VII, and VIII 1 year after the first operation to confirm that the number of metastases in the liver had not increased further.Macroscopically, the tumor in segment II was white and homogeneous, and it had a clear border Fig. a. PatholWe reported herein a rare case of 13 synchronous liver metastatic lesions from rectal cancer with a complete pathological response after neoadjuvant bevacizumab-containing chemotherapy. Another unusual feature of the case was that the response evaluation on CT after 6 cycles of chemotherapy indicated a progressive disease because a new lesion (the 13th lesion) emerged in the liver.For patients with initially resectable CRLM, the survival benefit of NAC is still unclear. Potential disadvantages of NAC are the inherent risk of disease progression preoperatively and the risk of liver toxicity. The routine use of NAC for patients with clearly resectable lesions limited to the liver is not recommended owing to a lack of benefit on survival .However, both imaging and pathological responses of liver metastases to preoperative chemotherapy are good prognostic variables , 6. NAC A complete radiological response was achieved in 3 to 14 % of patients in the neoadjuvant setting with cytotoxic chemotherapy  for CRLM –10. EvenBevacizumab is the only anti-angiogenic agent that has been extensively studied in the setting of resectable  or unresectable CRLM. Although the complete radiological response rate in patients treated with bevacizumab-containing regimens ranges from 0 to 9 % –18, a paThe optimal timing of primary tumor and liver metastases resection in patients with synchronous resectable CRLM is still controversial. Our strategy has been to perform resection of the primary colorectal tumor first followed by hepatectomy . The patient was treated with NAC for synchronous liver metastases following the resection of primary cancer because the number and size of liver metastases had rapidly increased before the planned hepatectomy.On contrast-enhanced CT performed after 6 cycles of bevacizumab-containing chemotherapy, all the 12 liver metastases detected before chemotherapy had decreased in size, and radiological findings of the metastases showed effective signs corresponding to the optimal morphologic response . HoweverComplete pathologic clearance of all liver metastases after chemotherapy is associated with considerable overall survival, and it is a strong predictor of both prolonged survival and disease cure . SeveralIn conclusion, the survival benefits of NAC are still unclear for patients with initially resectable colorectal liver metastases. We report a rare case of 13 synchronous liver metastatic lesions from rectal cancer with a complete pathological response after neoadjuvant bevacizumab-containing chemotherapy.


Malignant extrarenal rhabdoid tumor of the gastrointestinal tract is rarely reported in the literature. It is characterized by poor prognosis and aggressive metastatic features.A literature review evidenced only 19 cases, with poor outcome.We report a case of a colonic “pure” malignant extrarenal rhabdoid tumor with metastatic nodes in a 65-year-old Caucasian man. He was treated surgically with no recurrence, no adjuvant chemotherapy, and with 4-year survival without disease at the time of the submission of this article.We present an extraordinary case of long-term survival due to the extended surgical treatment.We believe that the absence of organ metastasis at presentation is a positive prognostic factor, although pathology confirmed node involvement (13/38 positive) on microscopy. A malignant rhabdoid tumor (MRT) is a subtype of Wilms’ tumor; first described by Beckwith and Palmer in 1978 . PresentA 65-year-old Caucasian man was admitted to Padova University Hospital for diffuse abdominal pain associated with generalized weakness, decreased oral intake, and weight loss of 20 kg in 2 months.A physical examination at admission showed pale conjunctiva and skin, bilateral auscultation revealed clear lungs, and heart auscultation did not elicit any murmurs, rubs, or gallop.An abdominal examination induced abdominal pain with rebound tenderness; bowel sounds were present in all quadrants. A right inguinal mass suggestive of lymphadenopathy was found on examination. The rest of the physical examination was unremarkable.Patient history revealed ischemic cardiomyopathy treated with endovascular coronary revascularization, diabetes, and depression. Diverticulosis of the colon and sigmoid polyposis were reported in a colonoscopy performed the previous year. No family history of malignancy was reported. During the recovery, our patient underwent esophagogastroduodenoscopy (EGD), which was negative, and a colonoscopy, which showed an ab-extrinsic compression of the cecum.A total body computed tomography (CT) scan was performed, and it described a 10 × 8 × 10 cm mass invading the cecum and ascending colon Fig.  with lymOur patient underwent