We present the uncommon case of a subcutaneous fascia-based extramedullary plasmacytoma in the leg, which was confirmed by the pathology report and followed up until its remission. We report the differential diagnosis with other more common soft tissue masses. Imaging findings are nonspecific but are important to determine the tumour extension and to plan the biopsy. Extramedullary plasmacytoma (EMP) accounts for 3% of myeloma cases and is usually localized in submucosal lymphoid tissue of nasopharyngeal and paranasal sinuses . Soft tiA 52-year-old male was referred to the orthopaedic surgeon for evaluation of a painless bulge in his calf detected 1 year earlier. An MRI was performed in a Signa 1.5 T GE, obtaining axial and sagittal T1-  and T2*-  weighted images. A homogeneous, oblong and well-defined mass was detected superficial to the Achilles tendon. It was isointense on T1-weighted images and hyperintense on T2*-weighted images and showed homogeneous moderate enhancement after intravenous gadolinium injection .A Tru-Cut biopsy was performed, and the pathology report described a neoplastic proliferation of differentiated plasma cells. Immunochemistry studies showed cells to be positive to CD138 (plasma cell marker) and negative to CD20 (accompanying B-lymphocytes) and CD3 (accompanying T-lymphocytes). These findings supported the diagnosis of plasmacytoma . The preThe patient was treated by radiotherapy. A very small residual flat thickening of soft tissues, which shows no activity on PET-CT, remains unchanged after four years of followup. The patient is considered to be in complete remission.Myeloma usually appears as a generalized disease (multiple myeloma). In a small proportion of patients (<5%), it can present as solitary bone plasmacytoma (SBP) or a solitary soft tissue mass, extramedullary plasmacytoma (EMP) .Primary plasmacytoma, whether osseous or nonosseous, is distinguished from multiple myeloma by absence of hypercalcaemia, renal insufficiency and anaemia, normal skeletal survey, absence of bone marrow plasmacytosis, and serum or urinary paraprotein <2 g/dL .The diagnosis of EMP requires demonstration of a monoclonal plasma cell infiltrate with no evidence of myeloma at any other site. Therefore, patients with presumed solitary plasmacytoma of bone or soft tissue (extramedullary) plasmacytoma should undergo a radiological bone survey and MR imaging to ensure that only one lesion is present. Imaging studies should focus on the early detection of additional or recurrent lesions and the presence of regional lymphadenopathy, which will influence the clinical management.Solitary plamocytomas can arise at any site, but almost 90% develop in the head and neck area and especially in the upper respiratory tract . Infrequent sites of involvement include the gastrointestinal tract, liver, spleen, pancreas, lungs, thyroid, breast, testis or skin , 5. SoliMalignant fibrous histiocytoma is the most frequent mesenchymal malignancy and accounts for around 24% of all soft-tissue sarcomas, with 7–10% of cases confined to the subcutaneous tissue, where it may or not extend to the fascia. Subcutaneous lesions tend to be smaller, implying a better prognosis. MR imaging of soft-tissue malignant fibrous histiocytomas reveals lesions with intermediate to low signal intensity on T1-weighted images and inhomogeneous high signal intensity on T2-weighted images. Inhomogeneity may be due to calcification, myxoid degeneration with cystic cavities or necrosis. After gadolinium administration, irregular enhancement is seen in the viable tumour .Synovial sarcomas account for around 10% of all soft-tissue sarcomas. The MR signal is usually complex, due to the presence of fluid levels, haemorrhage and internal septation. Neoplasms with a diameter <5 cm may have a more benign appearance, with well-circumscribed margins and homogeneous high signal intensity on T2-weighted images .Among fibrous tumours, nodular fasciitis can present as a localized and circumscribed subcutaneous fascia-based mass. The histologic diversity of nodular fasciitis likely accounts for the variable MR appearance of the lesions. In hypercellular lesions, they can appear isointense to slightly hyperintense relative to skeletal muscle on T1-weighted images and hyperintense to adipose tissue on T2-weighted images. Highly collagenous lesions show a hypointense signal on all MR images. Contrast enhancement is typically diffuse and homogeneous, except in cases of myxoid degeneration leading to cystic degeneration .Soft-tissue haemangiomas can arise at various anatomic sites, including striated muscle, skin, subcutaneous tissue and synovial tissue, depending on the histologic subtype. If sufficiently large, they can manifest as a smooth, palpable soft-tissue mass. MR imaging reflects the mix of tissues present in the haemangioma. It usually shows heterogeneity, due to the variable amount of fat, arteries and veins, which can present a serpentine pattern. Nevertheless, small lesions with no fat content can have a homogeneous signal and well-defined border .Subcutaneous neurofibromas are usually associated with neurofibromatosis-1. An entering and exiting nerve cannot usually be identified in superficial lesions. They generally present as a fusiform-shaped mass with a signal that is isointense to skeletal muscle on T1-weighted MR images and hyperintense to skeletal muscle on T2-weighted images, with variable degrees of inhomogeneity and enhancement .Musculoskeletal involvement by lymphoma can occur in disseminated disease or as an isolated manifestation . The skin is the second most common site of extranodal involvement of Non-Hodgkin Lymphoma after the gastrointestinal tract. Imaging features of cutaneous lymphoma are nonspecific and include soft tissue thickening, infiltration or a mass .Most of the above diagnoses have to be considered before soft tissue EMP is diagnosed. Although EMP findings are not specific, imaging can be used to delineate the extent of the mass, plan a biopsy and rule out other lesions. In MRI studies, it has been described as a mass that is isointense or slightly hyperintense on T1-weighted images and isointense to high hyperintense on T2-weighted images, with homogeneous-to-heterogeneous enhancement on post-contrast T1-weighted images . Its appThe prognosis is significantly better for EMP patients than for SBP patients, with two-thirds surviving >10 years , and curIn summary, we present a rare case of subcutaneous fascia-based EMP, reporting the differential diagnosis with other more common soft tissues mass. Imaging findings are nonspecific but invaluable for studying the extent of the tumour and planning the biopsy.


A 69-year-old woman with a history of uT2 N0 post-treated anal squamous cell cancer (SCC) presented for EUS for perianal pain. Two months prior, a digital rectal examination was significant for an indurated lesion on the left lateral rectal wall just proximal to the dentate line. A sigmoidoscopy revealed mild narrowing of the anal canal and an ulcerated friable mucosa in the same area. A biopsy demonstrated ulceration without malignancy. EUS showed a hypoechoic, non-circumferential, left-sided distal rectal mass. EUS-FNA was performed. Cytology demonstrated poorly differentiated SCC. This was confirmed by subsequent surgical resection. While endoscopic biopsy of suspected anal recurrences is usually sufficient, histologic or cytologic confirmation are necessary, as radiation-induced changes are difficult to differentiate from tumor recurrence. This case demonstrates that EUS-FNA is useful in surveillance of anal SCC when there is a high clinical suspicion of recurrence. Anal squamous cell cancer (SCC) is an uncommon diseaseA 69-year-old white woman with a history significant for hypothyroidism and neck immobilization was diagnosed with uT2 N0 anal squamous cell carcinoma (SCC) of the left lateral anal canal. She initially presented with perianal pain. A pelvic CT scan performed at the time of diagnosis was unremarkable. She underwent chemotherapy and external beam radiation. Her chemotherapy regimen consisted of mitomycin C and oral capcitebine. Following chemotherapy and radiation, a repeat lower endoscopy with an anal biopsy was performed which revealed persistent SCC in situ. She then underwent a second course of chemotherapy and radiation.Four months later during follow up an indurated lesion on the left lateral rectal wall adjacent to the dentate line and an anal stricture was appreciated on digital rectal examination. A gastroenterologist performed a sigmoidoscopy which was significant for a left lateral rectal ulcer and an anal canal stricture. Biopsies were taken from the rectal ulcer and anal dilation was performed. The biopsies were negative for malignancy. One month later, the patient presented with rectal bleeding and severe perianal pain exacerbated with defecation and sitting. A second sigmoidoscopy revealed mild narrowing of the anal canal and ulcerated friable mucosa in the left lateral distal rectum involving the anal canal. Repeat biopsies of the ulcerated mucosa in the rectum and anal canal demonstrated ulceration without malignancy.One month later and 6 months after completing her second course of chemotherapy and radiation, she presented for EUS of the anal canal. Prior to the EUS, the gastroenterologist performed a digital rectal examination. The examination was significant for exquisite tenderness and induration of the left lateral anal canal and distal rectal wall extending approximately 2 cm from the anal verge.On radial EUS  examination a hypoechoic, left-sided distal rectal mass was seen . The masThe patient underwent abdominal perineal resection and colostomy. The posterior wall of the vagina, anus, and rectum were involved with poorly differentiated SCC . The tumEndoscopic ultrasound (EUS) provides accurate TNM staging and has been used to determine clinical responsiveness after treatment.7This case demonstrates the utility of EUS-FNA after non-diagnostic endoscopic biopsies were taken. The EUS-FNA cytopathology result was not able to differentiate between keratinizing and non-keratinizing SCC. However, given similar biology, prognosis and treatment, this differentiation is not necessarily required for management of anal SCC.To our knowledge, this is the only report of EUS-FNA used to diagnose post-treatment anal cancer recurrence after negative cold forceps biopsies. Our case demonstrates that EUS-FNA may be useful in surveillance of anal SCC following treatment when there is a high clinical suspicion of recurrence. Further prospective study of EUS surveillance of anal SCC recurrence is warranted in select patients.


