Mazabraud's syndrome is a rare but well-described disorder characterized by fibrous dysplasia in single or multiple bones associated with one or more soft-tissue myxomas. In this report, we describe what is, to the best of our knowledge, the first case involving an intra-osseous myxoma. This finding supports, and could provide new insight into, the pathological association between fibrous dysplasia and myxomas.In this report, we describe the case of a 49-year-old Caucasian woman known for years to have fibrous dysplasia in the left femur and tibia who presented with progressive pain in her left leg and soft swelling in the left quadriceps region. After surgical intervention with excision of the soft-tissue mass, the diagnosis of Mazabraud's syndrome was confirmed. During follow-up, our patient presented with a painless mass located on the lateral side of the left knee, next to a second, intra-osseous lesion with the same characteristics in the left lateral tibial plateau. The histopathological examination was consistent with a soft-tissue intra-osseous myxoma.In the international literature, 67 cases of Mazabraud's syndrome have been described so far.To our knowledge, the present case report is the first to describe the combination of polyostotic fibrous dysplasia and intra-muscular as well as intra-osseous myxoma. Mazabraud's syndrome is a rare but well-described disorder. It is characterized by fibrous dysplasia, which can develop in a single bone (monostotic) or in multiple bones (polyostotic), associated with one or more soft-tissue myxomas.et al. in 1967 [The first case was described by Henschen in 1926 [Fibrous dysplasia is a benign, intra-medullary, fibro-osseous lesion and usually develops in childhood or early adult life. In the 67 cases of Mazabraud's syndrome described so far, most patients had the polyostotic form of fibrous dysplasia.Soft-tissue myxomas are benign mesenchymal tumors. In Mazabraud's syndrome, they usually occur (1) when the patient is at a more advanced age and (2) in close vicinity of the bones most severely affected by fibrous dysplasia.We present the first case of a patient with Mazabraud's syndrome with an intra-osseous myxoma next to soft-tissue myxomas.A 49-year-old Caucasian woman was referred to our center. She was known for years to have fibrous dysplasia in the left femur and tibia.When she was 49 years old, an attempt was made to excise the fibrous dysplasia from the proximal femur. Extreme hemorrhaging complicated the operation. The attending orthopedic surgeon decided to refer the patient to our specialized center. At the time of her visit to our center, she complained about progressive pain in her left thigh and lower leg. Her clinical physical examination revealed soft swelling in the left quadriceps region.Conventional X-rays of the left leg showed typical features of fibrous dysplasia in the proximal femur Figure  and the Magnetic resonance imaging (MRI) of her left leg showed extensive fibrous dysplasia in the entire femur with expansive growth in the greater trochanter. The left proximal tibia also showed signs of fibrous dysplasia extending 20 cm distally from the tibial plateau. Four well-delineated, cyst-like, intra-muscular soft-tissue lesions were seen in the quadriceps region. Surgery was planned in two sessions. Prior to both operations, angiography and embolization of the pathologic vascularization in both the femur and the tibia were performed.During the first operation, intra-lesional excision of the tumor of the left proximal femur was performed, followed by cryosurgery. A correctional osteotomy of the left hip was performed, followed by homologous bone transplantation and fixation with a proximal femoral nail. Because of a weakened femoral head, the femoral neck screw had to be stabilized with bone cement. Allograft bone chips were impacted to induce bone matrix. During the procedure, an incisional biopsy of the soft-tissue lesions in the quadriceps muscle was performed.Three weeks later an intra-lesional excision of the fibrous dysplasia of the left tibia was performed, followed by cryosurgery and implantation of an massive allograft inlay in the tibia. The allograft was fixed with AO screws. During this procedure, the soft-tissue lesions in the quadriceps muscle were excised.Microscopic examination of the bone lesions of both femur and tibia showed hypocellular fibrous tissue with irregular bone formations and without cytologic atypia compatible with fibrous dysplasia. In one lesion, the remnants of fracture callus were present Figure .The soft-tissue lesions showed a paucicellular tumor with spindle-shaped or stellate not atypical cells embedded in a loose myxoid alcian blue-positive inter-cellular matrix with sparse capillary blood vessels. Confocal microscopy revealed a thin fibrous capsule at the tumor margin; however, at the interface with skeletal muscle, infiltration between the individual muscle fibers was evident, which is typical of intra-muscular myxomas Figure , provingPost-operatively, the patient's treatment consisted of non-weight-bearing mobilization for three months in plaster of Paris. During follow-up, plain films were obtained at six-week intervals. Eventually, these images showed consolidation of the femur osteotomy and incorporation of the allograft.Nine months post-operatively the patient developed progressive pain in the left thigh. Conventional X-rays showed protrusion of the femoral neck screw. Repositioning of the screw was performed. Her post-operative follow-up with X-rays obtained regularly Figures  and 6 waAlmost two years later the patient complained about a painless mass localized on the lateral side of the left knee. A solid mass was felt anterolaterally of the proximal tibia. Plain film radiographs did not show abnormalities; however, an ultrasound examination showed a homogeneous, hypoechogenic, soft-tissue mass with a cyst-like aspect. MRI showed a well-defined soft-tissue lesion in the lateral retinaculum and vastus lateralis muscle with a homogeneous high signal intensity on T2-weighted images and a homogeneous low signal intensity on T1-weighted images, consistent with fluid. A second intra-osseous lesion with an approximate diameter of 3 cm and the same signal characteristics and enhancement pattern was detected in the lateral tibial plateau Figures  and 8.The lesions were considered to be a new soft-tissue myxoma and a relapse of fibrous dysplasia in the proximal tibia, respectively. Surgical excision of both lesions was performed without pre-operative biopsies, followed by cryosurgery and homologous bone implantation. A histopathological examination of the intra-muscular lesion showed a myxoma. As a unique finding, histopathological examination of the intra-osseous lesion in the lateral tibial plateau was consistent with myxoma as well Figure .During follow-up, MRI of the patient's left leg was performed at regular intervals. These scans showed no signs of recurrence in the first post-operative years.Three years after her last operation multiple tumors in her left upper leg were felt, which raised clinical suspicions of myxomas. MRI scans showed five new soft-tissue lesions consistent with characteristics of myxoma in the upper and lower leg regions. All lesions were marked ultrasonographically and excised, followed by cryosurgery. Histopathological examination of all intra-muscular lesions confirmed the diagnoses. Post-operatively the patient recovered gradually. Every three months ultrasonography of her left leg was performed, which showed no signs of myxoma until 2009. By then, she had developed several new intra-muscular myxomas, which were not treated. At her last follow-up examination in early 2010, our patient was in good condition. Several myxomas had developed but were found to be stable on the basis of MRI.sα gene, leading to an increase in cyclic adenosine monophosphate, which leads to so-called downstream effects important in the pathogenesis of fibrous dysplasia [Fibrous dysplasia in itself is not a rare disorder; it is reported to represent 5% to 7% of benign bone tumors . MonostoAn intra-muscular myxoma is usually a solitary lesion that is not generally associated with any other clinically apparent abnormalities . It is aMultiple intra-muscular myxomas are rare and usually are associated with fibrous dysplasia, a condition known as Mazabraud's syndrome. In Mazabraud's syndrome, the polyostotic form of fibrous dysplasia is present in the vast majority of patients. In general, the onset of fibrous dysplasia antedates the appearance of intra-muscular myxomas, and the soft-tissue lesions become apparent many years later, usually in the fifth or sixth decade of life . The sitGNAS1 mutations exist in intra-muscular myxomas, which may play an important role in tumorigenesis in intra-muscular myxomas as well as in fibrous dysplasia [There are different hypotheses for the association between fibrous dysplasia and intra-muscular myxomas. Some authors have suggested a basic metabolic error in both tissues during the initial growth period . Variousysplasia .The radiological diagnosis of fibrous dysplasia is generally established by plain films. Being larger, polyostotic lesions are more commonly accompanied by deformation, including coxa vara, the shepherd's crook deformity, bowing of the tibia, and protrusion acetabuli .Ultrasound and MRI can be helpful to detect soft-tissue lesions in patients with Mazabraud's syndrome ,10. BothHistopathologically, the characteristics of fibrous dysplasia and intra-muscular myxoma are as described in this case report. Both lesions are completely benign, and malignant transformation in fibrous dysplasia is uncommon.In many cases, treatment of fibrous dysplasia is not necessary, since the lesions are discovered incidentally on plain radiographs and are asymptomatic. Clinical observation through follow-up radiographs is warranted to verify that there is no progression.Different studies have reported clinical improvement in patients with fibrous dysplasia after bisphosphonate therapy -13, althSurgical treatment of fibrous dysplasia may be necessary to correct a deformity, to prevent a pathological fracture, and/or to relieve pain in symptomatic lesions. Upper-extremity lesions can often be treated conservatively, but surgical intervention is required for many comparable lower-extremity lesions. Other factors influencing the type of intervention used are the size and biological behavior of the lesion and the patient's age.The use of curettage only (with or without autogenous cancellous bone grafting) is associated with a high risk of recurrence, since the cavity or graft of normal bone is replaced gradually by dysplastic bone in the healing process, returning the patient to the pre-operative state. Cortical allografts last longer, since these grafts show far less and slower internal replacement by host bone and are therefore preferable for reconstruction.The treatment of intra-muscular myxomas is dependent on the extent of the lesions, and these lesions should be excised if pain or pressure symptoms develop ,14. Our Mazabraud's syndrome is a rare disorder; hence the small number of publications in the literature since it was first described by Mazabraud in 1967.According to a recent article, there have been only 67 reported cases to date . Our preWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.WAW did the literature research, studied the case, and composed the article. HÜ was a major contributor to the writing of the manuscript. JWJR reported the X-rays and MRI scans and contributed to the description of the radiological examinations. UF performed the histological examinations and contributed to the description of the pathological examinations. RPHV revised the manuscript critically. All authors read and approved the final manuscript.


