Neurofibroma is a benign tumor of the peripheral nerve sheath characterized by proliferation of Schwann cells, perineural cells and endoneurial fibroblasts. Different types of neurofibromas can be identified, including localized, plexiform, and diffuse types. Diffuse neurofibroma is an uncommon form of neurofibroma that occurs primarily in children and young adults. The head and neck regions are the most common sites of involvement. Diffuse neurofibroma is an ill-defined infiltrative lesion and tends to involve the skin and subcutaneous tissues. It produces localized thickening and induration of the skin. We present a case of a 12-year-old boy who had a diffuse neurofibroma on the scalp since the age of 2 years. Diffuse neurofibroma is a newly recognized distinct clinical entity. Diffuse neurofibroma is an uncommon, but distinct, variety of neurofibroma usually affecting the trunk, head, and neck regions of adolescents and young adults. It is not clear exactly how often diffuse neurofibromas are associated with neurofibromatosis, although it has been suggested that about 10% of patients with diffuse neurofibromas have neurofibromatosis 1 .1There are very few previously reported cases of diffuse neurofibroma of the scalp in the Indian literature. We report a case of a diffuse neurofibroma on the scalp of a young boy, which was confirmed by radiologic imaging and histopathologic examination. The case is reported for its rarity and its unique presentation.cafe-au-lait spots or axillary/inguinal freckling. An ophthalmologic opinion was also sought for which did not reveal any abnormality.A 12-year-old boy was seen at our outpatient clinic because of a bulging mass with loss of overlying hair on the posterior aspect of his scalp. The mass was first observed at the age of 2 years, and it had slowly increased in size since then. Physical examination revealed a soft 4 × 4 cm skin colored mass with mild tenderness on the occipital scalp . There wAt the time, the tentative differential diagnoses included Alopecia Areata, hemangioma, lipoma, and eccrine spiradenoma.Plain skiagram of the skull showed no bony erosion. An ultrasound was performed, which showed a hypoechoic mass suggestive of hemangioma. A computed tomography (CT) scan performed subsequently failed to reveal any intracranial extension. Rest of the laboratory data was normal.The patient subsequently underwent surgery for wide excision of the mass. Histologic examination of the excised specimen  showed aThe tumor was completely excised. No recurrence was observed during the first year of follow-up. The patient’s physical and mental development showed no abnormalities, and a diagnosis of Von Recklinghausen’s neurofibromatosis could not be established.Neurofibromas are benign tumors of the peripheral nerves that have a neuroectodermal origin. On the basis of the histology, three types of neurofibroma can be distinguished: the localized, plexiform, and diffuse types. The locaThe essential cells in neurofibromas are of Schwann-cell origin. Diffuse On histopathologic examination, the tumor is composed of elongated, spindle-shaped cells with round or fusiform nuclei and eosinophilic cytoplasm within a loose matrix of fine fibrillary collagen. Meissner bodies are characteristic, but are not always present. Neurofibromatous tissues show immunoreactivity with S-100 protein, a sensitive, but non-specific, marker of benign nerve sheath tumors.The treatment of large neurofibromas consists of partial or complete surgical excision. Even after complete excision, clinical recurrence may develop because of the infiltrative growth pattern of the tumor. Because of possible recurrence, yearly follow-up is recommended.cafe-au-lait spots, Lisch nodules, and bilateral acoustic to exclude neurofibromatoses.Because of the lack of distinctive clinical characteristics, especially in isolated lesions, it is difficult to diagnose the lesion preoperatively, and such lesions, remain largely, a histopathologic diagnosis. Proper history-taking, physical examination, opthalmologic and radiodiagnostic investigations are warranted in such cases, with special attention to detection of Thus, diffuse neurofibroma should always be kept in mind in the differential diagnosis of any indurated mass on the scalp. The present case has been presented for its rarity and its unique presentation.


We present a young patient with respiratory complaints that was found to have a mass in her right posterior mediastinum. The mass was diagnosed to be ectopic histologically unremarkable hepatic tissue. We have also reviewed several of the few intrathoracic ectopic liver cases in the literature, along with a brief discussion of the significance of such a finding. A young patient admitted from the emergency department with complaints of difficulty breathing and shortness of breath. Physical examination was unremarkable, including auscultation, palpation and percussion of her chest. She was ambulatory at admission and awake, alert, and oriented. Her medical history included diabetes, anemia, hyperlipidemia and asthma. Her past surgical history included endometrial ablation and multiple kidney biopsies. She was taking simvastatin, low dose aspirin, lisinopril, metformin, and a number of asthma medications. In the past she had taken antidepressants and antipsychotics. Her labs were mostly within normal limits with the exception of microcytic anemia and slight leukocytosis with slight neutrophilia.Her symptoms were attributed to her asthma, for which she was treated, but an X-ray and chest CT were ordered. The X-ray was unremarkable except two left sided possible granulomas. The CT scan , in addiThe diagnosis at admission was a benign mediastinal neoplasia, possibly a benign paraganglioma. A video assisted thorocoscopy with possible thoracotomy and excision was consented to by the patient and performed. The mass was well circumscribed and soft and was resected in its totality. The surgeon's finding agreed the CT in seeing no transdiaphragmatic connection between the thoracic mass and the liver. It was noted in the operative record that the mass “appeared to have the consistency of liver”. Two specimens were sent to pathology for further investigation. There were no postoperative complications.Grossly, the specimens were two fragments of well encapsulated nodule measuring 4 × 3.8 × 3.2 cm in aggregates. Cut section revealed soft, fleshy, dark-brown tissue. Microscopic examination revealed sheets of benign polygonal cells about 25–30 microns, arranged in three dimensional plates that radiated away . The celDifficulty breathing, the patient's presenting complaint, could indicate abnormalities in any number of thoracic structures. With the results of the CT scan, it became apparent that this symptom was a mass effect in her mediastinum on her lungs. After the surgical excision of the mass of hepatic tissue, there were no further symptoms.  Other cases of ectopic liver have been described in the literature, but are uncommon and generally occur in adjacent structures, most often the gall bladder, or elsewhere in the abdomen . Ectopic Etiologies of ectopic liver are varied. They may be congenital, as the liver and lungs develop embryologically in close proximity , 6, but Although the hepatic tissue itself was benign, there is still a need for treatment. Clearly in this case there was a mass effect that produced respiratory symptoms, but could have involved any other mediastinal or thoracic structure leading to symptoms in the gastrointestinal, cardiovascular or neurological systems. There is an additional risk, beyond the mass effect, that the ectopic tissue could undergo a malignant transformation and there have been numerous reports , 9 of he The differential diagnosis for a mediastinal mass includes various tumors , lymphadenopathy (infectious or neoplastic as in Hodgkin disease), vascular aneurysms, cysts and spinal lesions. As this and other cases have shown, ectopic liver, though rare, could be added to this list. Although excisional biopsy was the right choice in this case, there may have been room for further preoperative investigation. The preferred diagnostic method would be endoscopic ultrasound with needle biopsy, although percutaneous fine needle biopsy is also an option. The endoscopic approach has been shown to be effective in investigating mediastinal masses of various origins . Should Mebrophenin scintigraphy can localize hepatic tissue rather specifically, and could create a clear and non-invasive view of the ectopic liver, as well as highlight any other foci of hepatic tissue than may need to be addressed . Obvious


The tumors in which odontogenic differentiation is fully expressed are the odontomas. Odontomas are considered as hamartomas rather than a true neoplasm. These tumors are composed of enamel, dentine, cementum and pulp tissue. It is most commonly associated with the eruption of the teeth. They are usually discovered on routine radiographic examination. In exceptional cases, the odontoma erupts in to the mouth. Nine cases of erupted compound odontomas are reported in the English literature, and the present paper reports another case of an erupted compound odontoma in a 22-year-old female patient. Odontoblastic hyperactivity and alterations in genetic component is responsible for controlling dental development. Persistence of a portion of lamina may be an important factor in the etiology of a compound odontoma.5Most odontomas are asymptomatic; occasionally, however, signs and symptoms relating to their presence do occur.Histologically, they are composed of different dental tissues, including enamel, dentine, and cementum and, in some cases, pulp tissue.4Odontomas erupting into the oral cavity are extremely uncommon, with the first case being reported in 1980 by Rumel et al.2 Howards lists odontoma as fourth category of supernumerary teeth.6 However, this classification is universally not accepted. Although there are many reported cases of odontomas in the literature, erupting odontoma is a rare occurrence. In this paper, a case of an erupted compound odontoma in a young female is reported.According to world health organization (WHO) classification, odontomas are classified as complex odontoma and compound odontomas. A malformation in which all dental tissues are formed, but occurring in less orderly pattern is complex odontoma. A malformation in which all dental tissues are arranged in a more orderly pattern than complex odontoma is compound odontoma.A 22-year-old female who was apparently healthy reported to the Department of Oral Medicine and Radiology with the chief complaint of forwardly placed upper and lower front teeth. Medical history was non contributory and there were no hereditary disease in antecedents. Extra-oral examination was unremarkable. Clinical intraoral examination revealed normal compliment of teeth in upper and lower arch with multiple yellowish-brown, dental tissue-like lesion which had a calcified appearance with an irregular surface in the third quadrant. It was situated on the lingual aspect in between the lower left lateral incisor and the lower left canine, which was erupting into the oral cavity . LesionsPatient was advised with a set of radiographs including intraoral periapical and occlusal radiographs to assess the precise location and extent of the lesion, as well as its relations to the surrounding anatomical structures Figures  & 3. Mul7 Eruption of odontomas is infrequent in the literature.The term odontoma was first used by Paul Broca in 1867. Later, In 1914 Gabell, James and Pyne grouped odontoma according to their developmental origin as epithelial, composite  and connective tissue. In 1946, Thoma & Goldman formulated a classification for odontomas.6 Later in 2009, Gabriel Serra-Serraet al8 reported in stating that, since 1980 only 17 cases of erupted odontomas were documented in the literature, and including their 3 patients, it would be 20 cases in the literature. Of the 20 reported cases of erupted odontoma, 9 correspond to compound odontomas and 11 to complex odontomas. The case in discussion here is the 21st erupted odontomas case and the 10th case of erupted compound odontoma reported in the English literature. A case of erupting multiple odontoma is also reported in the literature.9The first case of an erupted odontoma was described in 1980 by Rumel et al.10 Pain and swelling are the most common symptoms when odontomas erupt, followed by malocclusion. Recurrent infection following eruption into the oral cavity has been reported but the patient was asymptomatic in this case.11 There are two types of odontomas: complex odontomas and compound odontomas – the latter being twice as frequent as the former. Compound odontomas show a predilection in the anterior section of the upper maxilla while complex odontomas are typically found in the posterior mandibular region.12 In the presented case the erupted odontoma was the compound type and it presented on the mandibular anterior region, which is a rare occurrence.Odontomas are benign tumors containing various tissue components of the teeth. They are most common odontogenic tumors constituting 22% of all odontogenic tumors of the jaws.13 First stage is characterized by radiolucency due to the absence of dental tissue calcification, the second or intermediate stage shows partial calcification and the third or classically radiopaque stage exhibits predominant tissue calcification with the surrounding radiolucent halo described above.14 In the present reported case, the radiographic appearance was seen as a multiple dense radiopaque structure outside the jaw bone, with clear external margins presenting normal organization of dental tissues, i.e. enamel, dentine, and pulp. Treatment of choice is surgical removal of the lesion in all cases, followed by histopathological study to confirm the diagnosis. In this case, surgical removal of the lesion was performed. The excised specimen was subjected to histopathologic examination, the report of which matched that of radiographic diagnosis.Radiographically, odontoma presents as a well-defined radiopacity situated in bone but with a density that is greater than bone and equal to or greater than that of a tooth. It contains foci of variable density. It is present with a radiolucent halo, typically surrounded by a thin sclerotic line, surrounding the radiopacity. Radiolucent zone is the connective tissue capsule of a normal tooth follicle. Thin sclerotic line resembles the corticated border seen in a normal tooth crypt. Developmental stages can be identified based on radiologic features and the degree of calcification of the lesion at the time of diagnosis.A rare case of erupted compound odontomas has been reported. The most important and interesting feature in this case was that the odontomas were erupted from the lower anterior region of the jaw into the oral cavity like a tooth, which is a very rare occurrence, causing a mild disturbance to the adjacent teeth. A surgical intervention was done and the odontomas were extracted and further orthodontic treatment was followed for the patient.