Glomus tumor is an uncommon neoplasm derived from cells of the neuromyoarterial glomus or glomus body. Most glomus tumours occur in the dermis and subcutaneous tissues. A case of a primary pulmonary glomus tumour originating in the right upper lobe is presented.A 74-yr-old male was admitted with siccus cough, dyspnea and right-sided chest pain. Computed tomography of the thorax revealed a 4 cm growth of the right upper lobe. Fiberoptic bronchoscopy demonstrated an endobronchial hypervascular mass causing obstruction of the apical segmental bronchus. Pathology report was consistent with pulmonary glomus tumor. The patient underwent a typical right upper lobectomy with mediastinal lymph node dissection. Twelve months later he is free of disease.th one presented in the international literature) and are often confused with other solid neoplasms such as carcinoids, hemangiopericytomas and tumors belonging to the family of Ewing's sarcoma/primitive neuroectodermal tumours.Occasionally glomus tumors can occur in extracutaneous sites such as the gastrointestinal tract, bone, genitourinary system and respiratory tract. Primary pulmonary glomus tumors are very rare (our case is the 19 Glomus tumors are neoplasms originating from glomus bodies in the dermis or subcutis of the extremities . ExtracuA 74-year-old smoking male patient was referred with a persisting siccus cough, dyspnea and right-sided chest pain. Apart from hypertension, his history was negative.Physical examination and routine laboratory tests were normal. Chest x-ray revealed a right upper lobe growth. Chest computed tomography (CT) showed a tumor without inlying calcifications in the parahilar region of the right upper lobe, with a size of 4.0 × 2.6 cm Figure . PositroThe patient underwent a right mucle-sparing anterolateral thoracotomy and a right upper lobectomy with mediastinal lymph node dissection.Macroscopically, a circumscribed soft mass, measuring 3,4 cm in greatest dimension, with white to pink cut surface was found. Histologically, the tumor was encapsulated and was composed of sheets and nests of small, uniform, rounded cells with centrally placed, round nuclei; amphophilic to lightly eosinophilic cytoplasm and prominent nucleoli (glomus cells) surrounding capillary sized vessels Figure  and vimeThe patient made an uneventful recovery. Twelve months later he is free of disease.Solitary glomus tumors are more frequent in adults than in others. Multiple glomus tumors develop 10-15 years earlier than single lesions; about one third of the cases of multiple tumors occur in those younger than 20 years. Congenital glomus tumors are rare; they are plaquelike in appearance and are considered a variant of multiple glomus tumors.Glomus tumours can be subdivided pathologically into glomus tumour proper, glomangioma and glomangiomyoma, based on the relative predominance of the three major constituents: round glomus cells in glomus tumour proper; blood vessels in glomangioma; and spindle cells in glomangiomyoma. Glomus tumour proper is the most common, followed by glomangioma. Glomangiomyoma is the rarest variant with a frequency as low as 8% of all glomus tumours . Glomus The differential diagnosis consists of a wide variety of neoplasms, most notably: carcinoid tumor, hemangiopericytoma, paraganglioma, smooth muscle neoplasms and metastatic tumors . Carcinoth one. Complete surgical excision is the treatment of choice with excellent prognosis [Despite that intrapulmonary glomus tumors are generally benign neoplasms, four malignant cases have been described so far, with the present case to be the 5rognosis -11.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.Authors' contributions AK, NB, CA, IK, EK, AG and JA took part in the care of the patient and contributed equally in carrying out the medical literature search and preparation of the manuscript. AN and AB were responsible for the pathology report. All authors approved the final manuscript.


Renal cell carcinoma is characterized by its potential of metastasizing widely and to unusual sites, with the metastases occasionally preceding clinical recognition of the primary tumor. Synchronous bilateral adrenal metastases from renal cell carcinoma, without other metastases, are rare and, to our knowledge, only 17 cases have been published in the literature to date. In general, patients with synchronous bilateral adrenal metastases from renal cell carcinoma have a poor prognosis.We report a case of right-sided renal cell carcinoma with simultaneous bilateral adrenal metastases in a 58-year-old woman. The primary tumor was localized in the upper and mid pole of the kidney. The diagnosis was established preoperatively by abdominal ultrasound and computed tomography. Surgical treatment consisted of a right radical nephrectomy and bilateral adrenalectomy. Postoperative cortisone acetate replacement was instituted. The pathological findings of the right renal tumor showed clear cell carcinoma and both adrenal tumors showed the same pathology as the right renal tumor. There was no evidence of recurrence after 6 months of follow-up.Patients with bilateral synchronous adrenal metastases should be considered to have disseminated metastatic disease. However, good performance status, the presence of paraneoplastic syndrome and the alleviation of refractory pain are important reasons make an urologist to consider radical nephrectomy in renal cell carcinoma patient with metastases. It is well established that the incidence of adrenal metastases in certain types of malignancy, such as carcinomas of the breast, lung and melanoma is high . Renal cA 58-year-old Caucasian-Greek woman presented with a dull pain located in the right flank for 4 years. Other symptoms, including hematuria, were not present. The patient had a medical history of spondylolisthesis, osteoporosis, hypertension and hyperlipidemia. The gradual deterioration of pain in the last month before her admission caused her to seek medical attention. The pain initially was attributed to spondylolisthesis and she was prescribed a relative medication. Due to the persistence of pain despite the administration of the specific medication she was submitted to sonography. The ultrasonography revealed a right upper pole renal mass and bilateral adrenal masses. Because of these findings she was admitted for further evaluation. A physical examination revealed a mild tenderness in her right flank. No lymphadenopathy was found. The patient’s blood pressure and the pulse rate were normal. A contrast-enhanced abdominal computed tomography (CT) confirmed the presence of a 6 cm × 5 cm × 4 cm solid mass at the upper and mid pole of the right kidney with heterogeneous and enhanced areas and bilateral involvement of the adrenal glands . A chestRight radical nephrectomy was performed through a bilateral subcostal incision (Chevron). The contents of Gerota’s fascia were removed en bloc, followed by a total left adrenalectomy. Thorough exploration of the abdominal cavity did not disclose any other metastatic focus.Histological examination of the resected specimens revealed a solid tumor with golden-yellow appearance, relatively well circumscribed and measuring 5.6 × 4.8 × 3.5 cm in the upper half of the right kidney. Almost the entire left adrenal gland, measuring 6.2 × 3.1 × 2.7 cm, was occupied by a brownish compact tumor while only a thin rim of adrenal tissue was preserved. A same mass, with a maximum diameter of 1.9 cm, was also observed in the right adrenal gland. Microscopic examination of the renal and both adrenal gland tumors revealed the diagnosis of clear cell renal carcinoma, Grade3 and pT3 according to the TNM classification system . The carConvalescence was uneventful. The dosage of cortisone was gradually tapered to 12.5 mg per day orally. Serum cortisol and electrolytes were normal during the follow-up period. She was discharged 10 days after the operation.The patient is alive with no evidence of disease recurrence or metastatic disease after six months postoperatively treated with adjuvant chemotherapy.Adrenals involvements are usually silent because metastatic lesions fail to destroy enough tissue to produce clinical signs or symptoms of insufficiency. However, adrenal function is usually maintained even though bilateral adrenal glands are affected by malignancy. There is a higher incidence of upper pole primary renal tumors than either mid or lower pole renal tumors in patients with adrenal metastases . PatientThe presence of ipsilateral adrenal involvement appears to portend a poor prognosis. These patients have high-stage primary tumors (T3 or greater). Antonelli et al.  concludePatients with RCC and a single contralateral adrenal metastasis should be considered as having a solitary metastasis. They should undergo radical nephrectomy and contralateral adrenalectomy. Ipsilateral adrenalectomy should be performed if there is suspicion of metastasis . These pPatients with bilateral synchronous adrenal metastases from RCC should be considered to have disseminated metastatic disease. There are only 18 cases of bilateral adrenal metastases from RCC, without other metastases, that have been reported in the literature, including our case. The follow-up duration is less than 4 years. Despite prophylactic treatment after the operation with chemotherapy and immunotherapy the prognosis remains poor.An alternative treatment option in cases of RCC with bilateral adrenal metastases is partial adrenalectomy, sparing the normal adrenal tissue . This prHowever, the goal of any surgical or medical procedure should be to improve the quality of life and duration of survival. Radical nephrectomy in RCC patients with metastases may be considered in the presence of paraneoplastic syndrome provided the patient is of good performance status. Surgical intervention may also be indicated for palliative reasons. In our case, the patient hadn’t paraneoplastic syndrome, was in good performance status, and the surgical intervention performed for the alleviation of severe, refractory pain.In our opinion, some patients with bilateral adrenal metastases from RCC and without metastases in other site may be benefited by radical operation, as regards their overall survival, because the reduction of the tumor burden may enhance response rates to postoperative adjuvant therapy.