We presented a rare extratesticular neoplasm, papillary adenocarcinoma of rete testis, which manifested variable symptoms and mimicked most frequently seen benign extratesticular lesions. Due to its rarity, the treatment is therefore uncertain. Our patient's clinical manifestations mimicked an inflammatory lump and underwent radical orchiectomy after pathological report had been confirmed. Unlike other reports, our patient survives and has a good outcome. No definite predictor and tumor marker can be used to define the prognosis. Early diagnosis and surgical treatment may have a good outcome. Papillary adenocarcinoma of rete testis is an extremely rare extratesticular neoplasm with only a few sporadic reports in the literature . The cliA 30-year-old male patient came to outpatient department presented with a swelling and tender left scrotal lump for 2 weeks. There were associated symptoms of dysuria and frequency in urination within these days. He did not have fever nor body weight loss. Physical examination revealed a hard nodule at the head of left epididymis with severe tenderness. Ultrasonography of the scrotum revealed a 1.6 cm hyperechoic lesion over left epididymis . The iniα-fetoprotein (AFP) and β-human chorionic gonadotropin (βHCG) were within normal limits one week after partial epididymectomy. Computerized tomography (CT) of the abdomen revealed no visible lymph node in the retroperitoneal space or pelvic cavity. He further underwent a left inguinal radical orchiectomy 1 month after the first operation. The final pathological report revealed no residual tumor in testis, epididymis, or the stump of the dissected spermatic cord. There was no further adjuvant treatment such as radiotherapy or chemotherapy. He had been followed by serum AFP, βHCG, and abdominal CT annually without evidence of tumor recurrence for at least 48 months.Microscopically, there were hyperchromatic cuboid and columnar cells with papillary protrusions which fulfill the center hilus of testis Figures  and 3. TTo our best knowledge, papillary adenocarcinoma is a rarely extratesticular malignancy with only about 60 cases reported in the literatures , 4. It tβHCG to detect the tumor earlier. The diagnosis of adenocarcinoma of rete testis was often made by the pathologist after surgery. There are 5 criteria for the diagnosis of a primary tumor of the rete testis proposed by Nochomovitz and Orenstein: (1) absence of histologically similar extrascrotal tumor, (2) tumor centered on testicular hilus, (3) morphology incompatible with any other type of testicular or paratesticular tumor, (4) demonstration of a transition between the unaffected rete testis and the tumor, and (5) a predominantly solid appearance [The exact etiology of this tumor remains unknown. The most common symptoms of papillary adenocarcinoma included scrotal mass, indurations, pain, and swelling, which mimicked an inflammation. The misleading symptoms may delay the diagnosis and adequate treatment. It is difficult to make differential diagnosis with other extratesticular lesions because adenocarcinoma of rete testis may present with epididymitis, cryptorchidism, hydrocele, or inguinal hernia –10. Therr hilus,  morpholoThe prognosis of papillary adenocarcinoma seemed poor in the earlier reports. Cancer cells may invade locally, metastasize via lymphatics to the para-aortic and iliac lymph nodes, or hematogenously spread to the sites of lung and bone . The invUsually, the patient was a sexualactive young man with symptoms of inflamed left scrotum and lower urinary tract symptoms which were considered as a common epididymitis and urinary tract infection. The scrotal tumor was relatively small and was difficult to be differentiated with a swollen epididymis. It was lucky to identify the tumor from ultrasonography in a small size and early stage. After radical orchiectomy, the patient had at least 48-month tumor-free period. Good prognosis was probably due to the fact that our patient was young with a small and early-stage tumor. Although Sanchez-Chapado et al. suggested the benefit of RPLND , we did In conclusion, papillary adenocarcinoma of rete testis is a rare extratesticular malignancy which may have a diverse prognosis in different age. No definite predictor and tumor marker can be used to define its prognosis and method of treatment. A better prognosis may be reached if diagnosis and surgical were conducted treatment earlier.


Clear cell adenocarcinoma of the colon has been described scarcely in the literature. It affects elderly men more commonly than women and usually appears in the left side of the colon. A Hispanic 41-year-old female came to the emergency room with abdominal pain, vomiting, and distension. Physical exam revealed generalized tenderness without peritoneal signs. Laboratory data was unremarkable. A CT scan showed an apple-core lesion in the distal colon. A flexible sigmoidoscopy revealed an obstructive mass that made further evaluation impossible. Exploratory surgery revealed a hard mass obstructing the descending colon, which was resected. Histopathology analysis with immunohistochemistry staining was positive for cytokeratin 20, cytokeratin 10, CDX2, and villin, while it was negative for cytokeratin 7, RCC, vimentin, and CD31. These results confirmed the clear cell variant of the adenocarcinoma. Clear cell adenocarcinomas usually arise from the kidneys and Müllerian organs. Immunohistochemistry is crucial for establishing the origin of these neoplastic cells. A cytokeratin 20+/7− with positive CDX2 is highly specific and sensitive for intestinal neoplastic origin. The main treatment has been surgery alone with moderately good results. More research and information about this malignancy is needed, especially in regard to prognosis and in order to provide the best treatment option. Clear cell adenocarcinoma of the colon is a well-recognized but very rare subtype of colorectal cancer. Its true incidence and prevalence are unknown. Fewer than 20 cases have been reported in the English literature. We report a case and also review the current literature about this subtype of neoplasia.α-fetoprotein (AFP), and carcinoembryonic antigen (CEA) were within normal limits. She was started on pantoprazole and enoxaparin for deep venous thrombosis prophylaxis after an active bleeding was ruled out. A nasogastric tube was placed, but there was no fluid obtained. A flexible sigmoidoscopy was performed which revealed an obstructive mass that made evaluation of the colon above the mass impossible. Multiple biopsies were taken and displayed features that resembled adenocarcinoma of the colon.A 41-year-old Hispanic female presented to the emergency room with abdominal pain, progressive distension, nausea and vomiting, and occasionally bloody stools for 2 months. She had an episode of melena one day prior to admission. Surgical history included a tubal ligation. She denied smoking, allergies, and medications as well as positive family history of cancer. On clinical examination, she had abdominal distension and mild generalized tenderness without peritoneal signs. The rectal examination was unremarkable. Hemoccult blood was negative. A contrast CT scan of the abdomen and pelvis revealed mild atelectasis in the lung bases, moderate distension of the gall bladder, and an apple core lesion in the distal descending colon measuring 3.4 cm long resulting in prominent dilation of the colon and small bowel proximal to this lesion. The colon distal to the lesion was not distended. There was also mild ascites. All laboratories including her basic metabolic panel, liver function tests, complete blood count, She underwent surgical exploration. Intraoperative findings revealed an indurated, thick, obstructive lesion in the descending colon which was edematous but without perforation. A partial colectomy with end to end anastomosis was done.The surgery and postoperative course were uneventful. The histologic examination was consistent with colonic adenocarcinoma, with no perineural or vascular invasion noted. There was one positive lymph node out of 28 for metastatic tissue, and the specimen's margins were free of tumor. After the initial Hematoxylin and Eosin staining was performed, the clear cell variant was considered . SubsequThe tumor was staged American Joint Committee on Cancer (AJCC) IIIA, C, and C1 according to the Dukes and Astler-Coller staging systems, respectively. Tumor grade was moderately differentiated.After the surgery, the patient was discharged on the fifth postoperative day in stable condition.Since first reported in 1964 by Hellstrom and Fisher –5. ThereAccording to current literature, one way to differentiate a primary intestinal neoplasia from a Müllerian neoplasia is with cytokeratins –5, 10–14There is no study that evaluates the invasive characteristics or tendencies of this type of tumor. It is interesting to note that there have been no reported cases of perineural invasion. Surgery has been the main treatment in the reported cases. Four cases reported metastatic tissue either at time of diagnosis or after resection of the primary tumor , 10, 16.We can conclude that there are still some major aspects of CCACC that need to be further studied. An actual review of incidence, prevalence, prognosis, and survival is recommended. The clear cell origin of these tumors needs to be elucidated. Moreover, an adequate algorithm or plan for treatment must be elaborated.