an elective right hemicolectomy with extended lymphadenectomy in the hepatogastric and aortocaval areas, and renal fat excision. The post-operative period was uneventful and he was discharged from the surgical unit on post-operative day 6.The pathologic diagnosis was rhabdoid tumor. The tumor stage was defined as T3N2M0 (13/38 positive nodes). Immunohistochemistry revealed cells positive for vimentin Fig. .Fig. 2ImWe performed a systematic review of the literature regarding cases of colonic intestinal MERT. Case reports and center series were identified by PubMed “MeSH” search up to October 2016. Search items included: MERT, malignant rhabdoid tumor, colon rhabdoid tumor, rhabdoid colon cancer, and rhabdoid carcinomas. The cases obtained were from 19 manuscripts plus our case report, for a total of 20 patients to be analyzed.A thorough search of the literature revealed 19 cases of primary rhabdoid tumor of the colon. Table et al. treated their patient with FOLFOX4 and leucovorin, flurouracil, and irinotecan (FOLFIRI) regimen, with a survival of 7 months [et al. described therapy with adjuvant radiotherapy (40 Gy) and chemotherapy  [All patients underwent surgical resection as the first line of therapy. The overall survival for this tumor, even after surgical intervention, seems to be limited, with a large majority of the patients surviving less than 6 months. Only three studies described a survival of 1 year; in these patients there was no distant metastatic organ disease at the time of presentation. Six patients received adjuvant chemotherapy, five of them were N1. One patient received 12 cycles of leucovorin, flurouracil, and oxaliplatin (FOLFOX) and he was still alive at the time the manuscript was written, surviving 36 months without recurrence or distant metastasis . Another7 months . Han et iplatin) .Malignant rhabdoid tumors (MRTs) are aggressive neoplasms that were first described in young children with renal primary neoplasms , 11. MRTThese tumors are described as “composite” when the rhabdoid phenotype is mixed with another type of identifiable neoplasm and termed “pure” when the rhabdoid features are the only identifiable phenotype. When adenocarcinoma and rhabdoid tumor coexist, the rhabdoid features remained similar in all of them .et al. [The histogenesis of MERT is unclear. Ota et al.  suggesteThe management of these tumors is usually surgical resection if the tumor is operable. The rare occurrence of MERT has made it complicated to establish adequate survival-improving protocols. Most of the reports indicate that MERTs are highly aggressive tumors, with more than 75% of patients dying within 6 months of the initial diagnosis , 4.GI rhabdoid tumors are very rare. The majority of extrarenal rhabdoid tumors occur in children, while GI rhabdoid tumors are more common in the elderly population. Cytokeratin and vimentin are frequently found on immunochemistry.Historically, all the rhabdoid colorectal cancers reported in the literature primarily affect older people, with ages ranging from 62 to 84 years of age and with no gender preference. Nevertheless, we found a few cases in young patients in our review.et al. concluded in their meta-analysis that surgery and actiomycin therapy may improve survival in MERT [et al. [et al. [Recent reviews suggested that these tumors are large and tend to be located in the transverse colon or proximal ascending colon , 4, howe in MERT . Benefic [et al.  and Remo [et al.  reported [et al. .We report an extraordinary case of a patient with long-term survival due to the extended surgical treatment. We believe that the absence of organ metastasis at the presentation should be a positive prognostic factor, although pathology confirmed node involvement (13/38 positive) on microscopy. Since we performed an extended right hemicolectomy with lymphadenectomy (upto the main nodes), omentectomy, and removal of the perirenal fat, we believe that the combination of no organ metastasis and careful extended surgery could explain this extraordinary case of long survival. Our Department of Oncology did not propose chemotherapy for our patient due to the absence of a therapeutic protocol with proven efficacy in the literature for this rare tumor. The follow-up CT scans showed absence of disease.In conclusion, the current case report is, to our knowledge, the only case of a patient with colonic rhabdoid tumor with extended surgical resection with a survival of 4 years without recurrence or metastatic disease evidenced in control CT scans. All the published cases exhibit similar clinical-pathological features, as well as the characteristic histological features of sarcoma differentiation of rhabdoid cells, which appear to indicate aggressive biological behavior. Further investigations into this highly aggressive colonic carcinoma showing rhabdoid features are required in order to determine specific and effective treatment for this type of tumor.