Introduction. Masses arising from structures adjacent to the female urethra can cause obstructive urinary symptoms. Subpubic cartilaginous pseudocyst is a rare degenerative lesion of pubic symphysis that may cause these symptoms. Materials and Methods. A 61-year- and 57-year-old women presented with symptoms of difficult micturition and dyspareunia. Physical examination revealed a painless smooth, rounded, firm, and cystic mass, at the anterior vaginal wall of about 4 cm width. The mass caused inward deviation of the external urethral meatus. Cystoscopy and MRI were done. Results. Cystoscopy of case 1 (61 y) demonstrated anterior external urethral compression with normal urethral mucosa. Cystoscopy was not possible in case 2 (57 y) because the urethra could not be entered under local anesthesia. MRI showed almost the same findings in both cases: midline, rounded, and cystic mass ~3 × 3 × 4 cm, anterosuperior to the urethra, and posteroinferior to the pubic symphysis, with normal features of the urinary bladder. Open surgical excision of theses lesions was performed in both patients. Histopathologic assessment of the specimen obtained from both patients showed degenerated hyaline with areas of fibrinous and mucoid degeneration, a picture suggestive of cartilaginous subpubic pseudocyst. After 11-month and 4-month followup of patients numbers 1 and 2, respectively, there is no evidence of local recurrence of the lesion, either clinically or radiologically and both patients void empty. Conclusions. Subpubic cartilaginous pseudocysts are rare benign lesions with only 13 cases were reported in the literature. Patients present with a spectrum of gynecological and/or urological manifestations. Sizable lesions severely compressing the urethra need surgical excision to restore the voiding function. The bladder outlet obstruction (BOO) is one of the voiding dysfunctions that presents with a spectrum of symptoms of hesitancy, slow or intermittent urinary stream, straining, and terminal dribble . BOO is The pubic symphysis is a midline joint connecting the pubic bones of the pelvis, with an intervening fibrocartilaginous disc. It can resist tension and shearing; however, it has the ability to widen during pregnancy .Subpubic cartilaginous pseudocyst is a rare degenerative lesion related to the pubic symphysis. It was first described by Algucial-Garcia and Littman  in the yA 61-year-old, postmenopausal, lady, G 3, P 3, with normal vaginal deliveries, presented with complaints of difficult micturition and dyspareunia. On physical examination, a painless, smooth, rounded, firm, and cystic mass was observed at the anterior vaginal wall of about 4 cm width; it appeared to be related to the pubic symphysis. The mass caused inward deviation of the external urethral meatus. Cystoscopy revealed compressed external urethral meatus with normal urethral mucosa. Urodynamic data are not available for this patient; however the history and physical examination were suggestive of BOO. Magnetic resonance imaging (MRI) showed a midline, rounded, cystic mass, of 3.2 × 3.0 × 3.9 cm dimensions, located anterosuperior to the urethra and posteroinferior to the pubic symphysis Figures . There wSurgical excision of the mass was done under spinal anesthesia. A transurethral catheter was inserted and a suprapubic horizontal incision was made. A blunt dissection was made ventrally of the pubic bone down to the cyst. When the cyst was opened it showed clear liquid content. The cyst was then sharply dissected-off of the pubic ramus with scissors and curettage. The vaginal wall and urethra were intact and felt separate from the cyst. The periosteum of the pubic symphysis was reapproximated with vicryl.Histopathologic examination of the specimen showed degenerated hyaline with areas of fibrinous and mucoid degeneration, a picture suggestive of cartilaginous subpubic pseudocyst . ThirteeA 56-year-old postmenopausal lady, G1 P1, with normal vaginal delivery, presented with complaint of difficult micturition in sitting position; she had to lean backward to initiate and to maintain the act of voiding. On physical examination, a painless, smooth, rounded, firm, and cystic mass was noticed at the anterior vaginal wall, of 4 cm width, which appeared to be related to the pubic symphysis. The mass caused inward deviation of the external urethral meatus.2O. Cystoscopy was not possible due to severely compressed, posteriorly deviated urethra.Urodynamics investigation revealed a bladder capacity of 243 cc, evidence of detrusor overactivity, postvoid residual urine of 120 cc, and Pdet. Qmax of 21 cm HMRI showed a midline, cystic mass that measured about 3.0 × 3.8 × 2.7 cm, located anterosuperior to the urethra and posteroinferior to the pubic symphysis, with a wall thickness of 9 mm. Figures .Surgical excision of the mass was done under general anesthesia. A urethral catheter was inserted. Through a Pfannenstiel incision, sharp dissection was done with scissors deep until the level of the cyst was reached Figures . The blaHistopathologic assessment of the specimen revealed a degenerated hyaline with areas of fibrinous and mucoid degeneration, a picture suggestive of cartilaginous subpubic pseudocyst . Five daThe subpubic cartilaginous pseudocyst is a rare degenerative lesion of the pubic symphysis; it is usually associated with narrowing and subchondral sclerosis of the pubic symphyseal joint. The fibrocartilaginous disc of the pubic symphysis joint may undergo mucinous cystic changes as a result of pregnancy, vaginal delivery, or pelvic trauma . The mucA comprehensive search of the literature revealed that a total of 11 cases –12 of siClinical assessment of our two patients revealed the presence of the midline, hard, cystic lesion that caused deviation of the urethral external meatus downward and backward, to an extent that urethroscopy was not possible in case number 2. Urodynamic evaluation of patient number 2 revealed an obstructive pattern which was taken into account when we made a final decision of surgical interference. This test was not done in most of the previous cases reported in the literature and we think it should be applied together with urethroscopy as routine investigations in patients with suspicious subpubic cartilaginous pseudocysts. In the two patients, the masses were sizable enough to compress the urethra and disturb the act of voiding which finally necessitated surgical removal of these masses.The histopathologic patterns of the masses in both cases were almost the same being a composite of cartilaginous wall with intersections of connective tissue showing cystic degenerative changes and necrosis. This also goes in line with the histopathologic patterns of the previously reported cases in the literature which supports the assumption that these lesions develop as a result of degenerative changes of the fibrocartilaginous disc of the pubic symphyseal joint that may undergo mucinous cystic changes .Subpubic (or parapubic) cartilaginous pseudocysts are rare lesions with only 13 cases being reported in the literature since the year 1996 till now. The patients present with a spectrum of gynecological and/or urological manifestations. Characteristic imaging features of these lesions are being developed which might save a number of unneeded biopsies and even unnecessary surgical excision of these benign lesion. However, in some patients with sizable lesions, the urethra could be severely compressed, and surgical excision should be considered in order to restore the voiding function in these patients.


Primary splenic diffuse large B-cell lymphoma (DLBCL) is a rare clinical condition, which is generally treated by six to eight cycles of chemotherapy involving a combination of rituximab and the cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) regimen. However, the treatment for chemorefractory primary splenic DLBCL remains controversial. Therapeutic splenic irradiation (SI) might be a reasonable and possibly the only treatment option with curative intention for patients with chemorefractory primary splenic DLBCL. However, the efficacy and safety of therapeutic SI are unclear. Herein, we present the case of a primary splenic DLBCL patient who was refractory to multiple chemotherapy regimens but achieved complete remission after administration of therapeutic SI. However, his condition was complicated with severe gastric variceal bleeding due to splenic venous thrombosis, which was successfully treated via splenectomy and short gastric vein ligation. On the basis of our findings, we concluded that the splenic venous thrombosis-induced gastric variceal bleeding was a rare but life-threatening adverse effect of the therapeutic SI administered for primary splenic DLBCL. Surgical intervention involving splenectomy and short gastric vein ligation is mandatory and should be performed as soon as possible for such patients. A combiet al.  reported3, normal range: 4000 to 8000/mm3; hemoglobin level: 12.7 g/dL, normal range: 12 to 16 g/dL; platelet count: 319000/mm3, normal range: 140000 to 400000/mm3; neutrophil: 65.3%; lymphocyte: 25.2%). The patient’s liver and renal functions were within the normal ranges. However, the level of lactate dehydrogenase was elevated . Examinations for detecting coagulopathy yielded normal prothrombin time , activated partial thromboplastin time , protein C level , protein S level , and antithrombin III level . Exploratory laparotomy and biopsy confirmed the diagnosis of DLBCL, which was immunohistochemically positive for CD20 and bcl6 but negative for CD3, CD30 and bcl2. The Ki-67 index was 80%. The tumor was staged as stage IIX because of the absence of bone marrow and chest involvement. The age-adjusted International Prognostic Index score was 2 [A 32-year-old man who was a hepatitis B carrier with Child-Pugh A liver cirrhosis presented with abdominal fullness of three-month duration. Abdominal computed tomography showed the presence of a large mass over his spleen and adjacent lymph nodes Figure . Laborat-2·d-1) for 2 d); however, this procedure was not successful. Salvage chemotherapy involving two cycles of etoposide, cisplatin, methylprednisolone, and cytarabine (ESHAP) regimen was administered, but a residual tumor was still detected over his spleen via PET and computed tomography one month after the last ESHAP treatment. Subsequently, therapeutic SI comprising 2400 cGy in 15 fractions was administered. One month later, the patient developed upper gastrointestinal bleeding. Upper gastrointestinal endoscopy showed large gastric varices (GVs) with a red color sign  and computed tomography detected a residual tumor over the spleen after therapy completion. The patient underwent autologous hematopoietic stem cell harvest after high-dose cyclophosphamide mobilization  participated in drafting the manuscript and in treating the patients. All authors read and approved the final manuscript.