Ancient Schwannoma, though benign, can cause diagnostic dilemma because of its clinical presentation and imaging features. We report the management of a giant retroperitoneal schwannoma in a 19-year-old young lady who presented with lower abdominal distension. CT scan reported a large heterogenous lesion in the abdominopelvic retroperitoneum (42 cm × 16 cm × 16 cm) as a malignant tumor. The unique problems we encountered were the enormous size, the location of major part of the tumor in the pelvis, the need for fertility preservation, the external iliac vessels stretching over the tumor making mobilization surgically demanding, and the prospects of neurological deficits. An en bloc resection of schwannoma with common iliac, external iliac and internal iliac veins, internal iliac artery, femoral and obturator nerves, and iliopsoas muscle was done maintaining oncological principles. External iliac artery that was cut to facilitate tumor mobilization was reanastomosed at the end of the procedure. Postoperatively patient had uneventful recovery with patchy sensory loss, foot drop, and quadriceps weakness which was rehabilitated with a foot drop splint and active physiotherapy. Giant retroperitoneal schwannoma, though benign, can cause diagnostic dilemma because of its clinical presentation and imaging features. In addition, it is a surgical challenge due to its enormous size and proximity to large vessels and other organs in the retroperitoneum. We are presenting a case, probably one of the largest retroperitoneal schwannomas reported in English medical literature, to discuss the unique surgical problems encountered in the management. The importance of proper anatomical localization of vessels, preoperative planning, and optimal involvement of specialists of other subspecialties for effective resection is reiterated.19-year-old postmenarchal young lady presented with lower abdominal distention and neuritic type of pain in the right thigh of two-year duration. The lower abdominal distension was progressively increasing and was associated with an increasing pain in the leg as described. There were no neurological symptoms.Clinically she had ECOG performance status 1, antalgic gait, and lower abdominal distension up to umbilicus with a firm to hard fixed mass arising from the pelvis. There were no neurocutaneous markers. Computed tomography study revealed a large lobulated heterogeneously enhancing mass lesion in the pelvic retroperitoneum  . The masThe external iliac vessels stretching over the mass precluded any mobilization without vascular injury. Hence the external iliac artery was cut at the middle to mobilize the tumour . The intPostoperatively patient had uneventful recovery. She had patchy sensory loss, foot drop, and weakness of quadriceps muscles. She was rehabilitated with a foot drop splint and active physiotherapy. At last follow-up, she was able to walk without support. Two years after surgery patient is without any evidence of disease.Ancient schwannomas are rare tumours originating from Schwann cells in the peripheral nerve sheath. Up to 20% of cases are associated with Neurofibromatosis Type 1  fluoro-D-glucose(FDG) on PET scanning is of limited value as a preoperative diagnostic imaging technique for the assessment of schwannoma versus sarcoma . High FDSince in our case the CT scan report was suggestive of malignancy, we decided to do FNAC and core biopsy under ultrasound guidance. This was reported as being benign nerve sheath tumor.Literature search about surgical management of such tumors revealed a tendency to confuse ancient schwannomas with malignant tumors on imaging and histology . ConfusiSome workers have argued that since it is a benign disease, even piecemeal excision even with laparoscope is an acceptable alternative . In suchHence after deliberations, we decided to treat the mass as potentially malignant and do excision taking into consideration oncological safety. Care must be taken in attempting removal of retroperitoneal and intrapelvic schwannomas. It is of utmost importance to meticulously plan and to involve experts in multiple specialties for optimal management. Sufficient amounts of blood products have to be readily available including fresh frozen plasma and platelets. The anesthetist should be made aware that a high volume blood loss might be encountered.Carpenter reported one intraoperative death related to uncontrollable hemorrhage from severing the right common iliac artery during a difficult dissection . In a caIn our case, both the right external iliac artery and vein were stretched over the tumor. Hence before mobilisation of the tumor, vascular clamps were applied and the artery was cut in the middle. This made mobilisation possible without a catastrophic hemorrhage. The accompanying vein was ligated at superior and inferior edges of the tumor. Venous ligation has the added advantage of preventing a pulmonary embolism in the postoperative phase, due to a preexisting thrombus in the affected lower limb. It is highly desirable to do a preoperative angiogram or MR angiogram to document collateral circulation. In situations where a vascular surgeon is not available, the ligation of major vessel supplying the limb might be the only alternative left. The ligation of internal iliac artery not only helped in mobilizing the tumor, but also decreased the tumor vascularity considerably. Preoperative radiologic guided obliteration of anterior division of internal iliac arteries has been shown to decrease the vascularity of such tumors. Haemostasis can be difficult especially in presacral schwannomas where there is involvement of presacral venous plexus. The vascular surgeon's expertise was a key event in the successful outcome of the surgery. Bull dog clamps are to be considered as an essential instrument in undertaking such surgeries. The end-end anastomosis of external iliac artery was done with No.7.0 Prolene. Postoperative blowouts at the anastomotic site have been reported. The wrapping of omental flap around the anastomosis is a simple but important aspect of surgery that may prevent catastrophic blowouts. Total blood loss was 2.5L. The femoral and obturator nerves were sacrificed as they were found to be going through the tumor. This, however, did not cause significant morbidity other than quadriceps weakness and patchy sensory loss in the right leg. Patient also developed foot drop probably due to nerve root damage. Since neurological deficits are to be anticipated, proper preoperative counseling and preparation of the patient are extremely important before undertaking such surgeries.We described a huge retroperitoneal abdominopelvic tumor, with radiological features of malignancy, treated by radical excision with organ preservation and minimal long-standing sequelae. Surgical management of this tumor gives insights into complexities that are likely to be encountered while managing such a case. The neurovascular supply of lower limbs has to be given due consideration and vascular surgery planned so that on-table surprises can be avoided.


The concept of fibroosseous lesions of bone has evolved over the last several decades and now includes two major entities: fibrous dysplasia and ossifying fibroma. Peripheral cemento-ossifying fibroma is a relatively rare tumour classified between fibroosseous lesions. It predominantly affects adolescents and young adults, with peak prevalence between 10 and 19 yrs. The cemento-ossifying fibroma is a central neoplasm of bone as well as periodontium which has caused considerable controversy because of confusion regarding terminology and the criteria for its diagnosis. The cemento-ossifying fibroma is odontogenic in origin, whereas ossifying fibroma is of bony origin. Lesions histologically similar to peripheral ossifying fibroma have been given various names in existing literature. Therefore, we present and discuss in this paper a series of cases of peripheral cemento-ossifying fibroma emphasizing the differential diagnosis. Benign fibroosseous lesions of the jaws present problems in diagnosis and classification. The 1992 WHO classification groups under a single designation (cemento-ossifying fibroma) two histologic types (cementifying fibroma and ossifying fibroma) that may be clinically and radiographically undistinguishable .Peripheral cemento-ossifying fibroma (PCOF) accounts for 3.1% of all oral tumors  and for Clinically, PCOF manifests as a pediculate or sessile nodular mass, which usually originates in the interdental papilla. Its color is similar to that of the mucosa unless the lesion is ulcerated. Most tumors measure less than 2 cm in diameter, although lesions larger than 10 cm are occasionally observed. About 60% of the tumors occur in the maxilla and more than 50% of all cases affect the region of the incisors and canines. A potential of tooth migration PCOF has been reported [Case  1. A 21-year-old male reported to College of Dental Sciences & Hospital, Rau, Indore, India, with his slow growing, painless growth that had been present facial in upper right premolar to molar region. Lesion started as a small papule approximately 1 year earlier. According to the patient, there was no bleeding and pain except difficulty in mastication. Examination revealed approximately 2 × 1.5 cm pedunculated nontender, firm, pinkish red growth present on the buccal gingival in relation to maxillary right canine to 1st molar. The lesion extended up to the level of occlusal plane and revealed indentations made by the occluding mandibular premolar. The surface of the occlusal plane was pinkish red in color (in color .Case  2. A healthy 25- year-old male reported to College of Dental Sciences & Hospital, Rau, Indore, India, with a lump in his back teeth. According to the patient, the reddish purple lump has been present for approximately 6 months and the lump was interfering with his bite and felt uncomfortable. Occasionally bleeding occurred when he brushed his teeth. Clinical examination revealed erythematous interdental papilla in relation to maxillary central incisors 11,12 visible from facial aspect with no evidence of lesion palatally. The lesion appeared exophytic and nodular with irregular surface. It measured approximately 10 mm laterally and 8 mm in anterior-posterior direction and 6 mm thick. It was slightly pedunculated with what appeared to be a broad-based attachment. The lesion was neither fluctuant nor did it blanch on pressure, but had a rubbery consistency. It was tender (s tender . Case  3. A 26-year-old male patient was referred by his general dental practitioner for gingival swelling in relation 15 to 16 region. The past dental history revealed presence of swelling since last one and half year duration. On examination, the associated soft tissue was slightly swollen but there was no ulceration, on palpation the swelling was soft rubbery in consistency, but no tenderness. The lesion was well demarcated and pedunculated measuring approximately 1.5 × 2 cm (5 × 2 cm .Case  4. A 31-year-old male reported to the College of Dental Sciences & Hospital, Rau, Indore, India complaining of inability to chew food since 6 to 8 months. The patient was apparently asymptomatic 18 months back when he developed a small swelling in the mandibular anterior labial region in 31, 41 which gradually increased in size. On examination, a uniform rounded swelling was present in mandibular anterior region due to which the patient could not chew the food. The size of the lesion was 2.5 × 2 cm and the shape was ovoid. The overlying mucosa was pinkish in color and firm in consistency. The texture was smooth. There was no compressibility or depressibility (sibility .Differential diagnosis consisted of irritational fibroma, pyogenic granuloma, peripheral giant cell granuloma, peripheral cemento-ossifying fibroma.ulcerated stratified squamous surface epithelium;benign fibrous connective tissue with varying numbers of fibroblasts;sparse-to-profuse endothelial proliferation;mineralized material consisting of mature, lamellar or woven osteoid, cementum like material, or dystrophic calcifications;acute or chronic inflammatory cells in lesion .A confirmatory diagnosis of peripheral cemento-ossifying fibroma is made by histopathologic evaluation of biopsy specimens. The following features were observed during microscopic examination:Peripheral ossifying fibroma is thought to be either reactive or neoplastic in nature. Considerable confusion has prevailed in the nomenclature of peripheral ossifying fibroma with various synonyms being used, such as peripheral cementifying fibroma, ossifying fibroepithelial polyp, peripheral fibroma with osteogenesis, peripheral fibroma with cementogenesis, peripheral fibroma with calcification, calcifying or ossifying fibroma epulis, and calcifying fibroblastic granuloma . OssifyiThe term cemento-ossifying has been referred to as outdated and scientifically inaccurate  because Though the etiopathogenesis of peripheral ossifying fibroma is uncertain, an origin from cells of periodontal ligament has been suggested . The reaLesions involving the gingival soft tissues are rare compared to the lesions appearing within bone . MesquitEversole and Rovin  stated tPOFs are believed to arise from gingival fibers of the periodontal ligament as hyperplastic growth of tissue that is unique to the gingival mucosa , 18. ThiPOF is a slowly progressing lesion, the growth of which is generally limited. Many cases will progress for long periods before patients seek treatment because of the lack of symptoms associated with the lesion. A slowly growing pink soft-tissue nodule in the anterior maxilla of an adolescent should raise suspicion of a POF. Discussion of the differential diagnosis should be done tactfully to prevent unnecessary distress to the patient and family. Zhang and others  noted th