Adrenocortical carcinomas (ACCs) are rare and often aggressive with more than 50% of the cases already in stage III-IV (ENSAT) at the time of diagnosis. Nearly 60% of ACCs present with hormone overproduction syndromes (Cushing’s syndrome and/or virilization), while the rest present with abdominal mass or incidental finding. Aggressive surgical resection is the mainstay of treatment usually followed by adjuvant mitotane monotherapy. For the advanced stage, adjuvant radiotherapy and combined chemotherapy with mitotane therapy can be added for survival benefit. Here, we would like to report a case of stage III high-grade ACC without syndromes of hormone overproduction, initially presented with pulmonary embolism. It was rapidly progressive with metastases to lungs, peritoneum and bone despite aggressive surgery followed by adjuvant mitotane monotherapy. However, after palliative radiotherapy to thoraco-lumbar spine for spinal cord compression, and adding chemotherapy  to mitotane, a significant partial remission was achieved. He has had 24 months of progression-free survival, and is currently on mitotane monotherapy with cortisol replacement. Discussion will support multimodality therapy for stage III high-grade ACC with surgery immediately followed by adjuvant radiotherapy and combined chemotherapy with mitotane therapy to prevent local recurrence and distant metastases. The incidence of adrenocortical carcinomas (ACCs) is very low with 1 - 2 per million population per year . ACCs arEven though the mainstay of treatment for ACCs is aggressive surgical resection followed by adjuvant mitotane therapy, there is limited evidence of the benefits from adjuvant radiotherapy and combined chemotherapy with mitotane therapy.Here, we would like to report a case of stage III high-grade ACC with capsular invasion in a 38-year-old male patient, who presented with pulmonary embolism and left flank mass, without syndromes of hormone overproduction.A 38-year-old man with no significant past medical history presented with left-sided chest pain, dyspnoea on exertion and left flank pain in April 2011. He was found to have left lower lobe segmental pulmonary embolus on a VQ scan, together with an incidental finding of a large soft tissue mass (15.5 × 12.0 × 16.7 cm) with no arterial phase enhancement, immediately superior to the left kidney in the CT scan. No local or distant metastases were noted in the staging CT scan .He underwent an open laparotomy for removal of the tumor, left kidney and spleen. Pulmonary embolism was treated with therapeutic enoxaparin.Macroscopically, a large, well circumscribed tumor (210 × 160 × 100 mm) with soft granular tan cut surfaces was found to be displacing the left kidney posteriorly and inferiorly . No adreMicroscopically, the tumor completely replaced the left adrenal gland, and was composed of sheets of large pleomorphic cells with eosinophilic cytoplasm . It demoTumor cells were strongly positive with Vimentin , weakly The above findings classified the tumor as stage III (T3N0M0) undifferentiated large cell ACC (as per both AJCC and ENSAT staging system).Since the initial presentation there were no signs and symptoms of hypercortisolism, though urine androsterone, etiocholanolone, tetrahydrocortisol and tetrahydrocortisone level were high. The latter went back to normal after the radical surgery.Adjuvant mitotane monotherapy (3 - 6 g/day) together with cortisol replacement was initiated after the surgery. The patient enjoyed disease-free period of 7 months until he developed metastatic disease in bones, lungs and peritoneum , 5a. At Majority of the adult ACCs 60%) have syndromes of hormone overproduction on initial presentation, and the remaining are non-functional, presenting with an abdominal mass, constitutional symptoms or an incidental finding. Rarely, ACCs can present with tumor extending into the inferior vena cava and even into the right atrium . A case 0% have sThe only potentially curative treatment for ACCs is complete surgical resection . For resFor recurrent or metastatic ACCs , surgical resection can also prolong survival in