We report a case of a rapidly progressing giant retroperitoneal liposarcoma weighing 22 kg in a 41-year-old Japanese man, successfully treated with surgical excision. To our knowledge, this is the largest liposarcoma in the Japanese population reported in the literature. Liposarcoma is a common soft tissue sarcoma which occurs in adults , 2. Its 2. A CT scan detected a 43 × 37 × 31 cm tumor in the right retroperitoneum, with the right kidney significantly displaced to the left and anterior side . After hospitalization, the patient was diagnosed with a liposarcoma by CT-guided needle biopsy. The patient was emergently transferred to our hospital due to worsening respiratory status. On admission his body surface area was 2.18 mior side . Whole-bior side .We concluded that surgical resection was the only option for saving the patient's life, so we performed a tumorectomy 3 weeks later through a midline incision. The operation took 3 hours and 20 minutes, and the estimated blood loss was 3990 mL requiring 800 mL blood transfusion. During surgery, the tumor and right kidney were completely resected.2. A CT scan performed 12 months after surgery detected no recurrence.Pathological examination showed that the resected mass weighed 22 kg and was 45 × 40 × 30 cm in size . GrosslyLiposarcoma is the most common soft tissue sarcoma and accounts for 10–20% of all cases. It commonly occurs between 40–60 years of age and has a 1:1 ratio between genders . AlthougAccording to the WHO classification, liposarcomas can be classified into five histologic types: well differentiated, myxoid, round cell, pleomorphic, and dedifferentiated . The myxDalal argued, on the basis of a study conducted at a single facility, that the most common forms of liposarcoma are well differentiated (46%), dedifferentiated (18%), myxoid (18%), round cell (10%), and pleomorphic (8%). A chimera gene, TLS-CHOP, which is only found in myxoid-type sarcomas, was discovered in a myxoid-type liposarcoma. This gene is presumed to be responsible for tumor growth –12. It cSurgical resection is still the principal treatment, and combined resection of involved organs is necessary for an R0 resection in many cases. A previous report stated that combined resection of adjacent organs is required in 75% of retroperitoneal tumors treated by tumorectomy .Ecteinascidia turbinate), has shown definite activity in soft tissue sarcoma in clinical trials [Multimodality therapies such as chemotherapy with ifosfamide, anthracycline, and doxorubicin, as well as the combination of intraoperative and intensity-modulated radiotherapy, are also effective . Recentll trials . Howeverl trials . Histolol trials . 83% of l trials . Here, w


Chondrosarcomas of the spine are rare and difficult to treat. In this paper a case of thoracic chondrosarcoma is presented. Chondrosarcomas of the spine are generally smaller, more difficult to excise and are followed by higher local recurrence compared with chondrosarcomas of the peripheral skeleton. The tumor is radio- and chemoresistant, making the surgical treatment of utmost importance. The most important prognostic factor for local control is wide or marginal tumor resection. Our patient was treated in two stages, with total excision of the tumor, using cryosurgery. Liquid nitrogen was used to freeze the damaged tissue at a cellular level and made the excision more efficient. Chondrosarcoma is the most frequent primary malignant bone tumour. It constitutes a family of malignant tumors in which the cells differentiate into cartilage and may originate from healthy bones (primary) or from chondromas or osteochondromas with sarcomatous degeneration (secondary). Chondrosarcomas are classified into three categories: central, peripheral, and periostal as well as two rare entities: mesenchymal chondrosarcoma and clear-cell chondrosarcoma. Primary chondrosarcoma of the spine is an extremely rare tumor. Only six to ten percent of chondrosarcomas arise from the mobile spine. The maximum incidence of chondrosarcoma is found in adult, male patients with a tumor diameter being generally greater than 5 cm.Radiographically it is identified as an area of bone destruction and a calcified soft tissue mass. The soft tissue mass may be absent in lesions originating from a vertebral body. Magnetic resonance imaging (MRI) can best demonstrate the extent of the lesion and of the cord compression. Usually chondrosarcoma does not manifest signs of cord compression at early onset . Chondrosarcoma of the spine is challenging to manage. The clinical course of chondrosarcomas originating from the spine is usually long because of the slow growth of the tumour . Local rWe report a case of thoracic chondrosarcoma, which was treated with total resection, using cryosurgrery. Cryosurgery is found to be effective against a localized disease and solid tumors larger than 1 cm. Liquid nitrogen was used to freeze the damaged tissue at a cellular level and form ice crystals inside the cells of the tumor making the excision more efficient and with lower rates of side effects. More damage to the tumor occurs when blood vessels supplying the diseased tissue freeze.Such malignant tumors occurring from the spine pose difficult issues in terms of surgical treatment , 9. TherA 37-year-old male consulted our department with a 7-month history of mild thoracic spine pain after a fall; in addition to the thoracic spine pain he noticed a painful, slowly growing mass. He had no past medical history, and physical examination was unremarkable except for the presence of a hard and tender thoracic spine mass. His father died of hepatic cancer. Clinical blood examination did not show any abnormality, including alkaline phosphatase.Initial posteroanterior chest radiograph revealed the presence of a left-sided soft tissue mass located at the level of the aortic arch . The lesThe surgical procedure was conducted in two stages. Posterior then anterior approaches were planned. For the first stage, the patient was placed prone and an incision was made in the midline extending from C5 to T7. The spinous process and bilateral inferior facets of T3 were removed. The posterior bony elements of T4 and T5 were removed. Instrumentation was placed from C5 to C7 and T3, T6, T7 and fixed with pedicle screws. A combination of Vertex and CD Horizon system (Metronic) of posterolateral fixation was used. Fusion was then accomplished by decorticating the transverse processes and an autograft with bone chips was applied after the exposed dura had been protected . The second stage of the procedure was performed 3 months after the first stage. The patient was placed in a lateral position with his right side down; surgical excision of the 5th allowed a large thoracotomy. The tumour had 3 lobes and was treated with liquid nitrogen (−17°C for 15 min/lobe). The equipment used was Candela CS5  with up to 5 probes, which can be sterilized and reused. We used 3.5 and 10 mm rod-shaped and a plate probe  with liquid N2 (55 psi) which can reach –196°C , shell of healthy tissue [Chondrosarcomas of the spine are rare. Some large series of this particular localisation have been published; however, no specific information on optimal surgical method has been clearly described . Spine cy tissue , 16–18. Since the role of the extent of surgical resection appears to have such an impact on recurrence rate and time of tumor recurrence, the best surgical technique should be used, to offer optimal results . Our resAlthough cryosurgery is found to be effective for use against localized disease and solid tumors larger than 1 cm, little is mentioned in the literature concerning the use of liquid nitrogen in the treatment of spine chondrosarcoma –14. It i