Synovial Sarcoma is a malignant mesenchymal tumor that comprises 5–10% of all soft tissue sarcomas. The mean age of onset is thirty years old. Intraosseous presentation is very rare and has only been documented a few times. We report herein a case of a 53-year-old man with synovial sarcoma arising in the left proximal tibia. The patient underwent a wide surgical resection and reconstruction, followed by adjuvant chemotherapy. Three years later, the patient developed a local recurrence that resulted in an above-the-knee amputation. Eight months later, the patient has completed chemotherapy and is without signs of recurrence. The current recommended treatment for synovial sarcoma is wide surgical resection followed by chemotherapy as well as long-term followup. Despite improved surgical techniques, long-term survival rates remain low. Synovial sarcoma usually arises in the soft tissues of the extremities and rarely presents as a primary bone tumor. The first reported case of an intraosseous synovial sarcoma occurred in the proximal tibia . Since tSynovial sarcoma is a malignant soft tissue tumor that comprises 5–10% of all soft tissue sarcomas . It mostIt usually arises in the lower extremities . The larA 53-year-old man presented with a 3-month history of left knee pain. He had difficulty weight bearing and complained of swelling of the calf as well as a lack of sensation in the toes. X-rays showed a large, 5 cm round lucency involving the lateral proximal portion of the tibia . The latμm tissue sections, for the detection of possible t. An SS18 rearrangement was observed in 49/50 nuclei examined, confirming the diagnosis of synovial sarcoma  and invasion of bone, nerve, or vascular structures , 17. In Studies have shown clear indications for the use of irradiation therapy for local control of synovial sarcoma. In 2000, Lewis et al. showed a local recurrence of only 10% in their series of 112 patients with primary localized tumors of the extremity . ApproxiWhile the rate of local recurrence has diminished, the rate of distant metastasis is still a challenge for clinicians. Lewis et al. found a 40% chance of metastasis within five years, despite wide surgical resection with negative margins . Five-yeWe chose chemotherapy treatment over radiation following surgical resection in order to decrease the chance of distant metastasis. The primary tumor, being larger than 5 cm, had an increased chance of distant metastasis . There wOverall, these recent studies are showing a positive progression of treatment methods. The lower rates of local recurrence are promising, but the low five-year survival rates and the high chance of distant metastasis still show the need for the development of adequate systemic therapy. Due to the aggressive nature of this disease and the high rate of metastasis, long-term follow-up is essential.


Objectives. To discuss the management of a squamous cell carcinoma in the presence of malignant otitis externa. Study Design. We present only the third reported case in the literature of a synchronous tumour with malignant otitis externa in the literature. Methods. A case report and review of malignant otitis externa and squamous cell carcinomas of the external auditory canal are discussed. Results. A 66-year-old female is presented here with a 2-month history of a painful, discharging left ear refractory to standard antibiotic therapy. Computerised tomography, magnetic resonance imaging, technetium 99 m, and gallium citrate Ga67 scans were consistent with malignant otitis externa. Biopsy in the operating theatre revealed a synchronous squamous cell carcinoma of the external auditory canal. Primary resection of the tumour and surrounding tissues was performed with concomitant treatment with intravenous antibiotics. Conclusions. This is only the third case to be reported in the literature and highlights several important diagnostic and management issues of these two rare conditions. Both conditions may present in a similar manner on clinical assessment and radiological investigations. Aggressive management with surgical resection and treatment with appropriate intravenous antibiotics is necessary to give the best chance for cure. Pseudomonas aeruginosa, although fungal species such as Aspergillus may also be involved [Malignant otitis externa (MOE) is a rare and potentially fatal invasive infection of the skull base. It can arise as a result of an infection of the external auditory canal “malignant otitis externa,” the middle ear, and sinusitis and as a complication of surgery of the skull base –3. Althoinvolved . This caPseudomonas aeruginosa and a moderate growth of Staphylococcus aureus. Her white cell count (WCC) and C-reactive protein were elevated at 11.3 × 103/μL, and 44.6 mg/L respectively. She was not diabetic or immunosuppressed. Computerised tomography (CT) scan showed soft tissue density involving the external auditory canal with bone erosion of the tegmen consistent with inflammatory destruction  scan showed no evidence of metastatic disease and was also correlated with CT and MRI findings . The tumOn subsequent followup there has been no recurrence of the disease, and approximately 3 months of intravenous antibiotic therapy along with the lateral temporal bone resection appear to have cured the malignant otitis externa.MOE is a rare, invasive infection which typically begins as a chronic infection of the external auditory canal (necrotising otitis externa), middle ear, and sinuses or may be as a result of skull base procedures –3. It evIt typically arises in patients who are immunosuppressed. Most commonly this immunosuppression takes the form of advanced age, diabetes mellitus, acquired immunodeficiency syndrome (AIDS), iatrogenic immunosuppression, or immunosuppression due to haematological abnormalities or malnourishment . The latP. aeruginosa. The infection can be polymicrobial with other organisms such as Aspergillus spp (fumigatus), S. aureus, Proteus mirabilis, Klebsiella oxytoca, Burkholderia cepacia, Candida spp, Scedosporium apiospermum, Pseudallescheria boydii, and Malassezia sympodialis. The majority of fungal MOE occurs in immunosuppressed individuals with AIDS [The predominant organism in MOE is ith AIDS . There has been a slow evolution of the treatment of MOE. Advances in antimicrobial therapy and the introduction of multidisciplinary care and long-term antibiotic therapy have seen this disease change into one which carried a high mortality of approximately 50% with frequent recurrence often requiring a major surgical intervention, to one with a relatively low mortality, and virtually no surgical intervention other than biopsy and debridement of necrotic tissue . The intInvolvement of the ear and lateral skull base by squamous cell carcinoma is usually the result of a cutaneous neoplasm that originates from the skin of the pinna or the external auditory canal. Ultraviolet light exposure or thermal injury (cold) and radiation exposure and chronic infection are thought to predispose patients to this disease . Rarely,The authors present only the third case in the literature of a synchronous malignancy and malignant otitis externa , 8. BothClinically, both conditions often present with a painful, discharging ear refractory to standard treatment regimes of ear toilet and antibiotic therapy. Both conditions may be present with cranial nerve palsies, trismus, and lymphadenopathy. Radiologically, both conditions on CT and MRI may have abnormalities of the external auditory canal, soft tissue, and fluid within the middle ear and mastoid cavity, eustachian tube, and parapharyngeal space with or without concomitant bony destruction . There aThis case and the other cases reported in the literature , 8 highl


The case reports present the endodontic management of two maxillary first molars with six canals. The diagnosis of morphology of multiple canal systems was identified under magnification of the dental operating microscope and was confirmed with the help of cone beam computed tomography. This paper discusses the variations in the canal morphology and the use of the latest adjuncts in successfully diagnosing and treating unusual canal anatomy. A thorough knowledge of the root canal anatomy, its variations, the presence of additional roots, and unusual root canal morphology is essential, as it determines the successful outcome of endodontic treatment . To ensuThe morphology of the maxillary first molar has been extensively studied and reported in the literature. Traditionally the maxillary first molar exhibits three roots and three canals. The occurrence of a fourth canal ranges from 50.4% to 95% –7 and a The occurrence of 2 canals in distobuccal root has been less frequent and has been reported in 3.6% of maxillary molars , 10, 11.The present cases report the successful management of maxillary first molars with three roots and six canals. The clinical findings were confirmed with the help of operating microscope and cone beam computed tomography (CBCT).A 43-year-old female patient presented with the chief complaint of pain in the left upper back tooth. The pain was continuous and aggravated on heat stimulation. The patient also complained of pain at night. The patient's medical history was noncontributory.Clinical examination revealed the left maxillary first molar with a deep carious lesion which was tender on percussion. Electric pulp testing gave a premature response, indicative of inflammatory pulpal changes. The radiographic examination revealed a radiolucent lesion on the mesial aspect of the crown extending to the pulp . After tThe tooth was anaesthetised with 1.8 mL of 2% lidocaine containing 1 : 80,000 epinephrine  followed by rubber dam isolation. A conventional endodontic access cavity was prepared. Clinical evaluation of the internal anatomy revealed 3 principal root canal systems: mesiobuccal (MB), distobuccal (DB), and palatal. After probing with a DG 16 endodontic explorer, small hemorrhagic points were noted 2 mm palatal to the MB and DB canals. As the dentin that was occluding the orifice of the palatal canal was removed, a second palatal canal was also identified. This was further evaluated and verified by a Surgical Operating Microscope . There seemed to be 2 distinct orifices in all the roots . A steriTo confirm this unusual morphology and to ascertain the pattern of the canals in a 3-dimensional manner, a cone beam computed tomography (CBCT) imaging of the tooth was advised. An informed consent was obtained from the patient, and a multislice CBCT scan of the maxillary left side was performed (Kodak 9000 3D) with a tube voltage of 80 KV and a tube current of 8 mA. The involved tooth was focused and a 3D morphology was obtained.The CBCT images confirmed the presence of six canals. The scans showed two mesiobuccal, two distobuccal, and two palatal canals . The mesAt the next visit, the working lengths of each canal were estimated by an electronic apex locator  and confirmed with a radiograph . The cleA 28-year-old female patient presented with the chief complaint of pain in the left upper back tooth. The pain was continuous and aggravated on heat stimulation. The patient's medical history was noncontributory. Based on clinical and radiographic examination a diagnosis of irreversible pulpitis was made and endodontic treatment was suggested to the patient.The tooth was anaesthetised with 2% lidocaine containing 1 : 80,000 epinephrine . A conventional endodontic access cavity was prepared under rubber dam isolation. Clinical evaluation of the internal anatomy revealed 3 principal root canal systems: mesiobuccal (MB), distobuccal (DB), and palatal. After probing with a DG 16 endodontic explorer, small hemorrhagic points were noted palatal to the mesiobuccal canal. On evaluation MB2 and MB3 were identified. Further exploration led to the identification of a second palatal canal. The distobuccal orifice also seemed to be indicating multiple canal system . This waFor further evaluation of this unusual morphology, a CBCT imaging of the tooth was advised. An informed consent was obtained from the patient, and a multislice CBCT scan of the maxillary left side was performed (Kodak 9000 3D) with a tube voltage of 80 KV and a tube current of 8 mA.The CBCT images confirmed the presence of six canals. The scans showed three mesiobuccal, two palatal, and an oblong distobuccal canal systems . The MB1At the next visit, the working lengths of each canal were estimated by an electronic apex locator  and confirmed with a radiograph . The cleAnatomical aberrations are commonly observed in maxillary first molar ranging from one to seven canals . It is gPrevalence of additional root canals has been reported and discussed by several authors , 16. CasProper access opening and modifying the shape of the access to approach all orifices is a key to success in identifying and negotiating unusual anatomy of root canals . In the Diagnostic measures such as multiple preoperative radiographs, examination of the pulp floor with a sharp explorer, troughing of grooves with ultrasonic tips, staining the chamber floor with 1% methylene blue dye, performing the hypochlorite champagne bubble test, and visualising canal bleeding points are important aids in locating root orifices . In the An important aid for locating root canals is the Surgical Operating Microscope (SOM). It brings minute details into clear view by enhancing lighting and visibility. Studies have demonstrated that magnification and illumination by the SOM increased the identification of MB2 canals tremendously , 18–20. Radiographic examination is an essential component for management of endodontic problems. But they produce only a 2D image of a 3D object resulting in superimposition of images . CBCT isIn the present cases, CBCT scanning was used for a better understanding of the complex root anatomy. For case 1, the images confirmed the presence of double canal system in three roots. The images showed that the palatal and distobuccal canals present with a Vertucci type II pattern and the mesiobuccal canals follow a Vertucci type IV configuration. Similarly, for case 2, the images confirmed the occurrence of multiple canal systems. The mesiobuccal root showed a Sert and Bayirli  type XV The simultaneous occurrence of double canal systems in all roots of maxillary first molar is an unusual finding, as is the occurrence of 3 mesiobuccal and 2 palatal canals in the same tooth. Thus, it is important to be conscious to variations from the expected and to use all the armamentaria available to locate and treat the entire root canal system.Although the incidence of root variations is rare, their importance should not be underestimated. Careful examination of radiographs and the internal anatomy of teeth are essential. The present cases confirm the necessity for meticulous examination of the pulpal floor at high magnification under sufficient illumination of the operating microscope and emphasize the importance of newer imaging techniques like CBCT in preoperative assessment.