An 83 year old caucasian gentleman presented with vomiting and left sided abdominal pain. A subsequent upper GI endoscopy demonstrated a large smooth mass impacted within the duodenum. A cholecysto-duodenal fistula was discovered at laparotomy, with a large gallstone impacted in the duodenum. A diagnosis of Bouveret's syndrome was made. The management of this rare cause of gastric outlet obstruction is discussed. Gallstones, in the majority of patients remain asymptomatic. The commonest clinical manifestation is biliary colic. Gallstone ileus occurs when a stone enters the intestinal tract via a cholecysto-enteric fistula. The authors present a case of Bouveret's syndrome, a rare complication of gallstone disease and rare cause of gastric outlet obstruction.An 83 year old gentleman was admitted with a one week history of vomiting after eating and left-sided upper quadrant abdominal pain. There was no history of dysphagia or weights loss. The patient had suffered a similar episode the year previously which had resolved spontaneously. Abdominal examination was unremarkable. No succession splash was evident. A full blood count, liver function tests and urea and electrolytes were normal. No free air under the right hemi-diaphragm was noted on a chest radiograph. A plain abdominal film was negative for evidence of aerobilia or gallstones. A naso-gastric tube was inserted. The patient subsequently underwent an oesophago-gastro-duodenoscopy (OGD) to exclude possible mechanical obstruction. At OGD, a mass was noted beyond the pylorus Figure . In the Computed tomography (CT) of the abdomen demonstrated a large calcified mass in the first part of the duodenum Figure . The patPost operatively the patient continued to produce large gastric aspirates via a naso-gastric tube. A repeat OGD demonstrated that both afferent and efferent loops were patent. The large gallstone was noted once again but this time in the second part of the duodenum. The patient returned to theatre where this time the gallstone Figure  was succGallstone ileus is rare . The majTreatment options include endoscopic and surgical management. Endoscopic removal should always be attempted first, but lithotripsy and stone extraction is rarely successful . IntracoThe authors present a case of Bouveret's syndrome in an 83 year old gentleman. The diagnosis should be considered in patients with symptoms of gastric outlet obstruction with or without a history of gallstones or aerobilia and typical endoscopic findings of a dilated stomach and a hard non-fleshy mass at the obstruction.The author(s) declare that they have no competing interests.DJ, AV and TR were involved in writing of the case report. CF and AD were involved in the review and re-writing of the case report. All five authors were involved in the patient's care.


Oncogenic osteomalacia is a rare association between mesenchymal tumors and hypophosphatemic rickets. It is more of a biochemical entity than a clinical one. The pathophysiology of the tumor is not clear. However, it has been seen that the clinical and biochemical parameters become normal if the lesion responsible for producing the osteomalacia is excised. For a clinical diagnosis a high index of suspicion is necessary. We present three such cases where in one the oncogenic osteomalacia reversed while in rest it did not. We present this case report to sensitize about the entity. Oncogenic osteomalacia is a rare endocrinological paraneoplastic syndrome associated with large or very small mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities, including hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase.2μg twice daily. At follow-up after 2 years, the radiographs show sound union [A 16-year-old female was admitted with complaints of pain in the left thigh and inability to bear weight on the left leg since 1 day, following a trivial fall. Her left thigh was swollen, tender and demonstrable abnormal mobility, and crepitus was felt on attempted range of motion of hip examination. Radiographs of the femur showed a lytic area, with pathological fracture of the mid-shaft of the femur . The patnd union . Her metcafé au lait spots on the back and abdomen [An 8-year-old female visited the outpatient department (OPD) with complaints of gradual-onset, generalized bone pains and deformity of both lower limbs  since 3 years. There was no preceding history of fever, trauma, or weight loss. On examination, she had bilateral genu valgum, widening of the wrist, frontal bossing, poor dentition, pectus excavatum, double malleoli sign, bony tenderness, significant  abdomen , blue sc abdomen . X-ray o abdomen . Her mot abdomen . UltrasoAt 18 months’ follow-up her bone pains are controlled and the intermalleolar distance has been reduced to 11 cm. X-ray shows evidence of healing rickets . Her bio3 volume) cavity filled with high straw/brown-colored fluid. The cavity had few membranes which were curetted out. The cavity was then packed with autologous fibular grafts and corticocancellous grafts. The fracture was secured with a dynamic compression screw and plate. She was kept on skeletal traction for 8 weeks and then mobilized on ischial weight-relieving calipers and axillary crutches. Postoperatively she was given oral phosphate 500 mg thrice daily, oral elemental calcium 1 g twice daily, and alpha-hydroxylated vitamin D 0.25 μg twice daily. Histopathological examination showed fibrous dysplasia. At follow-up at 1.5 years the fracture had united [A 38-year-old female presented to the emergency department with complaints of pain in the left hip and inability to bear weight for 2 days, following taking a sharp turn while walking. Clinical examination revealed swelling, tenderness, bruises, abnormal mobility and crepitus around a subtrochanteric area. Radiographs of the left hip and femur showed a pathological subtrochanteric fracture, with a lytic lesion in the proximal femur . The patd united  and labod united . The patet al. 40 years ago.3Oncogenic osteomalacia is a term given to a rare endocrinological paraneoplastic syndrome characterized by defective bone mineralization as a result of renal phosphate loss. McCance reported the first case of oncogenic osteomalacia in 1947 in the distal femur of a 17-year-old girl.The age distribution of patients ranges from 7 to 77 years, with the male:female ratio being 1.2:1. The onset is gradual and the patient presents with joint deformities, bone pain, waddling gait, muscle weakness, fatigue, anorexia and, rarely, long bone fractures. The clinical course is protracted.99mTc-sestamibi scan111, octreotide scintigraphy,201 TI scintigraphy, 99mTc-MIBI SPECT, and venous sampling for FGF23 with MR imaging.The metabolic picture of this syndrome shows hypophosphatemia, hyperphosphaturia, low or normal serum calcium, raised alkaline phosphatase, normal PTH, low concentrations of 1, 25-dihydroxycholecalciferol, decreased tubular resorption of phosphates,1In the two cases of fibrous dysplasia, one was polyostotic while the other was solitary. We did not have bone scan to document the polyostotic nature, however skeletal survey was done in every case and revealed fibrous dysplasia in a rib and the scapula in case 3. PET/SPECT can be helpful in cases of polyostotic fibrous dysplasia when the lesion inducing oncogenic osteomalacia is not known.in vitro, while only small amounts of FGF-23 were present. FGF-7 derived from oncogenic osteomalacia–producing tumor cultures was confirmed to be potent inhibitor of phosphate transport in vitro.4In oncogenic osteomalacia the biochemical and clinical abnormalities are caused by a circulating factor produced by the neoplasm. Tumor extracts injected in dogs, mice, and rats caused phosphaturia in experimental studies.1113The treatment of choice in oncogenic osteomalacia is tumor removal.However, the problems in management continue if one is not able to detect the oncogenic osteomalacia–producing tumor. Hence, one has to continue with calcium and phosphorus supplements, probably throughout life. With this treatment the clinical symptoms like bone pains, fatigue, and lethargy remains well controlled, while the biochemical parameters never return to normal. This happened in two of our cases in which polyostotic fibrous dysplasia and neurofibromatosis were the diagnoses. In cases of diffuse tumor leading to this condition, high doses of calcitriol and oral phosphate are indicated as in the treatment of X-linked hypophosphatemia.In conclusion, the oncogenic osteomalacia syndrome is caused by a protein secreted from oncogenic osteomalacia–producing mesenchymal tumors, and it manifests as osteomalacia with hypophosphatemia and hyperphosphaturia. Excision of the tumor causes improvement in general health and blood parameters. However, identification of the oncogenic osteomalacia-producing lesion is difficult, and this delays diagnosis and makes management lengthy. A high index of suspicion is needed to diagnose this entity.