Desmoplastic ameloblastoma is a rare variant of ameloblastoma. Up until now, less than 150 patients have been reported in the literature. We report a case of desmoplastic ameloblastoma in a 45-year-old female with a painless swelling in the left anterior maxillary region. Fine needle aspiration yielded no fluid. Periapical and panoramic radiographs as well as computer tomography scan showed a mixed lesion with multilocular appearance. The present case deserves special importance be-cause of its unfamiliar appearance, potentially aggressive nature and high chances of misdiagnosis. Moreover, the radio-graphic features of this lesion rarely point towards ameloblastoma. A partial maxillectomy for tumor resection was per-formed and the involved teeth were removed. This report is an attempt to help the dental community in developing familiarity with the clinical presentation and at the same time advocating to develop a high index of suspicion in recognizing such cases. The first detailed report on the desmoplastic variant of ameloblastoma in the English literature was given in 1984 by Eversole,3 Desmoplastic ameloblastoma warrants a separate clinicopathologic entity as it differs strikingly from the other forms of ameloblastoma in its anatomical location, morphology, and radiographic appearance. However, age and sex distribution do not differ from the other types of ameloblastoma. Clinically, this tumor has a predilection for occurrence in the anterior or premolar region of the maxilla or the mandible. It presents radiologically as unilocular or multilocular radiolucencies. In most of the cases, their radiographic appearances are often more typical of the fibro-osseous lesions with well-defined or poorly defined borders. Around the globe, surgeons as well as the radiologists are well versed with the clinicoradiographic features of the common ameloblastomas, however, may ignore this variation.8Ameloblastoma occurs in all areas of the jaws, but the mandible is the most commonly affected area. Within the mandible, the molar-angle-ramus area is involved three times more commonly than are the premolar and anterior regions combined. Radiographically, the intraosseous ameloblastoma is classically described in dental periapical and panoramic films as a multilocular or 'soap bubble' radiolucency.9 The purpose of this article is to report a case of ameloblastoma with the characteristic clinical, radiographic and histological features associated with desmoplastic ameloblastoma and to assist the dental community in better understanding of this variation in odontogenic tumor.Histologically, this unusual variant is characterized by extensive stromal collagenization or desmoplasia with small nests and strands of odontogenic epithelium.A 45-year-old female presented to the Department of Oral Medicine and Radiology, M.M. College of Dental Sciences and Research, Mullana, Haryana, India with an asymptomatic swelling of spontaneous origin in her left maxilla that had started one year previously. Since then, there had been gradual increase in the size of swelling to its present size. She denied experiencing any bleeding, pain or sensory changes. There was no history of trauma and the past dental/medical history was unremarkable.The physical examination revealed facial asymmetry due to swelling on the left side of face which was oval in shape and had a smooth surface. The skin over the swelling appeared normal. It was not tender on palpation.The intraoral examination disclosed a large mass, approximately 4.5 × 3 cm in size, extending from the left central incisor to the left first premolar buccally as well as palatally . BuccoliOn palpation, the swelling was non-tender and the inspectory findings were confirmed. The swelling was bony hard in consistency, nonfluctuant, nonreducible, noncompressible and nonpulsatile. The teeth in the affected area were not sensitive to percussion and no mobility could be demonstrated. Electric pulp vitality testing revealed that all the teeth in the vicinity were vital except the left upper lateral incisor. No lymphadenopathies or fistulae were present.Radiographic examination of the maxilla revealed a diffuse ill-defined mixed radiolucent / radiopaque lesion extending from the distal surface of the left central incisor to the mesial surface of the left second premolar, with an approximate size of 2.5 × 3 cm. Small flecks of radiopacities were seen within the lesion. There was loss of lamina dura around the involved teeth. The lesion caused divergence of the root of the lateral incisor, without any signs of root resorption . The floBased on the clinical and radiographic appearance, a provisional diagnosis of odontogenic myxoma was made. Points in favor of myxoma included its female preponderance, potential to attain considerable size without noticeable signs and symptoms, association with missing tooth (canine in this case), its predilection for premolar–first molar region in the maxilla and diffuse ill -defined borders on radiographs. Aspiration of the lesion was non-productive and a complete hemogram showed values within the normal range. An incisional biopsy was performed under local anesthesia to establish a definitive diagnosis. Histologically, the features were consistent with those of desmoplastic ameloblastoma .A partial maxillectomy for tumor resection was performed under general anesthesia and a transitional maxillofacial prosthesis was placed. The surgical specimen consisted of a portion of the maxilla and the teeth involved in the tumor. The postoperative course was uneventful and examination of the patient after 1 year showed no evidence of recurrence of the lesion.In view of the paucity of desmoplastic ameloblastoma case series, it continues to remain one of nature’s secrets. Up until now, less than 150 patients have been reported in the literature. A painless swelling or bony expansion is the most conspicuous clinical manifestation in most of the cases.8Desmoplastic ameloblastoma constitutes 0.9% to 12.1% of all ameloblastomas.The mean age at initial presentation is 42.3 years (range 17-70 years) with age and gender predilection similar to that of other ameloblastomas. Data from different geographical regions seem to suggest a biogeographical pattern in that the relative frequency of desmoplastic ameloblastoma is slightly higher in Asian population. However, more systematic studies on desmoplastic ameloblastoma are needed to prove such suggestions.10Regarding its origin, it is suggested that desmoplastic ameloblastoma develops from the periodontal membrane of the related tooth. Moreover, some suggest that desmoplastic ameloblastoma might arise from epithelial rests of Malassez in the periodontal membrane. In this case, disappearance of the lamina dura and the periodontal ligament space of the adjacent lateral incisor root was clearly identified.11Approximately half of the desmoplastic lesions are located in the maxilla, and the vast majority of them occur in the anterior or premolar portion of the jaws. This is in contrast to the location of the unicystic or classic types of ameloblastoma, which usually are found in the posterior area of the mandible. Maxillary lesions are more insidious than mandibular tumors owing to the proximity of vital structures and the maxillary sinus. Also, the very thin cortical bone of the maxilla forms a weak barrier for the spread of tumors. Consequently, maxillary ameloblastomas may be able to spread earlier and more quickly than do mandibular neoplasms.11 Tooth displacement is a common feature in desmoplastic ameloblastoma in almost 92% of the cases and root resorption is seen in just 33% of the cases.2The patient described in this report presented no root resorption, but displacement of the adjacent teeth and also the tumor was associated with a missing tooth.Desmoplastic ameloblastoma exhibits a more aggressive behavior than other types of ameloblastoma. This aggressiveness may be due to 1) potential to grow to a large size; 2) the common location in the maxilla leading to an early invasion of adjacent structures; 3) the diffuse radiographic appearance, and 4) histologic finding of bone invasion.12 Most of the cases present with poorly demarcated borders. According to Philipsen et al,13 radiographically ill-defined borders suggest an infiltrative process with propensity to recur. The panoramic radiograph, conventional computed tomography and magnetic resonance images of desmoplastic ameloblastoma are not specific; they are compatible with those of fibro-osseous lesions and can mimic the radiographic appearance of fibrous dysplasia, chronic sclerosing osteomyelitis, and if well-circumscribed, an ossifying fibroma. Therefore, it is necessary to include these in the differential diagnosis. High resolution bone algorhithm computed tomography images, however, reflect the invasion of tumor elements between peripherally situated bone trabeculae where resorption due to tumor expansion and the deposition of new bone around these resorbed trabeculae has occurred.14Radiographically, desmoplastic ameloblastoma usually appears as a mixed radiolucent and radiopaque lesion sometimes mimicking a benign fibro-osseous lesion. The mixed radiographic appearance is due to osseous metaplasia within the dense fibrous septa that characterizes the lesion, and it is not because of the production of a mineralized product by the tumor.In all the reported cases of desmoplastic ameloblastomas, typical radiographic features of ameloblastoma were not observed. The present case, too, lacked typical findings of ameloblastoma and was difficult to diagnose correctly. We suspected fibro-osseous lesion or odontogenic myxoma based on the radiographic findings.2A confirmatory diagnosis of desmoplastic ameloblastoma is made by histopathological evaluation of biopsy specimens. The following features are usually observed during microscopic examination: 1) stromal desmoplasia, in the form of moderately cellular, fibrous connective tissue with abundant collagen, which is the most consistent and distinguishing feature; 2) Islands of different shapes in the epithelial component; 3) peripheral layer of cuboidal cells; and 4) hypercellular central area composed of spindle-shaped or polygonal epithelial cells. Our case was consistent with these common features reported in the literature. Occasionally, desmoplastic ameloblastomas may exhibit interspersed zones of classic follicular or plexiform ameloblastoma; these have been designated as 'hybrid lesions.'15Histologically, the tumor might be misdiagnosed as another odontogenic tumor, as the characteristic palisading layer of ameloblastoma may not be present in all the epithelial clusters, particularly if the biopsy specimen is small. Areas with only narrow strands of epithelial cells within desmoplastic stroma may simulate odontogenic fibroma. The importance lies in the differences in the clinical behavior and management of these two tumors. Ameloblastoma is a potentially aggressive tumor that requires en bloc resection. Odontogenic fibroma is much less aggressive and enucleation is probably curative.15Another differential diagnosis is squamous odontogenic tumor. As this may have a fibrotic stroma, the squamous metaplasia observed in some areas of the desmoplastic variant of ameloblastoma may mimic it if the palisading layer of the tall columnar cells is not identified. Although some cases of squamous odontogenic tumor may have an aggressive clinical course, the currently preferred treatment is curettage, which is followed by few recurrences. Other differential diagnosis includes squamous cell carcinoma and ameloblastic fibroma. The former has prominent cytological atypia while the latter has a cellular stroma. Accurate diagnosis of the desmoplastic variant of ameloblastoma depends on the identification of the typical ameloblastic areas, and this may require examination of more tissue or a repeated biopsy.16 using various immunohistochemical techniques demonstrated that the tumor cells of desmoplastic ameloblastoma showed variable expression of S-100 proten and desmin. Similarly, it has been reported that connective tissue stroma in desmoplastic ameloblastoma exhibited a strong positive reaction for collagen type VI.17 This was interpreted as an active de novo synthesis of extracellular matrix protein, hence ruling out scar tissue.Even after two decades of the first report of desmoplastic ameloblastoma, the cause for this peculiar histologic appearance is still unclear. Ng & Siar18reported a higher recurrence rate (21.4%) for desmoplastic variant than the other types (10.1%) of ameloblastoma.Whether the recurrence is due to the nature of the tumor or due to the incomplete surgery remains speculative. Therefore, block excision is the most widely used treatment to avoid recurrence.With limited understanding of its biologic behavior and prognosis, the proper treatment strategies for desmoplastic ameloblastoma are not entirely defined so far. Resection is the most preferred treatment option for desmoplastic ameloblastoma although some cases were treated by enucleation and/or curettage. However curettage leaves islands of tumor within bone, which later manifest as recurrences. Keszler et alThe desmoplastic ameloblastoma is characterized by specific clinical, imaging, and histological features. For proper understanding of such cases, more in depth analysis and long term follow up is required. The clinician has to be alert regarding the unusual presentation of this neoplasm and should include desmoplastic ameloblastoma as differential diagnosis in any lesion ranging from simple abscess to any fibro-osseous lesions/neoplastic growth presenting in anterior maxilla/mandible. The definite diagnosis requires histopathological examination. Also with the potential for recurrence, such cases should always be treated by complete resection.