surgically accessible cases . ChemothIn our patient, initially, he presented with stage III left-sided ACC (ENSAT), and underwent aggressive surgical resection including the tumor, spleen and the left kidney. Subsequently, adjuvant mitotane therapy was started with cortisol replacement. However, he did not have adjuvant radiotherapy. Seven months after the diagnosis, while he was on adjuvant mitotane monotherapy, the patient developed multiple pulmonary, peritoneal and bony metastases without hypercortisolism. After going through palliative radiotherapy to the thoroco-lumbar spine (35 Gy in 10 fractions) for spinal cord compression, chemotherapy (six cycles of EDP) was added to the adjuvant mitotane therapy. As a result, peritoneal metastases resolved completely with only two stable sub-centimeter lung nodules remained and stable bony metastatic disease. He has been followed up 3-monthly till now with no evidence of disease progression leading to progression-free survival of 24 months with continuous mitotane monotherapy.In conclusion, ACCs can have different unusual clinical presentations; pulmonary embolism is the initial clinical presentation in our case. The experience in our case supports multimodality therapy for stage III high-grade ACC due to the short disease free interval from surgical resection and relapse with metastatic disease. Multimodality therapy includes adjuvant radiotherapy and EDP in addition to mainstay aggressive surgical resection and adjuvant mitotane therapy.


Hidden caries is a dentinal lesion beneath the dentinoenamel junction, visible on radiographs. A single report described this lesion in primary dentition. This case report describes a case of hidden caries in a mandibular second primary molar, misdiagnosed as malignant swelling. A 3-year-old white girl was referred to the Department of Pediatric Dentistry with a chief complaint of pain and extraoral swelling on the right side of the mandible for the last 3 months. She was earlier referred to the surgical department for biopsy of the lesion. Radiographic and computed tomography scan examination showed a periapical lesion with buccal plate resorption and radiolucency beneath the enamel on the mesial part of tooth 85. The tooth was extracted, and follow-up of 2 years showed normal development of tooth 45. The main problem is early detection and treatment, since the outer surface of enamel may appear intact on tactile examination.Gera A, Zilberman U. Diagnosis and Management of Hidden Caries in a Primary Molar Tooth. Int J Clin Pediatr Dent 2017;10(1):99-102. Usually, a single tooth is affected. However, cases of multiple PICRLs in individual subjects have also been reported.2 A single report of PICRLs in primary teeth has been published.3 Nearly half of the lesions extend to more than two-third of the dentin.4 No association was found between PICR and gender, race, medical conditions, systemic factors, or fluoride supplementation.145Hidden caries is a dentinal lesion beneath the dentinoenamel junction, visible on radiographs. It is also known as preeruptive intracoronal resorption (PICR), preerup-tive intracoronal radiolucent lesion (PICRL) or preerup-tive caries. The prevalence of PICR in permanent dentition is 2 to 6%, depending on the tooth and radiographic technique. When bite-wings were used, the prevalence is 4% for the mandibular first molar, 2% for the mandibular first premolar, and 1% for the maxillary first molar, maxillary first premolar, and mandibular second molar.5 local factors play an important role in the etiology. There is a significantly high association between ectopically positioned teeth and PICRL, which suggests that an ectopic position is a trigger factor. Pressure resulting from an abnormal position may induce sufficient local damage to the tooth’s protective covering causing resorp-tive cells to enter through the enamel. Loss of the integrity of the reduced enamel epithelium may allow osteoclasts, multinucleated giant cells, and chronic inflammatory cells to enter the tooth and initiate resorption of dentin.5The etiology of PICRL remains a controversy. Suggested