A 16-year-old female patient without any of the known risk factors presented with history of gradual-onset hoarseness of voice unrelieved by symptomatic treatments for 1 year. Contrast-enhanced CT scan of neck and laryngoscopy and histopathology of the tissue from irregular lesions along the medial margin of the left vocal cord diagnosed it as a case of carcinoma in situ of vocal cord. Absence of known risk factors and very young age of the patient made this case a rarity and hence the case is being reported.Carcinoma  Vocal cord cancer is usually a disease of the middle-aged chronic smoker, being more common among males (M: F = 3:1). Prior neck irradiation for some other disease is also a risk factor. Glottic cancer is more common than supraglottic cancer. Heavy smoking and adult age are the main known risk factors.in situ [Figures in situ of the vocal cord.A 16-year-old female patient presented to the radiotherapy department in July 2008 with complaint of unrelenting hoarseness of voice for the last 1 year. She developed it gradually over a period of time. Initial treatment was directed to common cold but was of no help. There was no associated sore throat, otalgia, localized pain or tenderness over thyroid cartilage, dysphagia or features of airway obstruction. The patient is a nonsmoker and is nonalcoholic and without any history of past or present marijuana smoking. There was no history of prior neck irradiation. An incidence of cancer in the family could not be elicited from a detailed history. An indirect laryngoscopy was performed, which showed both vocal cords to be mobile. There was no visible growth over any of the vocal cords. Contrast-enhanced CT scan of the neck was performed, and it showed a small irregular lesion over the left vocal cord at the junction of anterior one third and posterior two thirds . Thicken Figures  and 4. HA detailed physical examination was performed, which was unremarkable. There was no palpable lymph node in neck and other sites. Complete hemogram, liver function test, chest X-ray, renal function test and ultrasonography of the whole abdomen were performed as part of routine investigations before commencing therapy, all of which were unremarkable. The stage of the disease was Tis N0 M0.After reaching at a specific diagnosis and doing all pretreatment workup, specific anticancer therapy was started with radical radiotherapy  using Telecobalt (Theratron 780 C) machine. Radiotherapy was started with two lateral opposed fields to neck to include the vocal cord (a 5 × 5 cm field) extending from superior border of thyroid cartilage superiorly to lower border of cricoid cartilage inferiorly sparing the spinal cord and other organs at risk. A CT-based treatment planning was done to achieve maximum dose to vocal cord, sparing normal tissue as much as possible.in situ is common in vocal cord. Distinction between carcinoma in situ, micro-invasive squamous cell carcinoma and invasive carcinoma may be troublesome in many cases. The usual patient with laryngeal cancer is a middle-aged male chronic smoker. At presentation about 51% are localized to larynx, 29% have regional spread and 15% have distant metastasis.[Laryngeal cancer is a rare entity among children and yountastasis. Cigaretttastasis. Prior irIt should be noted that our case is unique in the sense that all these known risk factors were absent in this patient.Squamous cell carcinoma is the major histopathological type. Less frequent pathological subtypes are spindle cell carcinoma, verrucous carcinoma, neuroendocrine tumor and minor salivary gland tumor. There arHoarseness of voice not responding to treatment for common conditions is the most common presenting symptom for most of the early-stage vocal cord cancers. Advanced cancers may present with sore throat, otalgia, localized pain and tenderness over thyroid cartilage; or sometimes, dysphagia. If the tumor is bulky or large enough, it can produce symptoms and signs of airway obstruction. Incidence of clinically positive neck nodes varies according to the tumor stage: T1, approximately 0; T2, ≤ 2%; T3, T4, 20%-30%. Contrast-enhanced CT scan of the neck is the investigation of choice for laryin situ of true vocal cord may be treated by stripping of the lesion, CO2 laser excision and early irradiation. However, early irradiation often provides better voice preservation[in situ.Patients diagnosed as having carcinoma servation and elimIt may be stated that vocal cord carcinoma in a 16-year-old female without any risk factor is a rare entity, and clinicians should be aware of the possibility of this condition. Aggressive treatment has a chance of better outcome. Delay in suspecting, diagnosing and treating this disease may result in decreased cure rate.