Histological and radiological criteria are used to distinguish this benign condition from other malignant diseases but have their limitations, and biopsy of mediastinal masses can be risky. We report for the first time the diagnostic value of fluorodeoxyglucose 18 F avidity within the mass similar to that of normal thymus. The diagnosis of benign hyperplastic thymus was made.A 2 year old girl presented with new onset of emesis and constipation leading to the incidental discovery of an anterior mediastinal mass on radiograph. Chest computed tomography revealed cystic components within the mass concerning for a malignancy. Biopsy of the lesion and bone marrow aspiration and biopsy were negative but there was concern that the mediastinal biopsy may have missed the malignant component of the lesion. Hence, a positron emission tomography scan was obtained that showed mild homogeneous fluorodeoxyglucose 18 F positron emission tomography can help guide a clinician’s decision for further interventions and treatment.The differential diagnosis of an incidentally found anterior mediastinal mass includes malignancy, but benign lesions such as benign hyperplastic thymus must also be considered, particularly when the complete blood count and biochemical profile are normal. Fluorodeoxyglucose  Potential etiologies of anterior mediastinal masses in children include benign and malignant tumors whose incidences vary by patient age and symptoms at presentation. Benign hyperplastic thymus (BHT) is a rare condition that occurs mainly in infants and usually resolves spontaneously by 3 years of age, while it seldom occurs in older children and never in adults -3. BHT iDiagnoses such as germ cell tumor, thyroid cancer, and lymphomas (Hodgkin and non-Hodgkin) must be ruled out during diagnostic workup. BHT spontaneously resolves over time, without specific treatment or surveillance; thus, prognosis and management differ from that of malignant etiologies ,8. Fine-18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) scan. PET can help differentiate BHT from other conditions associated with an anterior mediastinal mass.Here we describe a child whose diagnosis of BHT was based on CT-guided core needle biopsy and supported by conventional imaging and A 2-year-old girl with no significant past medical history was in her usual state of health when she developed non-bloody and non-bilious emesis associated with constipation. A review of other systems was negative. She did not take any medications and family history was non-contributory. In the emergency room, she appeared well and in no acute distress, with a normal physical examination. Weight, height, and vital signs were normal for age and gender. A chest/abdominal radiograph to evaluate her constipation showed no intra-abdominal pathology but demonstrated mediastinal widening Figure A. A CT sThe patient underwent CT-guided core needle biopsy of the lesion, bilateral bone marrow aspirates and biopsies to rule out a malignancy. Hematoxylin and eosin staining and immunohistochemical studies of the mediastinal mass biopsy showed preservation of thymic architecture, with sheets of variably mature small- to intermediate-sized lymphoid cells and few Hassall's corpuscles, consistent with benign thymic tissue. Flow cytometry revealed T-lymphocytes with a full spectrum of orderly thymic maturation patterns, and bone marrow aspirates and biopsies showed normocellular marrow (80%) with trilineage hematopoiesis without evidence of leukemia or lymphoma.imaging is not a typical finding of BHT and raised concern for a malignancy such as teratoma or lymphoma, which may present with such features  that require immediate medical attention. Workup includes patient’s history, physical examination, routine laboratory tests, and anatomic imaging Figure . After ePhysiologic imaging, most often PET-CT, is recommended in the diagnostic process when uncertainty about the malignant versus benign nature of the mass persists Figure  35]..35].The pattern and intensity of uptake within such lesions on PET and morphologic appearance on CT can help differentiate benign from malignant etiologies  also se. To accu18 F-FDG PET are more meaningful than the SUV. There is an overlap in SUV range between normal thymus and other malignant anterior mediastinal tumor entities [Malignant conditions show intense FDG avidity that is usually heterogeneous in distribution . The appentities . Althougentities ,43.In conclusion, for incidentally found anterior mediastinal masses in otherwise healthy children, we recommend that clinicians expeditiously rule out oncologic emergencies then perform a diagnostic workup. PET scans can help differentiate BHT from other more serious conditions and may spare patients invasive diagnostic procedures.18 F-fluorodeoxyglucose positron emission tomography in patients with suspected benign hyperplastic thymus.Further studies including large pediatric series are needed to evaluate the importance of Written informed consent was obtained from the patient’s mother for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.18 F- fluorodeoxyglucose; PET: Positron emission tomography; SUV: Standardized uptake value.BHT: Benign hyperplastic thymus; BMA: Bone marrow aspiration; CT: Computed tomography; FDG: The authors declare that they have no competing interests.All authors have participated in study design, interpretation, and writing of the report. RN did the collection of the data, review of literature, and drafted the first version of the manuscript. JLC provided the figures and reviewed the manuscript. SCH reviewed the manuscript. MLM primarily participated in study design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/13/103/prepub


Lipomas are benign tumors which may appear in almost any human organ. Their diagnosis rate in the hand region is not known.We present the case of a 63-year-old Greek Caucasian woman with a giant lipoma of the hand and palm which was not initially diagnosed. After repeated surgical decompression of the carpal tunnel the patient was referred with persisting symptoms of median and ulnar nerve compression and a prominent mass of her left palm and thenar eminence. Clinical examination, magnetic resonance imaging, nerve conduction study and biopsy, revealed a giant lipoma in the deep palmar space (8.0 × 4.0 × 3.75 cm), which was also infiltrating the carpal tunnel. She had already undergone two operations for carpal tunnel syndrome with no relief of her symptoms and she also ended up with a severed flexor pollicis longus tendon. Definitive treatment was performed by marginal resection of the lipoma and restoration of the flexor pollicis longus with an intercalated graft harvested from the palmaris longus. Thirty months after surgery the patient had a fully functional hand without any neurological deficit.Not all lipomas of the wrist and hand are diagnosed. Our report tries to emphasize the hidden danger of lipomas in cases with carpal tunnel symptoms. The need for a high index of suspicion in conjunction with good clinical evaluation and the use of appropriate investigative studies is mandatory in order to avoid unnecessary operations and complications. Marginal excision of these tumors is restorative. Lipomas are the single most common soft tissue tumor . HoweverA 63-year-old Greek Caucasian woman was referred with symptoms of median and ulnar nerve compression and a prominent mass on her left palm and thenar eminence to the outpatient clinic of our upper limb and hand surgery unit. She already had had two operations for carpal tunnel release within the last three years in another hospital and despite that, her situation had worsened.Physical examination revealed a soft tissue mass which was slightly tender on palpation, with a diminished range of motion of the wrist . She was also unable to flex the distal phalanx of the thumb. The mass was apparent on plain X-rays and an MRI examination showed a well-orientated tumor, measuring 8.0 × 4.0 × 3.75 cm. It extended from the deep palmar space, between the tendons and the metacarpals leaving the periosteum intact Figures  and 2. TSurgery was performed under axillary block and by tourniquet controlled hemostasis. A palmar zig-zag incision was made. The tumor was identified and found to compress all the surrounding tissues. Intra-operatively, the median and ulnar nerves were carefully identified and protected. Marginal excision of the tumor was performed which decreased inter-compartmental pressure which had previously affected all relevant anatomic structures Figures  and 4. IAt the final follow-up thirty months postoperatively, the hand was pain-free without neurological deficit and exhibiting full range of motion. Thumb motion exhibited no deficits.Lipomas consist of mature fat cells, which may occur in subcutaneous, inter-muscular or intra-muscular locations. They generally progress slowly and painfully which explains their often large size at diagnosis, particularly if located deeply. The differential diagnosis between lipomas and well-differentiated liposarcomas is extremely important and allows appropriate monitoring and treatment planning. Recently, many cytogenetic studies have dealt with tumors originating from adipocytes, including benign lipomas, as well as malignant well-differentiated liposarcomas (WDLPS). The majority of these tumors present genetic mutations in regions 12q13-15 and 6p13q . Any sofAdipose tissue is so widely distributed throughout the human body that one would expect these tumors to be one of the most frequently encountered benign neoplasms. The small numbers of cases being treated for this kind of tumor might be an indication of a high rate of misdiagnosis. Apart from cosmetic reasons the only indication for surgical treatment is the existence of complications arising from compression of local neurovascular structures which is a high occurrence in the narrow spaces within the fascial sheaths demarcating the regions of the hand.Soft tissue lipomas are categorized by anatomic location as either superficial (subcutaneous) or deep and their contour is determined by the confines of the space the tumor occupies. When there is no limiting fascia, muscle, bone or organ, the lipoma is usually round. Fat tumors however have the ability to insinuate themselves into small recesses and thus produce tumors of any size or shape, infiltrating spaces not tightly guarded by protecting sheaths as fascia. This is especially true with tumors of the hand where lipomas occur in various anatomic locations within it. Superficial lipomas arise in the subcutaneous tissues while deep lipomas arise in the Guyon's canal, in the carpal tunnel, and the deep palmar space. Brand and Gelberman  advocateIt must be realized that not all lipomas of the wrist and hand are diagnosed. Our report emphasizes the hidden dangers of misdiagnosing or altogether missing lipomas in cases with carpal tunnel symptoms.The need for a thorough clinical examination and a sound intra-operative investigation of all anatomical structures involved in the carpal tunnel release operation is of paramount importance. Although treating physicians may consider this type of operation a simple one, a high suspicion index concerning tissue involvement in conjunction with good clinical evaluation and the use of appropriate investigative studies are mandatory in order to avoid unnecessary re-operations and complications. This case presentation shows not only that a sound original diagnosis might have prevented three further re-operations and a complication including the flexor pollicis longus tendon, but also points out the necessity of a structured and complete clinical examination.Our report stresses the fact that marginal excision of lipomas is restorative.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.TP assisted in the surgery, analyzed and interpreted the patient data and was a major contributor in writing the manuscript. PG performed the surgery and was a major contributor in writing the manuscript. AC was a major contributor in writing the manuscript and performed the histologic analysis of the excised tumor. All authors read and approved the final manuscript.