causes include apical inflammation of primary molars (relevant only to PICRL in premolars) and dental caries. The most likely hypothesis is that the defects are acquired as a result of coronal resorption. According to Seow et al,This report describes a case of PICRL in a mandibular second primary molar and the subsequent treatment.A 3-year-old white girl was referred to the Department of Pediatric Dentistry at Barzilai University Medical Center, Ashkelon, Israel, with a chief complaint of pain and extra-oral swelling on the right side of the mandible for the last 3 months. Her medical history was not remarkable. She attended several pediatric dentists for diagnosis, who prescribed antibiotic therapy. The swelling regressed and reappeared. Later, she was referred for a panoramic X-ray, misdiagnosed as malignant swelling, and scheduled for biopsy at oral surgery.Extraoral examination revealed swelling on the right side of the mandible, which was diffuse, tender, and warm to palpation. The overlying skin appeared flushed. Intraoral examination revealed intact primary dentition with buccal cortical plate expansion on the right side of the mandible .On panoramic radiograph, tooth 85 had a periapical radiolucency . The comLocal anesthesia was applied and tooth 85 was extracted. The exterior opening of the hidden caries was located using a #8 file that entered from the mesial central fissure and reached the pulp horn .Follow-up examination 1 week later showed soft tissue healing. 6 months later, no clinical symptoms or swelling were observed  and 7. TThis case demonstrated a misdiagnosed PICR of a man-dibular second primary molar, as malignant swelling.5 with curettage of the defect or to wait for tooth eruption to achieve occlusal access for restoration of the defect.6 In this case of a primary molar, extraction was the treatment of choice due to large periapical lesion reaching the underlying permanent tooth follicle and very young age.The purpose of this report is to increase the clinician’s awareness and clinical surveillance regarding diagnosis of PICRL, and to avoid further misdiagnosis . The management of PICRL in permanent dentition varies and may consist of immediate surgical exposure478 Although the etiology and factors associated with the initiation of resorption remain unknown, resorptive cells originating from the surrounding bone are thought to enter the dentin through a break in the dental follicle and enamel or cementum.39Clinical and histological evidence from several case reports has suggested that these lesions are likely to be resorptive in nature.10 calcification may begin as early as 18 weeks on the tip of the MB cusp, while the other cusps calcify later . At 28 weeks, all five centers of calcification are present, and only then the intercuspal coalescence occurs, and varies with respect to location. Union between the MB and distobuccal cusps may occur via their distal and mesial cusp ridges respectively (at 30 weeks), or the mesial marginal ridge may be the first to calcify (at 30 weeks), thereby connecting indirectly, or in a roundabout way the two mesial cusps. Whichever union occurs first, it is followed by the alternate coalescence to form a calcified ring uniting distobuccal, MB, and mesiolingual cusps (at 32 weeks). Coalescence is complete by 36 weeks, and a small area of uncalcified tissue remains in the middle of the occlusal surface on the distal half of the crown. The 36-week mandibular second molar crown features sharp conical cusps, angular ridges, and a relatively smooth occlusal surface, indicating that considerable deposition of enamel is yet to occur before the completion of calcification.According to Kraus and Jordan,Based on this evidence, hidden caries may be due to a faulty union or imperfection at the time of coalescence of the centers of calcification. This suggests that occlusal surface defects on molar crowns are intimately associated with the early calcification pattern peculiar to the individual teeth.Increased awareness of this entity may improve diagnosis, allow early treatment, and avoid misdiagnosis.