Simultaneous squamous cell carcinoma (SCC) of esophagus and gastroesophageal adenocarcinoma has rarely been reported. It is often difficult to diagnose this coexistence preoperatively due to the presence of esophageal stenosis. Herein, we report a patient with esophageal SCC whose gastroesophageal adenocarcinoma was also detected after pathologic examination of the resected specimen The simultaneous presence of esophageal squamous cell carcinoma (SCC) and gastric adenocarcinoma is very uncommon. There are rare case reports in the English literature.In this report, we present our first experience (during 20 years) of an 80-year-old man with esophageal SCC in which gastroesophageal adenocarcinoma was incidentally detected after surgery in the margin of surgical resection.An 80-year-old man presented with progressive dysphagia, weakness and weight loss since 5 months prior to admission. His past medical history was completely unremarkable. Physical examination showed a cachectic and pale old male. Heart, lung and other parts of the body were unremarkable. Laboratory findings were: WBC: 3400/ml, Hb: 7.3 gr/dl, platelet: 190000/ml. Occult blood was detected in the stool examination. Upper gastrointestinal endoscopy showed an ulcerative infiltrative mass in the lower third of esophagus. The lumen of the esophagus was narrowed by the tumor so that the tube could not be passed through stomach. A biopsy was taken with the pathologic diagnosis of well differentiated SCC.CT scan of the chest and abdomen did not reveal any additional finding without any evidence of metastasis. The patient underwent surgery for resection of the tumor, and distal esophagectomy (Orringer's operation) was performed. The specimen received in the pathology laboratory consisted of a segment of esophagus with an ulcerated infiltrative mass measuring 5Xx3x2.5 cm which has nearly obliterated the lumen with 2-cm distance from the distal resected margin of cardia which grossly seemed unremarkable except for mild firmness. Grossly, no overt mass was detected in the stomach.Microscopic analysis of the sections from the esophagus showed well differentiated SCC invading the adventitia . The disUnfortunately after resection of the esophagus, the patient developed pneumothorax and with the diagnosis of air leak and right main bronchus rupture, emergency right postrolateral thoracotomy performed and chest tube was inserted but the patient did not tolerate and died after cardiorespiratory arrest.Esophageal cancer is the 8th most common cancer and the sixth most common cause of death from cancer worldwide.2-4 SCC is the predominant histologic picture, but its coincidence with gastroesophageal adenocarcinoma has rarely been reported.5 Synchronous cancer was defined as a primary carcinoma of the extraesophageal organs detected within 1 year of the diagnosis of esophageal carcinoma based on the criteria reported by Warren and Gates.In a report from Japan, with a high incidence of both esophageal SCC and gastric adenocarcinoma, just 2.1% of 11732 patients with esophageal SCC, had synchronous cancers of other organs, the most common of which had been gastric early adenocarcinoma, but in WAll the previously reported cases have been in old male patients (>60 years of age). Diet, cigarette smoking and alcohol consumption have been considered as the main risk factors of this coincidence. Our pati


A chondrosarcoma originating from the hyoid bone is very rare. Here, we describe a case of low-grade chondrosarcoma of hyoid origin and discuss its preoperative imaging features, including those on positron emission tomography-computed tomography, and its recurrence rate.A 42-year-old Japanese man noticed a mass in the right submandibular region of his neck. A hard 3.0 × 2.8 cm tumor was noted on the right side of his hyoid bone. The mass was immobile and moved with deglutition.en bloc, and the tumor was diagnosed based on the results of pathological investigations. Close follow-up is recommended in such cases due to the potential for recurrences, because local recurrence occurred in 50% of the reported cases of grade one chondrosarcomas.Even though radiographic studies, including positron emission tomography-computed tomography, were inconclusive, the cartilaginous tumor was surgically removed  Chondrosarcoma, a malignant tumor characterized by the production of a cartilage matrix, accounts for about 11% cases of primary malignant bone tumors [A 42-year-old Japanese man noticed a mass in the right submandibular region of his neck in June 2010. He visited the department of otolaryngology at a nearby general hospital the next month and was referred for medical treatment. More than two weeks later, he visited our University Hospital. A hard 3.0 × 2.8 cm tumor was noted on the right side of his hyoid bone. The mass was immobile and moved with deglutition. No spontaneous or tender pain was noted.Laryngeal endoscopy revealed no contributory abnormal findings. Computed tomography (CT) demonstrated a gourd-shaped distension of bone in the area extending from the body of the hyoid bone to the right greater horn. A multilocular cystic area of low density was observed inside the distension, and central ossification was evident in the anterior lesion Figure . MagnetiSurgery was performed to extirpate the tumor. His geniohyoid, mylohyoid, sternohyoid, and thyrohyoid muscles were detached from his hyoid bone. The tumor mass and surgical margins were resected bilaterally on the left side of the body of his hyoid bone and the right greater horn. There were almost no adhesions between the tumor and the tissue around it. Following a favorable postoperative course, our patient was discharged from hospital.A macroscopic examination identified the cartilaginous tumor as a semi-lobular mass covered by a thin fibrous coat. Histopathological examination revealed that it was relatively well differentiated, but with a somewhat high cell density and many binuclear cells. An open chromatin pattern was evident and intranuclear structures were observed in many cells. The condition was diagnosed as chondrosarcoma, grade one Figure . No addiGenerally, the most useful diagnostic imaging examination for chondrosarcoma is CT, which detects irregular bone destruction. Intratumoral calcification is noted in about 75% of patients using this tool ,3. MRI iIn most cases, bone scintigraphy examination reveals poor uptake in the chondrosarcoma itself and morbid uptake in the destroyed area of the affected bone . FDG-PETGenerally, the occurrence of metastases becomes more probable as the degree of malignancy increases, resulting in poor prognoses . The fiven bloc and the tumor was diagnosed based on the results of pathological investigations. Chondrosarcomas of hyoid origin have a high incidence of local recurrence and are sometimes metastatic. Therefore, it seems necessary to closely follow-up the patient for a prolonged period of time.The present case did not involve union of the lesion to the tissue around it. The tumor and surrounding intact hyoid bone tissue were completely resected. Pathological results led to the diagnosis of a grade one tumor of low malignancy. Even though our radiographic studies, including PET-CT, were inconclusive, we surgically removed the cartilaginous tumor Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.HT was involved in drafting the manuscript and reviewing the literature. HF was a major contributor in revising the manuscript and getting informed consent from our patient. MI was involved in reviewing the literature. YW reviewed the manuscript. All authors were responsible for the diagnosis, treatment and follow-up of the patient described in this case report. All authors read and approved the final manuscript.


Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. Usual treatment is surgery for benign tumors and chemotherapy including mTOR inhibitors for malignant tumors. Here we discuss the radiological and pathological diagnosis, evaluation, and management of a hepatic PEComa. We describe a 51-year-old patient who was diagnosed incidentally after unusual physical exam findings. The term PEComa was introduced by Zamboni four years after a tumor of the perivascular epitheliod cell was first described by Selvaggi et al. in 2011 , 4. Alth51-year-old Caucasian female who was previously healthy presented with complaints of skin thickening of her breasts. MRI and mammogram of both breasts were unrevealing. The patient underwent bilateral random breast skin biopsies. Pathology returned as subacute spongiotic dermatitis. She also complained of tender bony prominences in her scalp and hands. On plain X-rays, these were found to be bone islands. Patient worked as a nurse, and due to vague generalized symptoms she insisted on PET scan to be done which revealed a nonmetabolically active lesion in the liver, slightly less metabolically active than the surrounding liver parenchyma, with no other lesions being identified on PET scan as a primary site . A dedicPrior to 2011 approximately 100 cases of PEComas originating from different sites and less than 20 cases of Hepatic PEComa were reported. PEComas have a wide variety of presentations and behavior. Reports have suggested that criteria for malignancy include tumor greater than 5 cm, mitotic rate of more than 1 per 50 high power field, and necrosis, but this has not been universally adopted . ContrasMany hypotheses exist regarding the cell of origin and possibilities include neural crest, smooth muscle, or pericytic . HistoloA case series of 32 patients by Tan et al. sought to describe the radiologic characteristics of PEComas. Most PEComas were found to be of low density on CT, hypointense on T1 weighted MRI, and hyperintense on T2. Tumors typically had well-defined borders and enhanced heterogeneously on both arterial and venous phases .Primary hepatic PEComas appear to be less common than other PEComas. In 2000, 7 young patients with primary hepatic PEComa were reported . In eachRecently, Tan and Xiao published a 7-case series of primary hepatic PEComas . This reTan and Xiao also described the pathologic appearance of hepatic PEComas. The gross appearances of the tumors were smooth defined masses with clear boundaries. Microscopically cell membranes were defined as round or polygonal cells with distinct, round medium sized nuclei . Cells wThe natural history of primary hepatic PEComas is quite varied and not yet well established or predictable. Presentation ranged from a palpable abdominal mass  to acuteThe mainstay of treatment of PEComa has been resection. In a large case series of 26 patients by Folpe et al. , primaryA recently published review of 234 cases evaluated the benefits of both chemotherapy and radiation therapy for PEComa . They dePEComas express p70S6K which is involved in the mTOR pathway . SeveralPEComa is a rare but increasingly recognized tumor. There are characteristic imaging and pathologic findings to confirm the diagnosis. PEComas can display characteristics of both benign and malignant tumors and the primary treatment is resection. Our case adds to the volume of cases, in particular to primary hepatic PEComas, and helps to increase awareness and understanding of this rare tumor.


Mycobacterium tuberculosis in atypical lesions of oral cavity. Tongue is the most common site of oral tuberculosis. We report a rare presentation of lingual tuberculosis in a 65-year-old male, a chronic tobacco chewer, who came to us with swelling of the tongue which apparently looked like hemimacroglossia, leading to the clinical diagnosis of submucosal carcinoma of tongue. Enlargement of tongue is a slow process resulting from gradual invasion and lodging of bacilli in the tongue. Biopsy and histopathological examination revealed tuberculous etiology of the lesion and the patient responded well to antitubercular therapy.The underdiagnosis of extrapulmonary tuberculosis lesions, along with an emerging global resistance to antitubercular drugs, warrants an increased awareness of the involvement of  Mycobacterium tuberculosis, an acid fast bacillus that is transmitted primarily through the respiratory tract. Tuberculosis affects approximately 8 million people worldwide with relatively high incidence in the Indian subcontinent, South East Asia, and Africa [Tuberculosis (TB), an infectious disease is caused by d Africa . Tubercud Africa . This isA 65-year-old male presented to us with a painful, progressively increasing swelling of the tongue of 8 months duration. There was no history of cough, fever, dysphagia, or weight loss. He was a nonsmoker, but a tobacco chewer for the past 15 years. He was an alcoholic since past 15 years. He was not a known case of diabetes mellitus, but a hypertensive since past 4 years and was on regular treatment for it. On examination, patient was well built with good general condition. Examination of oral cavity revealed an 8 × 6 cm firm swelling over the left side of anterior 2/3rd of tongue, crossing the midline, with smooth surface and well-defined margins. The lesion apparently looked like hemimacroglossia . There wM. tuberculosis could be due to underreporting [A significant proportion of patients 15–25%) exist in whom active TB is manifested in an extrapulmonary site . Oral ca–25% exiseporting . The cleeporting . Morgagneporting . Lingualeporting . Tubercueporting . Primaryeporting . Clinicaeporting . “MacrogTubercular ulcers: It is painful and usually develops as a small tubercle which later on softens to form an ulcer. Typically they are shallow, ovoid, with undermined, margins and lined with pale granulation tissue.Tuberculoma: Originates as a lump anywhere in the tongue resembling lingual gumma. The lump in due course of time softens to form an ulcer.Tuberculous fissure: They usually occur on the side of tongue, can be appreciated only by separating their edges.Tubercle papilloma: An overgrowth of the margins of tubercular fissures.Tubercular cold abscess: Due to breakdown of a tuberculoma.Aird has described five pathological types of lesion due to tuberculosis .Tubercul Investigation for an oral TB should almost universally include a tissue biopsy, which has been found to be 88% specific in published case reports . With reHistory and clinical examination plays a vital role in the diagnosis of oral tuberculosis. However, in our case, the rare presentation of lingual tuberculosis in the form of swelling of the tongue in the form of hemimacroglossia, led us to the clinical misconception. Finally, the diagnosis was established only after a biopsy and histopathological examination which thus emphasizes the role of this gold standard investigation in the diagnosis of such tongue masses. Primary involvement of the tongue in this case could be explained with the history of chronic tobacco chewing, leading on to trauma and subsequent infestation by tubercle bacilli. Such a big lesion has probably resulted from lodging of bacilli in the tongue and gradual invasion into the substance of tongue over the course of many years, although the patient could have noticed it in later stages. If the amount of bacilli is high enough in oral tuberculosis lesion, there is a possibility that more lesions could be induced and/or disseminated to other individuals via saliva. Therefore, no matter whether primary or secondary oral tuberculosis, an early suspicion and timely intervention can lead to favorable outcome in such cases, both for the patient and community. We would like to conclude that though primary tuberculosis of a tongue is a rare entity and such a presentation is even rarer, yet tuberculosis needs to be kept in the differential diagnosis of a tongue mass. Biopsy plays a key role in establishing the diagnosis in such a situation. A meticulous clinical and pathological evaluation of atypical but curable lesion of tongue is being emphasized.


Isolated soft tissue cysticercosis of the trunk in the absence of concurrent central nervous system involvement is uncommon and may be difficult to diagnose. We report 4 such cases in the pediatric age group. Preoperative diagnosis of soft tissue cysticercosis was considered only in 1 patient, the rest were diagnosed only after biopsy. Complete excision (without rupture) was done. All of them underwent a CT scan head along with ophthalmic examination to rule out the more common sites of occurrence of cysticercosis. No further treatment was undertaken as the evaluation was negative. In endemic areas like ours we must suspect this entity not only in the limb muscles, but also in the subcutaneous tissues of the trunk. If diagnosed accurately, it can be treated medically, eliminating the need for surgery.  Human cysticercosis is caused by infestation with larvae of pork tapeworm Taenia solium and is endemic in India.[1] The central nervous system is involved in 60-90% of cases.[2] Soft tissue cysticercosis usually involves skeletal muscles of extremities and less frequently the subcutaneous plane.[3] Isolated soft tissue cysticercosis without central nervous system involvement is uncommon and may be difficult to diagnose.[4] A literature search revealed paucity of data on soft tissue cysticercosis in the pediatric age group. We report four such children who underwent surgical excision of isolated soft tissue cysticercosis involving the trunk  over a period of 2 years.Case 1A 5-year-old boy presented with a painless swelling on the right chest wall, just beneath the sterno-clavicular joint, of 5 months duration. There was no history of preceding trauma. He was a vegetarian residing in an urban locality. On examination there was a spherical, freely mobile swelling, measuring 2 cms in diameter, in the subcutaneous plane. Fine needle aspiration cytology (FNAC) was inconclusive. He underwent excision biopsy with a clinical diagnosis of epidermoid cyst. Histopathology (HPE) however confirmed cysticercus cellulosae surrounded by palisading histiocytic reaction  and inflammation extending into the surrounding musculature along with fibrosis. He did not have any neurological symptoms, seizures or visual disturbances. CT scan head and ophthalmic examination were were unremarkable. Hence no further treatment was undertaken.Case 2A 5-year-old boy was brought with a 2 cm swelling in the chest wall noticed for the last 6 months. He was also vegetarian, living near a milk dairy in the city. Clinical examination revealed a spherical, smooth, mobile and firm swelling arising from the subcutaneous tissues of the left 8th intercostal space in the mid-clavicular line. FNAC showed acellular eosinophillic material without any fragments of cyst wall, scolices or hooks. There were no neurological or ophthalmic abnormalities. Evaluation for cerebral and ocular disease was negative. Excision biopsy confirmed the clinical suspicion of parasitic cyst.Case 3A 4-year-old-boy complained of a painless swelling on his anterior abdominal wall for 7 days. He ate a non-vegetarian diet and belonged to a suburban locality. Clinical findings included a non-tender, spherical swelling, 1.5 cm in diameter, in the right lumbar region close to anterior axillary line.It was arising from the subcutaneous plane and was freely mobile. Hence neither sonographic evaluation nor FNAC were done. With a clinical impression of lipoma,he was taken up for surgical excision, which revealed cysticercus cellulosae with surrounding inflammation. No further treatment was undertaken as the neuro-ophthalmic evaluation was negative.Case 4A 12-year-old girl noticed an abdominal wall swelling for the last one month. It was insidious in onset, painless and non-inflammatory. She was a vegetarian from the suburban area. There were no similar swellings elsewhere in the body. There was a 1.5 x 1.5cm, firm, spherical swelling in the subcutaneous tissues of the left paraumbilical region. The lesion was too small for FNAC or ultrasonography. Clinically thought to be a neurofibroma, the cyst removed at surgery was found to be cysticercosis. Further investigations did not reveal any foci of cysticercosis in the brain or eye.The incidence of cysticercosis in India is alarmingly high though the exact figures are not known.[1] In a study done in rural India, the sero-prevalence of cysticercosis was found to be 22.4% and was known to increase with age.[5] Humans become dead-end hosts of the T. solium larvae when they drink contaminated water or eat raw/ poorly cooked vegetables or pork infested with larvae.[2] As was seen in our patients, cysticercosis is equally common in vegetarians. The reason being lack of basic sanitation facilities that promote faecal contamination of improperly washed uncooked food.[1]The most common site of occurrence of soft tissue cysticercosis is skeletal muscles of the upper extremities.[3] Abdominal and chest wall lesions are seen less often. The subcutaneous nodules are usually painless. However, rupture of the cyst wall with leakage of fluid and surrounding inflammation may present with pain in 20% cases.[6] The differential diagnosis includes lipoma, epidermoid cysts etc, hence the diagnosis was missed in our cases.High-resolution sonography can clinch the diagnosis by demonstrating presence of a scolex within the cyst. It was not done in any of our patients as the diagnosis of subcutaneous cysticercosis was not suspected. Other sonographic features described are surrounding oedema or abscess formation, which are often seen after death of the larvae. Rarely, multiple calcified cysticerci in the soft tissues may be seen even on plain radiograph as rice grain appearance.[8] Isolated soft tissue cysticercosis is often used as a marker of neurocysticercosis and an evaluation for co-existing central nervous system and ocular involvement is recommended.[6] This was done postoperatively in our patients.Fine needle aspiration cytology is extremely useful in reaching a preoperative diagnosis of soft tissue cysticercosis. The aspirate is usually blood stained. Clear fluid and a pearly white membrane are seen in 14.4% and 6% of cases respectively.[9] The presence of tiny parasitic fragments like spiral larval walls and detached hooklets in an inflammatory background of eosinophils and histiocytes can diagnose cysticercosis with certainty.[9] In a study of 132 patients, Khurana et al demonstrated parasitic fragments on FNAC in 75% cases.[9] In our patients FNAC was done only in 2 patients, none of which could confirm the diagnosis.Surgical excision of isolated soft tissue cysticercosis usually suffices if concurrent involvement of central nervous system and ocular disease have been ruled out. Conversely, if a definite diagnosis has been made on the basis of ultrasonography and FNAC without any evidence of abscess formation, surgery may not be required at all.[3] Complete resolution of the cysticercosis has been shown with high dose antihelminthic therapy with either praziquantel (50-75 mg/kg per day for 15-30 days), or albendazole (10-15 mg/kg per day for 8 days).In endemic areas like ours, isolated soft tissue cysticercosis must be considered as a differential diagnosis for subcutaneous nodules on the trunk. If diagnosed accurately, it can be treated medically, eliminating the need for surgery.Source of Support: NilConflict of Interest: None declared