Gastrointestinal stromal tumors are rare visceral sarcomas arising in the gastrointestinal tract wall. In this report we present a case of gastrointestinal stromal tumors with mesenteric and retroperitoneal invasion, describe and discuss its computed tomography findings.® biopsy was made, histopathologic and immunohistochemical analyses demonstrated stromal tumor which, C-KIT (+). The patient underwent left ureterectomy, left nephrectomy and total colectomy. Postoperative histopathological analyses revealed lower grade malignant GISTs. As of 17 months after the surgery, he is alive and free of recurrence.A 57-years-old male patient has been complaining of abdominal distention, weight lose, and hematuria. During physical examination, significant distention and multiple palpable tumor masses were identified on the abdomen. Abdominal computed tomography showed multiple, well-defined, soft tissue masses with homogenous and heterogeneous pattern, in the mesenteric and retroperitoneal areas. Unlike specific features of gastrointestinal stromal tumor, renal obstruction and atypical central calcification without chemotherapy that has not been yet described were seen in this case. Computed tomography did not reveal liver metastases and/or the lymph nodes with pathological size. Ultrasonography-guided true-cutWhen intraabdominal, multiple, large (>5 cm), well-circumscribed, homogenous or heterogeneous mass lesions without ascites, omental caking and lymph nodes metasteses were seen, gastrointestinal stromal tumors should be considered in the differential diagnosis. Gastrointestinal (GI) stromal tumor (GIST) is a rare visceral sarcoma arising in the gastrointestinal tract wall ,2. This ®, Siemens Medical Solutions, Erlangen, Germany). Multiple, well-defined, soft tissue masses with homogenous pattern, measuring maximum 6 × 5 cm in size were observed in the mesenteric and retroperitoneal areas -guided true-cutc Figure , 6 and ic Figure , CD34  and retroperitoneum because of the existance of an attachment of serosa of GI tract . The tumHe had an uneventful postoperative course and was discharged home on postoperative day 3. As of 17 months after the surgery, he is alive and free of recurrence.CT, magnetic resonance imaging (MRI) and fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) can be used for evaluation of GIST. Contrast-enhanced CT scan is, however, more widely available and is currently the imaging modality of choice for patients with suspected abdominal mass or biopsy-proven GIST. CT is used in staging and surgical planning. When a small tumor is found incidentally during endoscopy, the local extent of the tumor should be evaluated using either endoscopic ultrasound or CT. For those with known or suspected rectal GIST, a dedicated MRI provides better information than CT scan in preoperative staging work-up. Evaluation of FDG uptake using PET scanning is recommended when an early detection of tumor response to imatinib treatment is required .CT findings of GISTs are well described in the radiological literature -13. Maliet al., reported a GIST of the peripancreatic retroperitoneum with CT and MR imaging findings representing huge well-defined inhomogeneous mass with peripheral calcification [Exceptional to the reports published so far, Takao fication . In our The spread pattern of these tumors shows predilection for liver metastasis and peritoneal dissemination . In thatet al., reported that stromal tumors rarely obstruct viscera, despite their large size [et al., 116 malignant GIST were analyzed, and only one case has this behavior [Burkill rge size . In our behavior .et al., concordantly with our case, reported that ascites and omental caking were unusual findings in stromal tumors [In our case, we have seen multiple peritoneal tumor masses without peritoneal fluid. Burkill, l tumors .Tumor size, anatomic location, and mitotic count are considered independent prognostic factors for GISTs ,16, and ® biopsy and then histopathologic and immunohistochemical analyses were made. However, preoperative percutaneous biopsy carries a theoretical risk of peritoneal seeding or tumor rupture, and is indicated only for clearly irresectable disease or when treatment needs to be altered, as would be the case if the mass proved to be lymphoma or germ cell tumor [The differential diagnosis of an abdominal mass with well defined, extraluminal masses and homogeneous enhancement in the abdominal cavity includes peritoneal implants, lymphoma, primary peritoneal carcinomatosis, carcinoids, and other soft-tissue tumors  ,12,23. Ill tumor . One altll tumor .Immunoreactivity for CD117 (C-KIT) is a characteristic of GISTs; in addition, most GISTs express CD34 immunostain. Another marker that is considered useful in the assessment of GISTs is Ki-67 immunostain, with reported 100% specificity and sensitivity for malignancy on GISTs . ImmunohWhen intraabdominal, multiple, large (>5 cm), well-circumscribed, homogenous or heterogeneous mass lesions without ascites, omental caking and lymph nodes metasteses were seen, GIST should be considered in the differential diagnosis.The author(s) declare that they have no competing interests.CT examinations and writing of this case report were performed by Gulgun Engin. The case was operated by Oktay Asoglu and histopathological and immunohistochemical analyses were performed by Yersu Kapran. Gulsen Mert was contributed to the study by reviewing the pathology of tumor and by taking photos of the microscopic appearances of the tumor.


The presentation of ingested foreign bodies in the gastrointestinal system is common in the emergency setting. The majority responds to conservative management and passes spontaneously; however, giant foreign bodies pose a management difficulty. We report a peculiar case of a giant foreign body (spoon) that presented very late after ingestion and the management of this presentation.A 30-year-old British white male barrister presented with abdominal pain 10 years after he swallowed a spoon that never passed spontaneously. His workup revealed the spoon lodged in his ascending colon. Laparoscopic retrieval was not feasible so a laparotomy was done for retrieval. He did well and went home with no complications.Symptomatic giant ingested foreign bodies represent a management challenge sometimes and usually necessitate surgical intervention when all conservative means fail. We review the literature on management of giant ingested foreign bodies. In this article we present an unusual case of ingested giant foreign body that necessitated surgical intervention for its retrieval. A previously known fit and healthy British white 33-year-old male presented 10 years after he swallowed two tea-spoons while he was under the influence of alcohol. He passed one spontaneously per rectum a year after ingestion, but the other never passed. A subsequent CT scan showed the remaining spoon still in the ascending colon and was reported as being intraluminal. He was lost to follow up until he started experiencing some vague abdominal pain in the right iliac fossa intermittently which he addressed with regular over-the-counter analgesia and that managed his symptoms adequately at first. He never experienced severe pain, nausea or vomiting, neither the passage of any blood per stool. His laboratory profile seems within normal with a hemoglobin of 13.8 and white blood cells of 7.2 and C-reactive protein of less than 5. His dietary intake and bowel habits were unchanged. A plain abdominal X-ray confirmed the presence of the spoon in the right iliac fossa . SubsequIntraoperatively, open Hasson port pneumoperitoneum was achieved but initial laparoscopy revealed dense adhesions of the ascending colon to the anterior abdominal wall with the ascending colon and hepatic flexure very adherent to the retroperitoneum hindering adequate exposure and tactile feedback of the spoon’s position. There was also thick scar tissue and edema rendering the lateral side of the right colon adherent to the abdominal wall; and this is probably due to micro-perforations at the anterolateral border of the proximal ascending colon. In view of these findings the procedure was converted to a laparotomy through a right transverse incision where open lysis of adhesions was safely and meticulously carried out revealing the position of the spoon confirmed by manual palpation. A colotomy was performed, the spoon delivered and extracted. The colotomy was closed using a two layer technique. The spoon measured 13.5 centimeters long and 2.5 centimeters wide . The patForeign body (FB) ingestion is a common presentation to the surgical team, usually as children accounting for about 80%; the remainder usually including prisoners, psychiatric patients, alcoholics, and senile patients ,2. Most Complications of FB ingestion range from hemorrhage, bowel obstruction, perforation and erosion into adjacent viscera. Hemorrhage occurs when the FB injures the mucosa or lodges in a region close to visceral artery like in the pylorus eroding into the gastroduodenal artery. Bowel obstruction can also occur when the FB is bigger than any of the narrow anatomical areas of the intestinal tract explained above; hence lodging there and causing obstruction. This can progress to hemorrhage when it erodes into an artery or injures the mucosa. It can perforate freely in the peritoneum or extra-peritoneally when it reaches the distal rectum presenting with abscess and rectal bleeding .Intra-abdominal perforations can be acute or chronic. They may present acutely with peritonitis when they perforate freely. A chronic perforation may present as an inflammatory mass or abscess. Certain perforations may be completely asymptomatic when the perforation has been sealed of by the body defense mechanism and inflammatory reaction, such as this presentation. It is not uncommon that the presentation of an ingested foreign body may be more indolent. The perforation can occur slowly when the FB gets stuck at anatomical angulations or narrowing such as the ileocecal valve and the sigmoid giving ample time for the omentum and the surrounding viscera to seal off the slowly occurring perforation. It may eventually result in the FB eroding into these structures with a large amount of inflammation accompanying this process. Furthermore, parts of the duodenum and the colon are retroperitoneal and a perforation there may not present with the classical signs and symptoms of perforation but have a more subtle presentation . In thisSharp foreign bodies are the most challenging to manage; the most common are toothpicks, nails, bones, blades, teeth, dental prosthesis, pins, and needles. Amongst these the most common to require surgical intervention for extraction are toothpicks and bones . UsuallyWe feel that laparoscopic procedures should be attempted in all such cases owing to its well documented advantages such as reduced postoperative pain, shorter hospital stay, decreased ileus, and faster return to regular activity. Laparoscopy can be used to retrieve small sharp objects like pins and blades or even larger longer objects like forks ,10. In tIn conclusion, ingested FBs in most scenarios pass spontaneously. The size and nature of the FB may be a limiting factor. Of those that do not pass spontaneously, about 20% are endoscopically removed from the stomach and duodenum. Only 1% cause acute presentations like perforations and obstructions and warrant surgical intervention. It is advisable that all patients with a history of ingested and non passed FBs be closely followed up, properly evaluated and surgery advised to prevent complications in the event that these FBs are not passed spontaneously within a year of ingestion.