Malignant Peripheral Nerve Sheath Tumors (MPNSTs) of the prostate are extremely rare. A very unusual case of simultaneous adenocarcinoma and MPNST of the prostate is reported. A 60-year-old Caucasian male presented for annual urologic examination. Digital rectal examination revealed a painless, toughish, and asymmetrically enlarged prostate. Serum prostate-specific antigen was 1 ng/mL. Radiologic examinations demonstrated a large mass, which was arising from the left peripheral lobe of the prostate. The patient underwent transrectal ultrasound-guided biopsy of the prostate which revealed a smooth muscle tumor of uncertain malignant potential. Radical retropubic prostatectomy with en bloc removal of the mass and the seminal vesicles was performed and histology demonstrated low-grade MPNST and adenocarcinoma of the prostate. To the best of our knowledge, this is the first report of simultaneous prostatic adenocarcinoma and MPNST in the English literature. Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare soft tissue sarcoma. Sarcomas of the prostate are extremely rare. The aim of this article is to present the first case of simultaneous prostatic adenocarcinoma and MPNST in the English literature.In December 2015, a 60-year-old male patient visited the outpatient clinic of our department for routine urologic examination. Past medical history included hypertension, diabetes mellitus, acute myocardial infarction three years ago, and ischemic cerebrovascular accident thirteen years ago. On digital rectal examination, the prostate was painless, asymmetrically sizable, and toughish, with no other clinical findings. The patient's blood count and blood chemistry levels were in the normal range. Serum PSA was 1 ng/mL.Transabdominal ultrasonography demonstrated a solid, large mass, in contact with the urinary bladder. The prostate's volume was 31 mL and the postvoid residual of urine was 62 mL. Abdominal/pelvic computed tomography (CT) scan demonstrated the presence of a solid, lobed, well-circumscribed mass, which was arising from the left peripheral zone of the prostate and a possible infiltration of the left seminal vesicle. The urinary bladder wall was normal and the distal part of the left ureter crossed between the mass and the bladder wall . TransreThe patient underwent radical retropubic prostatectomy with en bloc removal of the mass and the seminal vesicles. A double-J ureteral stent was placed prophylactically in the left ureter, which was removed after the mass excision. The patient had an uneventful postoperative course.The total resected specimen weighed 145 gr. Macroscopic examination revealed a tan-gray mass, 7 × 6.5 × 5.7 cm in diameter, which invaded the posterior part of the left prostatic lobe . The proAbdominal MRI and chest CT scan were normal three months postoperatively and the patient received adjuvant radiotherapy. Serum PSA was 0 ng/mL. The patient has no signs of relapse and is in good general condition 6 months after the operation.MPNSTs arise from a peripheral nerve or from a preexisting benign nerve sheath tumor or demonstrate Schwann cell differentiation on histology –3. FurthMPNSTs comprise 5–10% of all soft tissue sarcomas . SarcomaMPNSTs may occur anywhere along the course of myelinated nerves, but they commonly appear in or near a nerve of the trunk or the limbs , 5. PelvThe differential diagnoses include benign neurofibroma, fibrosarcoma, liposarcoma, ganglioneuroma, hydatid cyst, hematoma, and connective tissue diseases .Preoperative radiologic examinations play a vital role in the diagnosis of a MPNST and in surgical planning. Ultrasonography can discriminate solid tumors from cystic masses . CT scanMacroscopically, MPNSTs are globoid or fusiform in shape, fleshy, and firm to hard in consistency and their color is typically tan-gray on cut section . Areas oMicroscopically, the tumor is characterized by hypercellular fascicles of spindle cells interrupted by hypocellular myxoid areas, often with hypercellular areas localized in close proximity to blood vessels , 3, 6–8.Complete surgical extirpation of the tumor with clear margins is the treatment of choice –3, 5–8. Prognosis is poor. Five-year overall survival is reported to be 15–50% . The locFollow-up guidelines have not been defined, but many authors recommend MRI imaging every 3 months for detection of local or distant recurrence .Although there has been progress in diagnosis of MPNSTs, related with advances in imaging methods, there is still a lot to be researched with regard to the genetics and the molecular biology of these tumors . Definin