A rare case of mucocele associated with improper feeding habit has been presented. An eight-month old male child presented with swelling in lower lip which was noticed by his mother a week earlier. A thorough clinical examination and history taking gave a diagnosis of mucocele resulting from improper use of feeding bottle. This case highlights and discusses the history, the clinical along with histologic features, and the clinical management of this lesion. Awareness of such an entity and the functional problems associated with the lesion will help the pediatric dentist to prevent any further complications. Mucocele is the common minor salivary gland lesion, which is clinically characterized by a single or multiple, spherical, and fluctuant nodules which are generally asymptomatic . They arYamasoba et al.  highlighThe present case reports the clinical and histopathologic findings of a lower lip mucocele in an 8-month-old child associated with an improper use of feeding bottle.An eight-month-old male child was brought to our Department by his mother with the chief complaint of a swelling on the lower lip for the past one week. The mother gave a history that the child has been weaned from breast feeding two months before and currently using the feeding bottle, which the child used to bite hard and kept in mouth most of the day time. The parent reported difficulty in feeding due to the presence of swelling. Intraoral examination revealed presence of upper and lower primary incisors, and a localized, compressible, and soft fluid-filled nodule of approximately 8 × 8 mm diameter 0  with a tConsidering the size of the swelling and the functional disturbances associated with it, the treatment plan was aimed for a complete surgical excision of the lesion. Excision of the mucocele was performed under local anesthesia, and the wound was sutured using a resorbable suture polyglactin 910 (Vicryl). The excised tissue was subjected to histopathological examination, which showed a mucous excavation cyst, lined by an epithelium Figures  and 3. TA mucocele or mucous cyst is a common, mucus-containing cystic lesion of the minor salivary glands in the oral cavity. Some authors prefer the term mucocele since most of these lesions are not true cysts due to the absence of epithelial lining . The literature review had cited the reason for some of the oral lesions like irritation fibroma and mucocele arising as a result of oral habits such as lip biting/sucking . During Mínguez-Martinez et al.  reviewedThe present case is an extravasation type of lesion, which has a tendency to occur in younger patients, whereas the second type retention mucoceles may occur most often in middle to late life. Few cases have been reported in the first decade of life and their occurrence in new-born babies has rarely been reported , 7. The main area of controversy surrounding the surgical excision of mucocele in very young children is related to the anesthetic modality and the degree of cooperation during the surgical excision. In most of the reported cases the lesion was excised under general anesthesia , 7. The The present case illustrates the importance of having a complete knowledge of the lesion and the need for careful history, which will in many cases lead to the diagnosis of main etiology for the lesion. Surgical removal was necessary in this case because of the associated functional problems, which is confirmed by the improvement in dietary habits postoperatively.


Intravesical retained surgical sponges are very rare and only a few cases have been removed by minimally invasive techniques.We report a case of an intravesical gossypiboma in a 71-year-old man from western Nepal, who presented with urinary retention and persistent lower urinary tract symptoms one year after open cystolithotomy. He was diagnosed with an intravesical mass using ultrasonography. The retained surgical sponge was found during cystoscopy and removed through endoscopy.Intravesical gossypibomas are rare and can mimic a bladder mass. This is one of the few reported cases of cystoscopic removal. Gossypiboma denotes a foreign body made of cotton that is retained inside the patient during surgery . Althougin situ. A urine analysis showed pyuria, with12 to 15 red blood cells, and a urine culture showed growth of Proteus mirabilis sensitive to nitrofurantoin. A renal function test and other investigations were within normal limits. An abdominal X-ray was unremarkable and ultrasound examination showed sludge in his urinary bladder, with a mass of 8 cm × 13 cm. Our patient was diagnosed with a bladder mass and cystoscopy was planned after a week of antibiotics. The cystoscopy revealed a large mass in the anterior wall of his bladder, mimicking a fungating bladder tumor. Upon further examination, pieces of cotton thread were evident and confirmed the mass to be a gossypiboma (Figure A 71-year-old man from western Nepal presented to us with a history of urinary retention for 20 days for which a urinary catheter was placed. He had been experiencing increased urinary frequency, dysuria, suprapubic pain, intermittent fever and hematuria for the last six months. He had undergone an open cystolithotomy for vesical calculus one year previously in a peripheral hospital. On examination, his vitals were stable with a lower midline scar (Figure a Figure . His posGossypiboma is rarely seen in clinical practice. Intra-abdominal gossypiboma is the most common. Intravesical gossypiboma is infrequently reported in the literature , probablThe preoperative diagnosis of retained gauze may be difficult, especially if radio opaque markers are not placed like in our case. Ultrasonography, computed tomography and magnetic resonance imaging can aid in diagnosis but still may be confusing . The preet al. described six patients in whom percutaneous extraction was successful [Once a gossypiboma is identified, it should be removed. Surgery had been the mainstay in the removal of foreign bodies for many years. However, various techniques have been applied for the removal of retained gauze, such as percutaneous, endoscopic and laparoscopic extraction and open surgery, depending on clinical presentations and facilities available. Nosher ccessful . Rafiqueccessful .Clearly, prevention of this condition is preferable; this can be achieved by a meticulous count of surgical materials in addition to thorough exploration of the surgical site at the conclusion of surgery. Although textiles impregnated with radio-opaque markers are widely used by surgeons in the developed world, it is not practiced in our part of the world and thus diagnosis is still difficult. Radio-opaque markers should be routinely used and if in doubt, intraoperative radiologic screening should be done. Gossypiboma is a surgical mishap which can be avoided if guidelines for operative theatre record keeping are carefully followed. The surgical team should not unquestionably accept correct count reports, but should develop the habit of performing a brief but thorough routine post procedure wound body cavity exploration before closure. The routine use of radio-opaque markers, not currently used in our part of the world, is a must. Despite all the technologic advances of the 21st century, human fallibility remains. The possibility of gossypiboma exists even in modern medicine. As litigation is becoming ever more common for this avoidable problem, prevention is highly desirable. Among the many alternative methods of treatment, endoscopic removal is possible in some cases.Intravesical gossypibomas are rare and can mimic a bladder mass. This is one of the few reported cases where removal was performed with the help of cystoscopic guidance.Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.RK is the principal author and assistant during the procedure. He collected the history and information from the patient and was the principal writer and performed the literature review. BKH was the principal surgeon. He reviewed and corrected the article. Both authors read and approved the final manuscript.