Small bowel tumors are rare, accounting for only 3-6% of gastrointestinal neoplasms, 1-2% of these being malignant. They must be considered whenever a patient presents with gastrointestinal bleeding, with normal upper gastrointestinal endoscopy and colonoscopy.We report a case of jejunal adenocarcinoma presenting as a blood loss anemia in a 65 year-old male, doing a brief review on the subject.Our case intends to highlight the fact that small bowel tumours are rare and frequently present to the Internist as non-specific clinical symptoms. Small bowel tumors are rare, accounting for only 3-6% of gastrointestinal neoplasms, 1-2% of these being malignant [A 65 year-old white male began symptoms of weakness for medium efforts in March 2007. Two months later, during one week, he had 2 episodes of dark feces, associated with periumbilical pain, irradiating to the flancs and epigastric area. He denied heart burn, pyrosis, regurgitation, post-prandial fullness, nausea, vomiting, diarrhea and loss of apetite. Blood tests revealed normocytic normochromic anemia (hemoglobin = 11.1 g/dL), and upper gastrointestinal endoscopy was normal. During the next two months there was aggravation of anemia  and progressive asthenia, without evident blood loss or gastrointestinal symptoms, coming then to the Emergency Room. The patient had a past history of chronic hepatitis B, arterial hypertension, gout, dislipidemia and had removed colo-rectal polyps, with an unknown histologic diagnosis, 3 years before. He presented an alcohol consumption of 75 g of ethanol / day, was a former smoker (10 pack years), and was medicated with losartan+hydrochlorothiazide, allopurinol and simvastatin+ezetimibe. In the past 18 months the patient referred a weight loss of 15 kg.Physical observation, apart from descoloration of skin and mucous membranes, was unremarkable, revealing no palpable adenomegaly (including absence of Virchow node), no abdominal masses, and a normal digital rectal examination. Blood tests confirmed a blood loss / iron-deficiency anemia , but were otherwise normal. Chest x-Ray showed cardiomegaly and clear lungs. The upper gastroduodenal endoscopy revealed no lesions. Colonoscopy identified 3 polyps, with no signs of bleeding, in the sigmoid, descendent colon and cecum, corresponding to tubular adenomas with low-grade dysplasia which did not seem to be responsible for the clinical presentation. Since we were unable to proceed with capsule endoscopy due to difficult availability in our hospital, we decided to do an abdominal CT-scan, which showed a diffuse upper jejunal thickening with local, retrocural and peri-aortic adenomegaly, along with distension of D3 suggesting a suboclusive pattern . The daySix months after surgery, follow-up CT scan revealed evidence of local recurrence of the tumour, again with lymph node involvement. The patient was proposed for chemotherapy with six cycles of capecitabine. By the end of the second cycle the patient started vomiting and abdominal pain relieved by metochlopramide and diazepam, allowing the third cycle. Due to recurrence of symptoms and marked weight loss he then repeated the CT scan, once more revealing diffuse upper jejunal thickening with distension of D3, extensive lymph node dissemination (abdomen and thorax), and a single hepatic metastasis at segment IV. A paliative bypass was performed with successful symptomatic relieve and the patient is still alive 14 months after diagnosis.The small bowel corresponds to 75% of the digestive tract lenght and 90% of its mucosal surface area , but surIn 1746, Hamburger made the first case report of duodenal carcinoma, and the first review of malignant small bowel neoplasms was published, in 1876, by Leichtenstein.In 2007, the American Cancer Society reported 5640 new cases of small bowel tumors, with 1090 estimated deaths [Data from Germany suggests an annual incidence of small bowel adenocarcinomas of 2.2-5.7 per million people each year [The mean age of diagnosis of small bowel tumors is 60 years of age, with a slight male preponderance ,2. Adenoras and elevated levels of p53), and epidemiologic features [Small and large bowel adenocarcinomas share similar pathologic (development from adenomatous polyps), genetic , nausea / vomiting 3372.5%), weight loss (38-52.5%), intestinal obstruction 31.3-44%), and gastrointestinal bleeding (23-33%) are the most common symptoms [.3-44%, a.5%, weigCapsule endoscopy has become the most sensible and specific diagnostic method for small bowel disease . It has et al [Surgical resection is the only hope of cure for patients with small bowel adenocarcinoma. Pancreatoduodenectomy is performed in tumors of the first and second portion of the duodenum, while resection followed by anastomosis is the option for tumors located elsewhere. In each case, resection must include regional lymphadenectomy due to the high prevalence of lymph node metastasis at the time of presentation. Curative resection of small bowel tumors is obtained in about 50% . In a reet al , curativet al [et al [et al [Chemotherapy for small bowel tumors is still a matter of debate. There are no randomised controlled trials to evaluate the effectiveness of adjuvant chemotherapy for adenocarcinoma of the small intestine, and so no optimal chemotherapy regimen has yet been established. In 1984, Jigyasu et al  reportedet al  observedl [et al  in 1998,l [et al  used iril [et al .Radiotherapy does not seem to be effective or benefit survival. It is technically difficult to localize the target field due to the mobile nature of the small intestine mesentery.et al [et al [The prognosis of small bowel tumors is poor, with most series reporting a 5-year-survival of 15-35%. After curative surgical resection this rate increases to 40-65% in most studies. In 1999, Howe et al  presentel [et al . In thisOur case intends to highlight the fact that small bowel tumours are rare and frequently present to the Internist as non-specific clinical symptoms. Although capsule endoscopy has become the most sensible and specific diagnostic method for small bowel disease, the use of other diagnostic tools, like CT scan, are still a valid option where capsule is not easily available. Despite surgery and trial of chemotherapy the disease tends to recur conferring a poor prognosis to this rare gastrointestinal tumor.


Sir,Histoplasmosis is an infrequently reported disease in India, and we have come across sporadic case reports from various parts of the country. We would like to share details of a case of oropharyngeal histoplasmosis that we recently came across in our practice, because of its unique presentation and its histopathologic appearance. The patient was a 62-year-old immunocompetent man who presented with a mass in the pharynx. His chief complaints were progressively worsening difficulty in swallowing and alteration of voice of 4 months duration. The patient was a known diabetic. Findings from his baseline investigations, including X-ray chest, were within normal limits. Indirect laryngoscopy revealed presence of a mass in the left posterior pharyngeal wall, extending up to the base of tongue and involving the epiglottis. The lower limit of the mass was not visualized. A clinical diagnosis of a possible malignant lesion in the pharynx was considered. A punch biopsy was performed. Histopathological examination revealed a highly cellular lesion composed of sheets of polygonal cells with eosinophilic granular cytoplasm, separated by small blood vessels . No mitoHistoplasmosis in immunocompetent host usually presents as a mild or clinically insignificant respiratory disease. Mucus membrane lesions are more common in indolent cases and are found in up to two thirds of the chronic cases. Most of 1The available information on the disease in India is largely based upon sporadic case reports and so the endemic zones of histoplasmosis have not been delineated; consequently, the disease should not be suspected only on the basis of the patient's origin.We present this case because histoplasmosis can pose a diagnostic dilemma to both the clinicians and pathologists. Oral histoplasmosis is difficult to diagnose clinically as it can resemble other more common clinical entities such as carcinoma (as in our case), syphilis, sarcoidosis, lymphoma and tuberculosis. A pathologist should suspect or look for histoplasmosis when an isolated population of macrophages is seen in absence of other inflammatory cells or granulomas.