Seminomas have been rarely associated with malignant hypercalcemia. The responsible mechanism of hypercalcemia in this setting has been described to be secondary to 1,25-dihydroxyvitamin D secretion. The relationship with PTHrP has not been determined or studied.The aim of this study is to describe and discuss the case and the pathophysiological mechanisms involved in a malignant hypercalcemia mediated by 1,25-dihydroxyvitamin D and PTHrP cosecretion in a patient with seminoma.A 35-year-old man was consulted for assessment and management of severe hypercalcemia related to an abdominal mass. Nausea, polyuria, polydipsia, lethargy and confusion led him to the emergency department. An abdominal and pelvic enhanced CT confirmed a calcified pelvic mass, along with multiple retroperitoneal lymphadenopathy. Chest x-ray revealed “cannon ball” pulmonary metastases. The histopathology result was consistent with a seminoma. Serum calcium was 14.7 mg/dl, PTH was undetectable, 25-dihydroxyvitamin D was within normal values and PTHrP and 1,25-dihydroxyvitamin were elevated . After the first cycle of chemotherapy with bleomycin, etoposide and cisplatin, normocalcemia was restored. Both PTHrP and 1,25-dihydroxyvitamin D, dropped dramatically to 9.0 pg/ml and 8.0 pg/ml, respectively.The association of seminoma and malignant hypercalcemia is extremely rare. We describe a case of a patient with a seminoma and malignant hypercalcemia related to paraneoplastic cosecretion of 1,25-dihydroxyvitamin D and PTHrP. After successful chemotherapy, calcium, PTHrP and 1,25-Dihydroxyvitamin D returned to normal values. It usuane (PTH) ,5.2D3 secretion. The underlying mechanism in the rest of the cases was not established. In the previous reports PTHrP was not measured nor related to hypercalcemia. In all of the previous documented cases, independent of the hypercalcemia related mechanism, normocalcemia was achieved soon after definitive treatment was initiated [Seminomas have seldomly been related with hypercalcemia. Less than ten cases have documented this exceptional association. Besides two cases related to bone metastasis, the mechanism of hypercalcemia in seminomas has been discussed in two previous reported cases to be secondary to 1,25(OH)nitiated -9.2D3 and PTHrP paraneoplastic cosecretion.Herein, we present a case of a patient with a seminoma and malignant hypercalcemia mediated by 1,25(OH)A 35-year-old man was referred to the Endocrinology Division for assessment and management of severe hypercalcemia related to an abdominal mass. Otherwise, he had an unremarkable medical history. One year before admission he noticed a painless hypogastric abdominal mass. The tumor progressively grew and two months before admission he developed asthenia, anorexia, general weakness, constipation, and an unintentional 10 kg weight loss. A month before admission the appearance of a left inguinal mass finally made him seek medical attention.An abdominal ultrasound revealed a 10 × 9 × 7 cm heterogeneous (hypoechogenic with hyperechogenic areas) retroperitoneal mass along with bilateral severe hydronephrosis. Serum creatinine was 2.8 mg/dl with a modification of diet in renal disease (MDRD) calculated glomerular filtration rate (GFR) of 35 ml/min. This consequently led to an uncomplicated unilateral left nephrostomy. An abdominal and pelvic enhanced computed tomography (CT) confirmed a calcified pelvic mass, along with multiple retroperitoneal lymphadenopathy (>5 cm each) . Phosphorous, magnesium, potassium, sodium, chlorine, and glucose were normal. PTH was undetectable, 25-dihydroxyvitamin D was normal, PTHrP was 35.0 pg/ml, and calcitriol 212 pg/ml  (Table 2D3. Pure seminomas (no non-seminomatous elements present) account for up to 60% of all testicular germ cell tumors. They usually affect males between the second and fourth decade of life, representing 1% of all cancers in men. Undescended testes (cryptorchidism), as in our patient, is a risk factor and they are usually associated with a good prognosis with an overall five-year survival of 95% and even 80% in intermediate and high-risk patients [2D3 secretion that accounts for less than 1% of cases [This case illustrates, to our knowledge, the first reported case of malignant hypercalcemia in a patient with seminoma related to paraneoplastic cosecretion of PTHrP and 1,25(OH)patients ,11. Hyerof cases ,5,12,13.2D3 was measured only in one case with an initial concentration of 79 pg/mL that decreased to 5 pg/mL after two cycles of chemotherapy and was associated afterwards with normocalcemia [Usually associated to granulomatous diseases such as sarcoidosis, tuberculosis, and systemic fungal infections, among others, calcitriol mediated hypercalcemia has been infrequently related to neoplasms . Lymphom2D3 levels were made [On the other hand, PTHrP has never been described in a patient with a pure seminoma. There are two reported cases of hypercalcemia associated with non-seminomatous germ cell tumors (NSGCTs). MacDiarmid et al. in 1995 reported a malignant hypercalcemia associated to an extragonadal NSGCT that was suspected to be probably due to a humoral hypercalcemia after bone scans were negative. PTHrP and vitamin D metabolites were not measured . Later oere made . In our 2D3, which is essential for bone and calcium metabolism/homeostasis. Nevertheless, 1α-hydroxylase has also been described in normal human tissues such as placenta, skin, gastrointestinal tract, testes, central nervous system and osteoblasts [The exact mechanism by which calcitriol is produced in malignancy remains open to debate. Normally, in the kidney, 1α-hydroxylase converts 25-hydroxyvitamin D into the biologically active 1,25(OH)eoblasts . More theoblasts . In lympeoblasts . Nonetheeoblasts . The exaeoblasts . This laeoblasts -9,23. Aseoblasts . Intereseoblasts . In this2D3 and PTHrP. After successful treatment, calcium, PTHrP and 1,25(OH)2D3 returned to normal values. The exact mechanism by which seminomas secrete 1,25(OH)2D3 still remains to be elucidated.Seminomas have seldom been associated with hypercalcemia. We have described the case of a patient with a seminoma and malignant hypercalcemia related to cosecretion of 1,25(OH)Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available upon request for review by the Journal Editor.PTHrP: Parathyroid hormone related-protein; 1,25(OH)2D3: 1,25-dihydroxyvitamin D, calcitriol; PTH: Parathyroid hormone; MDRD GFR: Modification of diet in renal disease calculated glomerular filtration rate; LDH: Lactate dehydrogenase; IL: Interleukin; CT: Computed tomography; NSGCTs: Non-seminomatous germ cell tumors.The authors declare that they have no competing interests.RRG and JGGG served as the principal investigators and led the conception, design, acquisition of data, review of literature, and drafted the manuscript. MAZR, DLQF, CRCL contributed in the acquisition of data, review of literature and reviewed the manuscript. FJLG and JZVP contributed the concept of research paper and critically reviewed the manuscript. All authors read and approved the manuscript.RRG and MAZR are endocrinology fellows at the Endocrinology Division. DLQF and CRCL are internal medicine residents. FJLG is professor and chief of the diabetes clinic of the Endocrinology Division. JGGG is the vice-dean of research of the Medical School of the Universidad Autonoma de Nuevo Leon and professor of the Endocrinology Division. JZVP is the chief and director of the Endocrinology Division.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6823/14/32/prepub


We report a case of a cervical rheumatoid nodule in close relation to the hyoid bone mimicking a metastatic carcinoma. A 74-year-old female with a 15-year history of rheumatoid arthritis (RA) on treatment with methotrexate presented with tenderness of the right base of tongue. Imaging demonstrated a 1.4 cm cystic lesion at the hyoid bone. Biopsies were unsuccessful and the patient required surgical resection of the mass. A trans-cervical approach was used. Pathology revealed a necrotizing granuloma compatible with rheumatoid etiology. The clinician should be aware that, in a patient with a neck mass, in the presence of active RA, rheumatoid nodules should be part of the differential diagnosis. Rheumatoid nodules have been reported at unusual sites, including the eyelid, soles, the spine and various viscera. RA also can affect the larynx, ear and nose with various otorhinolaryngological manifestations.The incidence of laryngeal involvement in RA ranges from 13 to 75%. Under aA 74-year-old female initially noted tenderness of the right base of tongue and right aural fullness one year prior to presentation. No additional head and neck symptoms were elicited. The patient had rheumatoid arthritis, for which she was currently being treated with methotrexate. An MRI of the neck was performed, which demonstrated a 1.4 cm cystic lesion at the hyoid bone protruding into the pre-epiglottic fat. This led to a provisional diagnosis of malignancy to the hyoid bone. On evaluation at our institution, the patient was noted to have tenderness to palpation at the right base of tongue, but no palpable masses. On flexible fiberoptic laryngoscopy, no lesions were noted. Normal vocal cord motion was seen bilaterally, and there was no palpable adenopathy in the neck.A CT scan revealed a small, well-circumscribed, ovoid mass with central necrosis in the left pre-epiglottic space, abutting the posterior surface of the hyoid bone Figure . NeitherIt was decided to proceed with surgical resection of the mass. Intraoperative laryngoscopy revealed negative findings in the vallecula and in the larynx. A trans-cervical approach to the left neck was undertaken. After division of the infrahyoid musculature, the lesion was identified and circumferentially resected with the lateral cornu of the hyoid bone. Frozen section analysis was consistent with a rheumatoid nodule. The laryngeal framework was then reconstructed by reapproximating the strap muscles and hyoglossus muscle to the hyoid to ensure the stability of the larynx.Final pathology evaluation revealed a necrotizing granuloma, compatible with rheumatoid etiology Figure . Stains We present the case of a patient with a tender mass in the pre-epiglottic region at the level of the hyoid bone, which final pathology demonstrated to be a rheumatoid nodule. Although rheumatoid nodules in the larynx have been described, focal involvement of the hyoid bone to our knowledge has not been reported in the literature.Laryngeal abnormalities in RA can be seen on CT imaging in 50-75% of patients,4. The p2 should also be considered.In a patient presenting with a laryngeal mass, the otolaryngologist should be cognizant that the differential diagnosis should include a malignant process, more frequently squamous cell carcinoma. Other malignant considerations include adenoid cystic carcinoma, lymphoma and sarcomas. Among the benign lesions, laryngocele, amyloidosis, papilloma and inflammatory processes such as tuberculosis and rheumatoid nodulesMost studies on laryngeal involvement of RA describe nodules at the CJ; however, nodules involving the hyoid, to our knowledge, have not been described. In our case, the nodule was traced to the posterior aspect of the hyoid bone. It is possible that the rheumatoid nodule had arisen from the union of the body to the lesser cornus of the hyoid, which is usually attached to the body of the bone by fibrous tissue, and occasionally to the greater cornua by distinct diarthrodial joints. This joint usually persists throughout life, but occasionally becomes ankylosed.The presence of a new neck mass should always involve a thorough clinical history, examination and further evaluation, including imaging and directed biopsies, to exclude the presence of a malignancy. However, in patients with known RA and active disease, the clinician should be aware that, although rare, rheumatoid nodules should be included as one of the primary differential diagnoses of neck masses.CJ: cricoarytenoid joint; RA: rheumatoid arthritisThe authors declare that they have no competing interests.FG participated in the collection of the data, review of the literature, drafting and edition of the manuscript. AE participated in the review and interpretation of the histopathology, NG participated in the interpretation of the imaging and edition of the manuscript, MK conceived the study, and participated in the writing and edition of the manuscript. All authors read and approved the final manuscript.Fernando Gomez-Rivera, M.D. (FG), Adel K. El-Naggar, M.D., Ph.D. (AE), Nandita Guha-Thakurta, M.D. (NG), Michael E. Kupferman, M.D. (MK)Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Squamous cell lesions of the urinary bladder are generally rare. Herein we describe a case of 74-year-old male patient with a benign squamous cell papilloma. Histologically, the tumor presented extensive keratinization at its surface and showed no nuclear atypia or stromal invasion. The tumor cells were negative for HPV DNA. These lesions are extremely rare, and even though they are considered benign and non-HPV related, they should be followed, since recurrence has been reported. Papillomatous lesions of the urinary bladder are quite common and include both benign and malignant tumors as well as nonneoplastic lesions . Tumors usually originate from the urothelium of the urinary bladder. When benign, they may be urothelial papillomas or inverted papillomas, and when malignant they may be urothelial carcinomas with or without invasion of the bladder wall. Malignant urothelial tumors outnumber by far their benign counterparts, with the latter being estimated in less than 1% of all bladder tumors . HoweverA 74-year-old male patient admitted to our urology clinic for recurrent episodes of macroscopic hematuria, severe frequency, urgency, and painful voiding during the last six months. His medical history was otherwise unremarkable, except for hypertension. Previous urological investigation had revealed moderate prostate enlargement with normal PSA levels and a digital rectal examination with no signs suggestive of malignancy. The patient was put under tamsulosin and finasteride treatment for benign prostate hyperplasia, and his symptoms were tolerable until the last six months. At this point his blood tests were within normal limits, while his urine samples demonstrated microscopic hematuria without increased inflammatory cells. However, an abdominal CT scan revealed some thickening of the bladder wall. All other organs were within normal limits.μm paraffin sections of the tumor, and molecular HPV detection followed using the linear array HPV genotyping test . This proved to be negative for HPV DNA. The postoperational course of the patient was uneventful, and the patient was released from the hospital the next day after the interventional procedure. On cystoscopy an extensive, whitish, slightly exophytic lesion was found at the mucosa of the floor and the posterior wall of the bladder, measuring 25 × 15 mm. Endoscopically, the lesion was appearing like necrotic tissue and clinically regarded as highly suspicious for an invasive urothelial tumor . A transOne month after the TUR procedure, the patient's irritative and painful symptoms had been resolved. Cystoscopy and kidney ultrasound scan at three- and six-month postoperative intervals were normal. Squamous cell lesions of the urinary bladder are generally rare, and they may be benign or malignant –7. Benig