Osteoblastoma is a rare bone tumor which is mostly found in the vertebral column and long bone. We describe a 59-year-old woman with osteoblastoma in the right fifth posterior segment of the rib, whose presenting symptoms were right back pain for two years and awakened at night. Chest computer tomography (CT) and thoracic spine magnetic resonance (MR) imaging findings included an expansile lesion of the right fifth rib and an ossified matrix. Surgical resection of the lesion confirmed a benign osteoblastoma. 12 months follow-up revealed disappearance of right back pain. Rib osteoblastoma in plain film has been described previously; however, to our knowledge this is the only case report emphasized in CT and MR imaging. Osteoblastoma is an uncommon bone tumor, which accounts for approximately 1% of all bone tumors ,3. The lA 59-year-old woman was admitted to hospital with a two-year history of right back pain. The pain was durative and had increased steadily in recent few months. She often awoke at night with the pain. There was point tenderness in the region of complaint sometimes occurring with pain radiation to the trunk. The pain cannot be relieved by aspirin. There was no fever or sweat. There was no history of tuberculosis, malignancy or surgery except hypertension. Her young sister had a history of lung cancer; nothing else was remarkable in family history. The woman consented kindly to our case report.Non-contrast enhanced chest CT was obtained using a 16-channel multidetector scan. A paravertebral expansile lesion that involved the right fifth posterior segment of rib and protruded into the thoracic cavity was revealed. Cortical expansion and an ossified matrix were noted (see Figure The surgical approach consisted of a right posterior thoracotomy at the fifth intercostal space. After opening the pleural cavity, the superior, inferior and posterior margins of the tumor were clearly visible. The tumor revealed calcified areas mixed with cystic areas containing hemorrhage. A complete excision of the tumor was performed.Photomicrographs showed a bone-forming tumor with osteoblasts rimming osteoid and bony trabeculae. The tumor consisted of predominantly disorganized osteoid surrounded by numerous large osteoblasts within the vascular stoma. No mitosis or anaplasia were seen. These findings were compatible with benign osteoblastoma Figures .Osteoblastoma is an uncommon primary bone tumor. The most common sites are the verterbral column and long bone, followed by the feet, skull, clavicle and the ribs. The rib is involved in less than 5% of patients ,3. PatieHistologically, osteoblastomas are benign primary bone tumors with some aggressive characteristics that can transform into osteosarcomas. Osteoblastomas will continue to grow and damage the bone and surrounding structures before excision, while the reoccurrence rate after operation is still 10% . TherefoOsteoblastoma may occur in an intramedullary, intracortical, or periosteal location. Therefore the radiological appearance of osteoblastomas are extremely variable and not specific ,7. AccurWe report an osteoblastoma of the rib, which was eosteolytic, expansile and well circumscribed with variable quantities of calcification or ossification areas. Certain typical CT and MRI features of osteoblastoma are well demonstrated, but the ectogenic growth with expansion of the adjacent rib is very rare which has not been previously described.Written informed consent was obtained from the patient for publication of the Case report and any accompanying images. A copy of the written consent is available for review by the Editor in Chief of this journal.CT: Computer Tomography; MR: Magnetic Resonance; STIR: short-tau inversion-recoveryThe authors declare that they have no competing interests.JY carried out studies and participated in the Literature review. LL participated in the Clinical information collecting including follow-up. JW paticipated in the Literature review, and SW paticipated in imaging reading. All authors read and approved the final manuscript.


Hemolymphangioma is a malformation of both lymphatic and blood vessels. The incidence of splenic hemolymphangioma is extremely rare. Laparoscopic partial splenectomy is feasible, reproducible, and safe in children with focal splenic tumors. We report on the case of a 12-year-old male with a large splenic hemolymphangioma successfully managed by laparoscopic partial splenectomy. The patient recovered well after operation. Laparoscopic partial splenectomy is a safe and minimally invasive technique for treatment of splenic hemolymphangioma located in the pole of the spleen. Focal splenic tumors, including splenic cysts and vascular neoplasms, are uncommon and require complete resection to prevent complications or recurrence . HemolymA 12-year-old male was admitted to our hospital with a complaint of abdominal pain for one month. There was no history of trauma, no weight loss, and no family history of cancer. A physical examination showed a tender left upper-quadrant abdominal mass. Laboratory tests, including serum tumor markers, were normal. Computed tomography confirmed a large 15.7 × 8.5 cm cyst occupying the upper pole of the spleen (Figure The patient underwent a laparoscopic partial splenectomy. In brief, the patient was induced under general anesthesia and placed in the supine position. Using Hasson’s technique, the pneumoperitoneum was achieved at a pressure of 8 mmHg and a flow rate of 1.5 L/min. A 10-mm port was placed sub-umbilically for laparoscopy. Four more ports were placed under vision: a 5-mm port in the subcostal border in the right mid-clavicular line, a 5-mm port under the costal border 6 cm in the right mid-clavicular line, a 5-mm port parallel to the umbilicus in the left anterior axillary line, and a 5-mm port parallel to the umbilicus in the left mid-clavicular line. A hilar dissection was carefully performed, and the superior lobar vessels going toward the superior pole and cyst were identified and dissected. The vessels were clipped, which resulted in a well-defined line of demarcation at the upper pole Figure . The cysNo post-splenectomy infectious complications occurred. His postoperative course was uneventful. The patient is asymptomatic on a follow-up of 1 year with no recurrence on ultrasonography and a normal platelet count. Microscopically, some dysplastic lymphatic vessels and blood vessels were observed in the tumor. The pathological diagnosis was hemolymphangioma Figure .Hemolymphangioma is a congenital malformation of the vascular system . The forIn conclusion, laparoscopic partial splenectomy is a safe and minimally invasive technique for the treatment of splenic hemolymphangioma located at the pole of the spleen.Written informed consent was obtained from the patient’s parents for the publication of this report and any accompanying images.The authors declare that they have no competing interests.YZ and X-MC drafted and revised the manuscript. D-LS and Y C were responsible for acquisition and interpretation of data. All authors read and approved the final manuscript.


A one-year-old child being investigated for urinary tract infection was diagnosed with a multiseptate gallbladder. The patient remains asymptomatic, and investigations demonstrate no associated anomalies. Forty-three cases, including 13 cases in children were identified in the literature. Their presentation and management were reviewed. Multiseptate gallbladder (MSG) is a rare congenital anomaly with less than 50 cases described in the English literature. Of these, 13 are in paediatric patients . We repoEscherichia coli urinary tract infection (UTI). Renal tract ultrasound was normal, but the gallbladder (GB) was incidentally noted to have “multiple thin smooth septa, giving a honeycomb appearance,” consistent with MSG . Magnetic resonance cholangiopancreaticography (MRCP) confirmed the diagnosis of MSG, and excluded intra- and extrahepatic biliary and pancreatic anomalies. Nuclear medicine HIDA (hepatobiliary imino-diacetic acid) scan revealed no evidence of obstruction to bile flow at any level of the biliary tree.Multiseptate gallbladder was first described in 1963 by Simon and Tandon . It is cIncluding the current case, 44 cases are described in the English literature, with a male-to-female ratio of almost 1 : 2. Overall the mean age at diagnosis of these individuals was 28.6 years (range from 15 days to 70 years). Thirty of these cases were in adults –36. ThirBiliary symptoms such as right upper quadrant pain, nausea and vomiting are the most common complaints in this condition, with 31 of the 44 cases presenting in this manner. Only three of the 44 cases were associated with cholelithiasis , 12, 13,There is no reported association between uncomplicated MSG and malignancy; however, there is a known link between biliary tract anomalies and cholangiocarcinoma. The incidence of malignancy in choledochal cyst is reported between 10% and 30%, and anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy , 17. ConThe majority of reported cases were diagnosed on ultrasound. Differential diagnosis includes desquamated gallbladder mucosa, polypoid cholesterolosis, hydatid cyst and acute hepatitis , 19. KocSaimura et al.  conducteIn symptomatic patients diagnosed with MSG, cholecystectomy provides relief of symptoms . The thrMSG is a rare biliary anomaly that may be diagnosed in the first decades of life. Associated biliary tract anomalies should be excluded, particularly when the diagnosis is made in childhood. Cholelithiasis is rarely associated with MSG, and has never been reported in a child. Investigation with ultrasound, MRCP, and HIDA scan is recommended. In the absence of symptoms attributable to the MSG, or an associated biliary tract anomaly, nonoperative management in children and regular followup is reasonable. Symptomatic uncomplicated MSG is successfully treated with cholecystectomy.


The results of pathological and immunohistochemical studies were consistent with the ependymal nature of neoplastic cells.Myxopapillary ependymomas (MPEs) are uncommon and account for ∼15% of all ependymomas. The current study presents a case of rare spinal MPE with abnormal hyaline degeneration. The patient was a 72-year-old male with a 10-month history of lower back pain. Magnetic resonance imaging revealed a mass involving the L4 and L5 vertebrae with local bone destruction. The tumor was completely resected. Histologically, the majority of the tumor exhibited low cellularity. A marked change in hyaline was observed in the blood vessels and stroma. In specific areas, the tumor showed reticular or tubular patterning embedded in hyaline materials. The tumor cells were cuboidal to columnar in shape with strong immunostaining for glial fibrillary acidic protein and S-100. A fluorescence  MPE is a rare benign variant of ependymoma that occurs most commonly in the cauda equina and filum terminale of the spinal cord ,2. The mA 72-year-old male presented with a 10-month history of lower back pain and dysesthesia at the lower abdominal level. The pain was of sudden onset with radiation to the legs. Weakness in the legs was also reported and the clinical signs were worsened by sitting. There was no history of trauma. The patient showed intermittent claudication after walking 200 m. Magnetic resonance imaging revealed a mass involving the L4 and L5 vertebrae with local bone destruction. The mass was hypointense on T1-weighted images, hyperintense on T2-weighted images and enhanced heterogeneously on post-contrast T1-weighted images . A totalin situ hybridization. No amplification of the EGFR gene was observed (Microscopically, the tumor largely consisted of areas with low cellularity. In less cellular areas, marked hyaline changes were observed in the blood vascular walls and stroma . In otheobserved . The reset al (MPE is a benign variant of ependymoma that has a peak incidence between the third and fifth decades of life. It generally occurs in the filum terminale, but has also been identified in extra-spinal locations, including subcutaneous tissue and the brain ,7. Cliniet al  previouset al . Certainet al ,12. In tet al . Metastaet al ,15.In conclusion, specific regressive changes are observed in MPE and marked hyaline degeneration may lead to an acellular growth pattern, therefore, it is important to rule out the possibility of other tumors. The best curative treatment lies in a complete surgical resection, and long-term follow-up of the whole neuraxis must be performed.