Adrenocortical carcinoma (ACC) is a rare malignancy, especially in children. The overall incidence is approximately 2 cases per million per year. In child Adrenocortical carcinoma (ACC) is an unusual, and a highly malignant childhood tumor with grave prognosis. It accounts for 0.002% of childhood malignancies, with most of the tumors being functional in children. Nonfunct4The tumor has bimodal age distribution, presenting in children under 6 years, and in adults 30-40 years old.[Nonfunctioning adrenal tumors remain a diagnostic challenge in early diagnosis and successful management as there are no early signs and symptoms. In a majority of cases, the tumor has either invaded adjacent organ or already metastasized to distant organ at the time of initial diagnosis. In most of the cases, it is mistaken for neuroblastoma which is the commonest intra-abdominal childhood tumor. Very rarA 5-year-old girl presented with complains of abdominal swelling and low grade fever since few days. No relevant past history of bowel or urinary complains was present. There was also no significant family history of cancer. Physical examination revealed distended abdomen with a palpable lump in the right hypochondrium. Her temperature was mildly raised, blood pressure was 110/72 mm Hg and rest of the vitals were unremarkable.Ultrasonography of abdomen revealed a large heterogenous mass of size approximately 11×10.6×9.26 cm in the right suprarenal region, with calcifications, large necrotic and hemorrhagic areas within and indistinct fat planes from the superior pole of right kidney. Left kidney was not seen in left renal fossa or anywhere else in abdomen, suggesting possible congenital absence. No focal lesion was observed in liver.the claw sign and organ embedded sign were negative, suggestive of extra renal origin [Contrast enhanced CT scan of abdomen confirmed the above findings Figures  and 2. Il origin . Medialll origin . It was l origin , suggestet al. was 21.Ultrasound guided biopsy revealed ACC. The tru-cut biopsy cores showed areas with patternless sheets of cell interrupted by a fine sinusoidal pattern and broad trabeculae. Wide expanses of necrosis were seen.  The indiRoutine laboratory investigations were unremarkable. Although there were no clinically evident signs of virilization or Cushing’s syndrome, plasma cortisol urinary levels of 17-ketosteroids and 17-hydroxycoticosteroid were measured and found to be within normal range.The patient was grouped as stage IV (according to TNM classification)ACC is an extremely rare tumor. In children, 90% of the adrenal tumors are neuroblastomas . Tumors arising from adrenal cortex are rare. Among them, ACC is most common and it accounts for only 6% of adrenal tumors. Adrenal tumors in children can be associated with hemihypertrophy and Beckwith Wiedmann syndrome.ACC are classified as functional and nonfunctional based on the hormonal syndromes they produce. Functional tumors are common and detected earlier than nonfunctioning tumors due to the production of hormones and associated clinical signs as well as symptoms. Nonfunct4Primary adrenocortical tumors are large tumors usually measuring more than 5 cm at presentation. The larger the tumor, more is the chance of it being malignant. Because they are large, the organ of origin often is difficult to determine. CT scan plays an important role in characterizing the organ of origin and in defining the extent of the primary as well as assessing the presence of metastatic disease. The common metastatic sites include lung and liver, with bone and bone marrow being less common. Neuroblaet al., stage IV ACC is not amenable to surgery and mitotane remains the first-line therapy.[et al. reported that debulking surgery along with mitotane in stage IV patients prolongs survival.[ACC tends to be highly malignant and locally invasive, and potential curative treatment is complete surgical removal. The role of tumor debulking in metastatic ACCs is controversial. According to Allolio  therapy. Conversesurvival.The reported median survival of stage IV ACC is less than 12 months. However, there are case reports showing longer survival in patients with stage IV disease, with surgery and chemotherapy. Various 12et al. of 253 patients, the overall survival rate was 38% and the 5-year survival rates were as follows: for stage I 60%; stage II 58%; stage III 24% and stage IV 0%. The overall 5-year survival in different series ranged between 16 and 38%. Median survival for metastatic disease (stage IV) at the time of diagnosis is still consistently less than 12 months.[Prognosis depends largely on tumor stage. In a study conducted by Icard P 2 months.10 The av2 months. In our cIn summary, nonfunctioning ACC is a very rare childhood tumor. Its early detection and appropriate treatment remains a continuing challenge. Although neuroblastoma is the commonest intra-abdominal malignant childhood tumor, in cases with a large nonfunctioning adrenal lesion with pulmonary metastasis and no definite evidence of bone metastasis, a nonfunctioning ACC, though very rare in children, should be kept in differential diagnosis.


Mesenchymal tumors represent a small number of bladder cancer cases. Leiomyosarcoma is the most common histology with over 100 cases reported in the whole literature. This tumor is been historically considered as highly aggressive and showing a poor prognosis. Despite very low survival rates showed in older reports, some authors indicate that some patients could have a better outcome. We report a review of the literature and a case of high-grade LMS of the bladder in a 68 years old woman. Diagnosis was delayed and disease was locally advanced. Symptoms and imaging of our case first oriented to a gynecologic condition with an adnexal or uterine origin of the mass, and, a genitourinary origin could be unveiled only intra-operatively. Non-epithelial tumors of the urinary bladder account for less than 5% of the overall bladder malignancies, with leiomyosarcoma being the 0.1% of bladder cancer . There iCases presented in literature had mostly gross urinary symptoms, more often with an earlier onset. This may, eventually, lead to earlier diagnosis, allowing a safer therapeutic approach. The case we report shows an unusual presentation, differing from the symptoms reported in literature, resembling mostly a pelvic mass. The patient underwent, in the early diagnostic steps, a gynecologic evaluation that addressed to a final multidisciplinary surgical approach to the disease. The high-grade and stage of the tumor, in association to the fatal outcome of the case, are expression of the aggressive behavior of the tumor, showing mostly a very poor prognosis.We report a case of a 68 years old woman presenting to the emergency department with macrohematuria and dysuria. She experienced fever several times, starting two months before medical examination. Fever was treated with NSAID. A history of recent pelvic pain was also reported. Her clinical status was good and no other symptoms or concurrent illnesses were present at the time of hospitalization.She was admitted to Gynecologic service for further evaluation.The patient was virgin and in post-menopausal status. Clinical history was unremarkable; negative either for previous medical or surgical procedures. Family history was positive for cancer with one male sibling deceased from a pancreatic malignancy; the remainder two siblings had no positive history for malignancies as well as parents and closest relatives. Social history was negative for use or addiction to drugs, alcohol and exposure to potential risk factors.A digital rectal examination had to be performed in place of the gynecologic bimanual examination. A gross, firm mass located in the pelvis was found at physical examination.Pelvic US, abdominal CT scans, descending pielography, cystoscopy and chest RX were performed to both assess local disease and evaluate the presence of local and distant metastases.Computed tomography imaging confirmed the presence of a complex mass in the pelvis (13 × 14 cm) showing irregular contours and small areas of calcifications. Uterus and adnexa could not be clearly recognized, showing as being part of the mass. Iliac vessels and bladder were displaced. Imaging technique reports stated that no vessels or bladder invasion was present. A hypoplasia of the left kidney was also reported. No other metastasis was reported in the abdomino-pelvic cavity.Pielography showed a delay in the opacification of the renal pelvis associated to a dilation of the right ureter that was compressed and displaced in its pelvic course till entering the bladder. No opacification of the contra lateral kidney and excretory ways could be obtained.Cystoscopy showed, eventually, a bleeding lesion onto the bladder wall that raised suspicion of neoplastic invasion. The lesion appeared more likely as a loss of continuity of the urothelial mucosa; an ulcerating mass was visualized and eventually described on final report.Chest RX and bone scan were negative for distant metastases.A multidisciplinary board, including a urologist, a radiologist, a radiation oncologist, an oncologist and a gynecologist, evaluated the collected data. It was eventually decided to surgically extirpate the tumor. A transurethral biopsy of the vesical wall lesion was not considered since major suspicion was addressing to uterine or adnexal neoplasms and the high stage was reasonably an indication to undergo definitive surgery.Surgery was eventually performed after pre-operative routine and evaluation.A longitudinal abdominal incision was preferred to access peritoneal cavity. A bulky mass was found in the pelvis. Uterus and adnexa appeared macroscopically free from neoplastic invasion. The mass showed a cleavage with recto-sigmoid tract, iliac vessels and pubic bone, whether uterus, adnexa, right ureter and left obturator nerve could not be separated from the tumor. A histological intra-operative examination revealed a malignant, spindled cell neoplasm. Surgery had to include a wide mass excision, in order to obtain free margins and control of local disease. Final treatment choice was a pelvic exenteration. This decision was made since local disease was advanced and it was set to obtain specimen's margins disease-free as a primary target.An en-bloc resection of the mass, together with uterus, adnexa, bladder, distal part of right ureter and left obturator nerve was performed (anterior pelvic exenterantion).A cutaneous ureterostomy was placed in the site of ureteral dissection. The decision to perform this procedure in place of a standard urinary diversion, as a ileal conduit or Miami pouch, was made on the evidence of poor prognosis that appeared from surgical exploration and intra-operative