Head and neck paragangliomas are tumors associated with the parasympathetic nerve system and typically show an indolent growth pattern. Therefore a conservative management strategy is considered in selected cases.succinate dehydrogenase -sub-unit-D (SDHD). She was operated on the right carotid body tumor and the tympanic tumor. Thereafter the follow-up was performed with MR examinations at 2-year intervals. After an initial stable phase, over the last 3 years a spontaneous near-total regression of the contralateral carotid body tumor was observed, with only subtle rest-abnormalities visible in 2011.We present a case of a female patient who presented in 2003 with bilateral carotid body tumors and a tympanic tumor, associated with a mutation in the The present case underlines the indolent growth pattern of head and neck paragangliomas and for the first time describes a rare manifestation of spontaneous regression of a carotid body tumor. The literature was reviewed to discuss this phenomenon. Common sites include the carotid body, the temporal bone, and the vagal body. The maj-D (SDHD). In the -D (SDHD). Diagnos-D (SDHD),7. Treat-D (SDHD).In 2003 a 32-year-old female patient was referred to our institution after magnetic resonance imaging (MRI) examination in a regional hospital had revealed bilateral carotid body tumors. She had discovered a swelling in the right neck since 6 months before, and only after questioning mentioned a long-existing pulsatile tinnitus on the right side. The family history for paragangliomas was positive: her father and uncle were affected.D (P-Asp92Tyr).Physical examination revealed a small reddish tumor in the right middle ear, with positive Brown’s sign and a tumor in the right neck at the level of the hyoid bone; on the left side there was no palpable lesion. The lower cranial nerves were intact and the hearing was normal. In general examination, the patient was all-over obese , blood pressure was 130/70 mm Hg, pulse rate 80/min. There were no other abnormalities. Urinary excretion (24-h sample) of catecholamines was repeatedly normal. Germline mutation analysis in DNA from a blood sample demonstrated a mutation in SDHMRI including a gadolinium-enhanced 3D time-of-flight MR angiography sequence was acquThe right carotid body tumor was surgically resected in 2004, followed by extirpation of the tympanic tumor in 2005. No radiotherapy was applied. There were no complications from the surgical procedures. In the postoperative phase a severe obstructive sleep apnea syndrome (OSAS) was diagnosed, for which she started continuous positive airway pressure (CPAP) therapy. In 2005 she started thyroid hormone suppletion for Hashimoto disease. Between 2005 and 2008, the patient intentionally lost weight from 136 kg to 93 kg, because of a pregnancy wish which was eventually unsuccessful. During follow-up the patient regained bodyweight in 2010 to 129 kg. In 2011 she suffered anamnestically from a transient ischemic attack , after which she started statin treatment and therapy with carbaselate calcium and dipyridamole.Follow-up MRI was performed with 2-year intervals , hormonal (such as contraceptive use), vascular , and operative mechanisms.SDHD mutation in the present case (p.Asp92Tyr), however, is relatively common - as it is found in almost 70% of 690 Dutch SDH-gene mutation carriers[Biological mechanisms related to tumor regression include genetic instability (telomerase inhibition) and programmed cell death, which has been described for neuroblastomas,17. The carriers and has Although changes in body weight may go with changes in catecholamine levels, we found no evidence for such changes in our patient, neither after the start of CPAP treatment for OSAS. Therefore a hormonal mechanism for tumor regression seems unlikely.Vascular mechanisms may be due to changes in tumor angiostructure, and have been reported in spontaneous regressed  neuroblastomas. Such vaOperative mechanisms include regression of a tumor after biopsy, which has been described in patients with Merkel cell tumors,20, and Although the present case describes spontaneous involution, surgical resection is the favored treatment in carotid body tumors. Nevertheless, resection is associated with complications such as cranial nerve impairment, stroke, or partial scarification of the carotid arteries.In conclusion, the present case underlines the indolent growth pattern of head and neck paragangliomas and for the first time describes a rare manifestation of involution of a carotid body 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.Succinate dehydrogenase-sub-unit-D.CPAP: Continuous positive airway pressure; HNPGL: Head and neck paragangliomas; MRI: Magnetic resonance imaging; OSAS: Obstructive sleep apnea syndrome; SDHD: All authors declare no competing interest.SH and MK carried out the MR examinations and drafted the manuscript. FH performed genetic analysis and drafted the manuscript. JJ and EC performed physical examinations and patient follow-up and drafted the manuscript. All authors read and approved the final manuscript.


Mucoepidermoid carcinoma (MEC), one of the most common salivary gland malignancies, is rare in children. MEC mainly occurs in the parotid gland, along with minor glands being the second common site, particularly in palate. Clinical, histological, and radiological findings of palatal MEC in a 12-year-old girl are presented with three-year follow-up. Pathologic lesions must be considered in differential diagnoses of intraoral asymptomatic lesions, and their detailed inspection should be taken into consideration. Mucoepidermoid carcinoma (MEC) is one of the most common salivary gland malignancies. As its name implies, MEC is composed of a mixture of cells, including mucus-producing, epidermoid or squamous, and intermediate types . When MEFew series or case reports describing salivary gland tumors in the pediatric population have been published –8. This A 12-year-old girl patient visited the Department of Pediatric Dentistry, Ataturk University, Erzurum, Turkey, complaining of the pain in maxillar left central incisor (no. 11) and mandibular left lateral incisor (no. 22). She had a history of untreated Ellis II trauma and grade two mobility in these teeth for two years. Also, clinical examination revealed enlargement of the soft tissue in left posterior hard palate, with 8 mm diameter . During Epithelial salivary gland neoplasms are rare both in adults and children, accounting for less than 3% of all head and neck tumors. 5% of these tumors occur in patients younger than 18 years old with girls mostly affected, while its occurrence in newborns is exceedingly rare , 9–11. MAs a typical intraoral presentation this malignancy has a painless and persistent enlargement, which lasts for about a year. Paresthesia, pain, and difficulty with swallowing are noted frequently when major salivary glands and tongue are involved. Intraoral lesions are observable as a localized fluctuant nodule with a bluish or reddish-purple, smooth, mucosal surface. Like in our study, mucus may be discharged from the tumor through a small sinus tract . High-grRadiographically low-grade MECs are similar to benign mixed tumors. They demonstrate smooth margins and are characterized by cystic components containing mucin. On the other hand high-grade MECs have poorly defined margins, local infiltrations, and solid appearances .The clinical and radiographic differential diagnosis of a palatal mass includes reactive and neoplastic lesions. In children, the most common of these entities is the palatal space abscess derived from pulpal necrosis. They are differentiated by tooth mobility, diffuse, erythematous swelling of sudden onset, suppuration, fluctuation in lesion size, and radiographic evidence of inflammatory pulpal disease. Mucocele is a frequently seen fluctuant reactive lesion of salivary glands, with transparent blue swelling including mucin. Deep mucoceles, often surrounded by a fibrous tissue wall, do not fluctuate, and if located at sites other than the lower lip cannot clinically and reliably be differentiated from salivary gland tumors. Our case was not similar to a mucocele because mucin discharged from a sinus opening. Palatal region vascular malformations like hemangiomas are differentiated by clinical examination and imaging. Neurofibroma and schwannoma are occasionally encountered as compressible or firm asymptomatic nodules and pink in color unless they are secondarily traumatized , 15. The tumor is dissected down to the periosteum to obtain adequate tumor-free margins . But, ifRadiation therapy should be used judiciously in pediatric patients with high-grade histology, positive margins, and lymph node involvement, due to its long-term consequences as facial deformity, trismus, xerostomia, osteoradionecrosis, and risk of secondary malignancy. Chemotherapy was not used as an adjuvant therapy in our patient and does not currently have a role in the standard treatment of MEC patients .There is a potential risk of the development of a mucoepidermoid carcinoma in parotid and minor salivary glands of children who have received chemotherapy and cranial irradiation , 20. MorIn this case it is noteworthy that swellings in the palatal area which could resemble a dental abscess can cause unnecessary treatments, waste of time, and delay in diagnosis. Although mucoepidermoid carcinoma and other tumors in this region are exceedingly rare, patients with these kinds of swellings must be considered cautiously, and multidisciplinary approach can lead to successful treatment.


Adult intussusception due to Meckel's diverticulum is an uncommon cause of intestinal obstruction. However, the surgeon should still be suspicious of this condition since the non specific symptoms and the rarity of it make a preoperative diagnosis uncertain. Considering the secondary nature of adult intussusception and the necessity of early surgical intervention to avoid morbidity and mortality, we report one case of intussusception due to Meckel's diverticulum in an adult. A 22-year-old patient was admitted to our hospital with vomiting and abdominal pain. The abdomen was hard with tenderness. We diagnosed an acute small bowel obstruction and performed emergency surgery. The intra operative findings were distention of the small bowel and intussusception of ileus due to an inverted Meckel's diverticulum located 70 cm from the ileocecal valve. 30 cm ischemic loop was identified. A segmental small bowel resection and hand-sewn anastomosis was performed. Histopathology distinguished Meckel's diverticulum measuring 5 cm x 3.5 cm x 1 cm and no signs of malignancy. Meckel's diverticulum is the most common congenital abnormality of the gastrointestinal tract, occurring in 1% to 2% of the population. It is usually asymptomatic and becomes evident when complicated. Intestinal obstruction due to Meckel′s diverticulum is the most common presentation in adult and is the second most common in children. Occasionally, inversion of Meckel's diverticulum into the lumen of the bowel can cause intussusception, ischemia and infarction. The incidence of intussesception attributed to an inversion of Meckel's diverticulum accounts for 4% of all cases presenting with intestinal obstruction due to intussusception.It occurs when the Meckel's diverticulum sags into the bowel lumen and then serves as a lead point to allow telescoping of the small intestine, first into the distal ileum and then in to the large intestine, causing ileo-ileal and ileocolic type of intussusceptions.A 22-year-old male presented to the emergency room complaining of abdominal pain and vomiting for one day. He had no significant past medical history or previous abdominal surgery. There was no family history of any hereditary illnesses. On admission he had normal vital signs and a temperature of 38°C. Physical examination showed a slightly distended abdomen with hyperactive bowel sounds. The patient had abdominal tenderness and muscle guarding. No masses or hernias were identified. Laboratory tests revealed increased white blood cells (WBC) of 13 200 µ/L. Plain abdominal X-ray demonstrated air fluid levels of the small bowel, there was no pneumoperitomeum. We diagnosed an acute small bowel obstruction. Because of severe abdominal pain, the patient underwent emergency surgery with a lower midline abdominal incision. The intra operative findings were distention of the small bowel and intussusception of ileus due to an inverted Meckel's diverticulum located 70 cm from the ileocecal valve. 30 cm ischemic loop was identified . A segmeHistopathology revealed Meckel's diverticulum measuring 5 cm x 3.5 cm x 1 cm without ectopic mucosa or malignancy. The postoperative period was uneventful and after 7 days the patient was discharged. At the 6 months follow-up, the patient had no evidence of complications.Intestinal obstruction due to Meckel′s diverticulum is the most common presentation in adult and is the second most common in children . There aIntra operatively, the intussuscepted bowel should be deployed and examined for ischemia. There is no doubt that in case of transmural ischemia, the bowel needs to be resected along with the diverticulum . IntussuAdult intussusception caused by inverted Meckel's diverticulum may be observed in any age. It is a rare but important clinical entity. The presenting symptoms of intussusception are nonspecific. Diverticulectomy or bowel resection is the standard treatment.


Renal primitive neuroectodermal tumor (rPNET) as a member of Ewing’s sarcoma family is extremely rare and usually occurs in children and young adults. Most literature about rPNET was isolated case reports.We reported a case of 45-year-old man with the complaint of right flank pain. Computerized tomography (CT) scan demonstrated a large substantive tumor involving the lower pole of the right kidney. Then the patient underwent radical nephrectomy. Pathologic characteristics and immunohistochemical analysis confirmed the diagnosis of rPNET. Additionally, the patient received three cycles of chemotherapy, and was still alive without metastasis at 15-months follow-up.rPNET is rare and presents aggressive clinical behavior and worse prognosis. We expect that further awareness and study of this rare tumor can be had by presenting our case. Primitive neuroectodermal tumor (PNET) composed of small uniform round cells, is characterized by a translocation resulting in a fusion transcript of the EWS gene and ETS-related family of oncogenes . PNET isA 45-year-old man with the complaint of right flank pain for 1 week was admitted in August 2010. Ultrasonography showed a very large tumor on the right kidney. Computerized tomography (CT) scan showed a 12×10×10 cm substantive tumor involving the lower pole of the right kidney, while in the enhanced phase the tumor presented inhomogenous contrast enhancement with focal cystic and necrotic areas Figure . Urine eThe preoperative diagnosis of the patient was right renal carcinoma and radical nephrectomy was immediately done. The whole procedure was successful and the right kidney with the tumor was integratedly resected Figure . After sThe microscopic examination revealed that the tumor was composed of monotonous round cells with hyperchromatic round nucleus. The interspersed small dark cells indicating pyknosis of the tumor cells could form rosette-like structures Figure . The cytThe patient then underwent three cycles of chemotherapy (one cycle every 3 weeks). The chemotherapeutic agents included ifosfamide  and epirubicin . After the 15-months follow-up, the patient was still alive without metastasis.et al. in 1975 is a very rare and aggressive malignant tumor [et al. in 2001 [rPNET first described by Mor nt tumor . rPNET unt tumor . The tum in 2001 . The age in 2001 .The presenting symptoms and images of rPNET are non-specific and similar to other renal tumors. Therefore it is often difficult to distinguish rPNET from renal cell carcinoma and Wilm’s tumor . HoweverPENT is characterized by a translocation resulting in a fusion transcript of the EWS gene and ETS-related family of oncogenes . CytogenrPNET appears to be an unique clinical entity that behaves more aggressively than PNET arising at other sites . ApproxiThis case presented with a relatively large localized tumor and had non-specific symptoms. Radical nephrectomy was immediately done based on the findings of CT of the right kidney. In this case, both the pathologic characteristic and the positive expression of CD99, S-100 and NSE in the tumor cells could support the diagnosis of rPNET. By reviewing the literature, the importance of combination therapy for rPENT was known. The patient underwent three cycles of chemotherapy including ifosfamide and epirubicin. After the 15-months follow-up, the patient was still alive without metastasis. Therefore, we suggest that postoperative adjuvant chemotherapy should be performed in cases of rPNET.rPNET is rare and presents with aggressive clinical behavior and worse prognosis. Immunohistochemical staining for CD99 and some neural biomarkers along with cytogenetic studies play a great role in the diagnosis of rPNET. Radical nephrectomy combined with chemotherapy and radiotherapy is the recommended treatment for rPENT. We expect that further awareness of this rare tumor can be had by presenting our case.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Series Editor of this journal.CT: Computerized tomography; NSE: Neuron specific enolase; PNET: Primitive neuroectodermal tumor; rPNET: Renal primitive neuroectodermal tumor.The authors declare that they have no competing interests.CS drafted the manuscript. SJ and JL did the operation for the patient. KX and WD collected the materials. ZD provided the pathological figures. All authors read and approved the final manuscript.


Desmoplastic fibroma is an extremely rare primary benign bone tumor. It occurs most often in the mandible, followed by the femur and pelvis. To the best of our knowledge, fewer than 200 cases have been described in the published literature. Furthermore, this case is the first report of desmoplastic fibroma with simultaneous presentation in two different locations.We present an unusual case of desmoplastic fibroma in a 56-year-old Caucasian man, who presented to our hospital with lumbar pain. Computed tomography and magnetic resonance imaging were performed, demonstrating two lytic expansile lesions affecting both his left iliac bone and his left sacral wing. Curettage and cortical-cancellous grafting was performed, followed by postoperative computed tomography and magnetic resonance imaging.Desmoplastic fibroma with unusual and simultaneous manifestations in two different locations has never been reported previously to the best of our knowledge. The purpose of this case report is to present the computed tomography and magnetic resonance imaging features of this rare tumor before and after the surgical treatment. Furthermore, the radiological findings with the description of the characteristics and the clinical presentation of this rare tumor, contribute to the wide spectrum of manifestations of this tumor, in order to recognize it and to have the appropriate management. Desmoplastic fibroma (DF) of bone is a rare, lytic, locally aggressive but non-metastatic tumor that was first described by Jaffe in 1958 . It occuA 56-year-old Caucasian man, with a history of lumbar pain, presented to our hospital for a CT lumbar spine scan. He had no history of trauma. CT scan demonstrated an osteolytic lesion of the left iliac bone with cortical breakthrough, marginal sclerosis, slight pseudotrabeculation and bone expansion Figures , 2. A smThe surgical treatment included curettage and intralesional extension with replacement by cortical-cancellous allograft. The pathological specimen was reported and the histological findings concluded the diagnosis of DF. The surgical management was followed by postoperative CT and MRI, which showed a reduction in size of the lesion Figures , 7. The DF is a rare benign primary bone tumor, histologically similar to the soft tissue desmoid tumor. Microscopic infiltrations of tumor are present beyond the perceived macroscopic margin. In our case, we described a rare bone tumor affecting simultaneously both the left iliac bone and the left sacral wing with interposition of macroscopically normal tissue.DF is considered an aggressive tumor, with a high incidence of local recurrence after surgical resection 42 to 67%) 2 to 67% -7.Almost any bone can be affected, but DF most often involves the mandible 22%), femur (15%), pelvic bones (13%), radius (12%), and tibia (9%) [2%, femurDF is a locally aggressive tumor with high recurrence rate and it is important to differentiate it from other bone lytic lesions as there are significant implications regarding surgical treatment. The radiographic appearance of DF is similar to other lytic lesions. Differential diagnosis includes giant cell tumor, aneurysmal and solitary bone cyst, hemangioma, fibrous dysplasia, nonossifying fibroma, and chondromyxoid fibroma.While CT best illustrates the extent of bone destruction, MRI better visualizes the medullary as well as the soft tissue extension of the tumor. Therefore, CT and MRI are complementary imaging techniques in suspected DF.DF of the bone is a rare benign tumor with clinical symptoms that are usually non-specific. It is a benign tumor that should be included in the differential diagnosis list of any lytic bone lesion, especially in young patients. However, it differs from other benign fibrous lesions in that the lesion is very destructive locally and often recurs after incomplete excision. Therefore, despite its rarity, knowledge of the radiological features in correlation with the pathological findings is crucial in the diagnosis and the treatment of this rare tumor.DF: desmoplastic fibroma; CT: computed tomography; MRI: magnetic resonance imagingWritten informed consent was obtained from the patient for publication of thiscase report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KS prepared the case report and reviewed the literature. SB, ET and DC participated in the conception, design, and data collection and interpretation, analyzed the article and made necessary corrections. All authors read and approved the final manuscript.