histology that was addressing to a malignant stromal neoplasm. It seemed to be reasonable to lower intra- and post-operative morbidity whereas the potential overall survival of the patient appeared to be prospectively low.A final histological diagnosis of high-grade leiomyosarcoma, Fig. , G3, FN. The spePost-operative course was free from complications and the patient could be discharged from the hospital fourteen days after surgery.The patient underwent chemotherapy one month after surgery, using doxorubicin as a single agent in an adjuvant setting. She died after the first cycle from a distant recurrence to the left lung.Sarcoma is the most frequent mesenchymal malignancy of the bladder with leiomyosarcoma as most common histology, according to two retrospective reviews of mesenchymal genitourinary tumors ,4. ConsiInitial reports for this tumor showed a very aggressive disease. In a series by Mackenzie et al. published in 1968, only 11 patients were alive at 3 years from surgery . FurtherHowever, the best prognostic factor seems to be the presence of free margins. In our case, we achieved disease-free margins; still, they did not determine a better outcome. In addition to margin status, local invasiveness and size, as well as tumor grade, seem to play an important role in determining the final outcome. In our case, as stated above, LVI was unavailable.According to one of the greatest series available to date, overall local recurrence of bladder leiomyosarcomas is about 16%, with most recurrences occurring in the pelvis. Overall recurrence of distant metastases is about 53%, with the most common sites of metastases being the lungs, liver, bone, and brain .Because of very low incidence, there is no universal consensus on the treatment of patients affected. Minimally invasive approaches, such as transurethral resection or laser fulguration, in addition to CTX or RT adjuvant settings, have been used for patients with small lesions. Long-term survival rates did not differ significantly to cases undergone a more radical surgery. A sole resection of the mass, even when free margins were achieved, as in case of partial cystectomy, should be considered a palliative treatment -14. MainA multimodal treatment with CTX settings targeting mesenchymal cancers  should be mandatory in the event of metastatic disease, . With poSarcomas should be considered as a possible histology at differential diagnosis, even if they are no frequent compared to other tumors. Unfortunately, obtaining a pre-operative histological diagnosis does not improve the prognosis. Rare tumors represent a great challenge for physicians. They require experienced teams and well-equipped centers for cancer cure. Bladder sarcomas, as other genito-urinary sarcomas, require close cooperation between urologist and gynecologic oncologist, as well as medical and radiation oncologist. A pathologist expert in mesenchymal tumors is mandatory.Nowadays, no statistically relevant evidences on therapeutic behavior can be found yet in the literature. Therefore, treatment should be tailored case-by-case, preferring a multimodal and/or multidisciplinary approach to the disease. A decision-making team made by physicians experienced in managing soft tissues sarcomas should be also strictly required.The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.ER conceived of the study, and participated in its design and coordination and drafted the manuscript. PM collected clinical data. MS participated in collecting data and read and corrected the manuscript. MM participated in design of the study and revisions, gave intellectual input and corrected the manuscript. All authors read and approved the final manuscript.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 case of a 64-year-old male patient who presented with a bulky osseous tumor metastasis from renal cell carcinoma is reported. The metastatic lesion extended from the acetabulum of the left iliac bone into the iliosacral joint.Treatment plan included selective arterial embolization followed by transdermal radiofrequency ablation, followed by consolidation irradiation. This sequence resulted in considerable necrosis of the bulk of the tumor and creation of a large tissue deficit, which healed over the period of several months despite anti-angiogenetic systematic treatment. The patient remains in good state of health, 20 months after the procedure. This case illustrates the usefulness of the embolization-ablation sequence in controlling large osseus metastasis. Renal cancer is a relatively uncommon malignancy which is however diagnosed with increasing frequency . It presAlternative approaches in the management of renal cell carcinoma include local embolization -5 or ablThe combination of embolization and radiofrequency ablation has not been adequately studied in the metastatic setting . It offeA 64-year-old Greek male patient underwent a radical nephrectomy for a stage 2, grade 3, renal cell carcinoma in September 2001. Five years later, he presented with left hip pain and he was eventually diagnosed with biopsy proven relapsed disease. Imaging with CT scans and bone scan revealed a prominent bulky metastatic lesion extending from the acetabulum of the left iliac bone into the iliosacral joint, a metastatic lesion of the second left rib, as well as several metastatic pulmonary nodules ,b. He waAfter a year of treatment, in October 2007, a necrotic ulceration appeared on the left flank area, proven by CT to be an extension of the increasing metastatic left iliac focus, which measured 8 cm in maximum diameter, associated with increasing pain. The lesion was considered too large to be controlled with irradiation. The patient underwent an arterial embolization of the metastasis using a femoral artery approach . One weeBulky bony metastasis of the axial skeleton of a relative radioresistant tumor such as renal cell carcinoma is usually a problematic medical situation associated with significant morbidity. In the case of our patient the comorbidity was exacerbated by the presence of skin infiltration and ulceration caused by direct contiguous tumor invasion. A conventional approach would have been to treat with external beam irradiation and analgesics. Such an approach would lead to local failure soon, given the tumor bulk. The sequence of embolization followed by radiofrequency ablation caused extensive necrosis of the tumor bulk and creation of a large tissue deficit, which was at first considered of ominous nature, but as it appears heralded a favourable outcome. The significant tumor reduction thus achieved may have resulted in the increased efficacy of the subsequent therapies provided, namely external beam irradiation and further antiangiogenetic therapy. Of note, excellent wound healing with secondary intent proceeded over the subsequent year, without complications and in spite of antiangiogenetic therapy. It is conceivable however that the administration of subsequent therapy may have delayed the healing process, although without apparent untoward effects.Therapeutic embolization of osseous metastases has been previously reported in small number of patients with a variety of tumors ,8. This In conclusion, our case illustrates the usefulness of the sequence of localized treatment for the management of bulky osseous metastasis. Such a specialized treatment approach should be entertained in selected patients as it can yield long lasting favourable outcomes.


Purpose.  Epithelioid leiomyoblastomas comprise the majority of gastric sarcomas            and are uncommon in other parts of the gastrointestinal tract. Diagnosis of this lesion            by fine-needle aspiration cytology has been occasionally described in the literature.            Two additional cases are herein reported.                    Subjects .  A 66-year old male with an omental mass and a 47-year old male with a            perihepatic tumor.                    Results and Discussion. Cytologic materials in both cases showed predominantly            round or epithelioid cells, along with polygonal to spindle cells, occuring singly and in            clusters, with oval to spindle-shaped nuclei.The nuclei were monotonous, usually banal,            and centrally-located with only focal suggestion of pleomorphism and rare mitosis.            Eosinophilic cytoplasm was noted in most of the cells, some demonstrating vacuolation.            Electron microscopy supported a primitive smooth cell derivation of the neoplastic cells.                    Conclusions.  The cytomorphology of the tumors of the two cases reported here is not            adequately known. More cases need to be collected and studied.


Surgical treatment is the usual therapy for patients with polycystic liver disease and with severe symptoms, yet the results of surgery are often disappointing and the optimal surgical approach is uncertain.We present the case of a 41-year-old Greek woman who underwent combined left hepatectomy with fenestration for symptomatic polycystic liver disease using ultrasound scalpel, fibrin glue and closed suction drain to prevent bile leakage, haemorrhage and ascites. Liver resection using the ultrasound scissors allowed quick parenchyma dissection under haemostatic conditions with safe coagulation of small vessels and bile ducts. Moreover, the ultrasound scalpel was applied to the cyst cavities exposed on the peritoneum to ablate the fluid-producing epithelial cyst lining. We also covered the cut cystic cavities exposed to the peritoneum surface of the liver with fibrin glue. Instead of allowing the opened cysts to drain into the abdominal cavity, we used two wide bore closed suction fluted drains. We did not observe excessive fluid loss through the drainage after the second postoperative day. The drain tubes were removed on the third postoperative day.In our patient, effective treatment of ascites and prevention of bile leakage and bleeding indicate that this new approach is promising and may become a useful surgical technique for polycystic liver disease. Polycystic liver disease (PLD) is a common manifestation of polycystic kidney disease and is associated with an autosomal dominant inheritance. Patients are usually asymptomatic . SymptomWe present the case of a patient who underwent combined left hepatectomy with fenestration for symptomatic PLD using the harmonic scalpel, fibrin glue and closed suction drain to prevent bile leakage and ascites.A 41-year-old Greek woman presented with chronic and unrelenting right upper quadrant pain, epigastric fullness, early satiety, nausea, vomiting and dysphagia. On physical examination, hepatomegaly and tenderness in the right upper quadrant were found. Abdominal ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) revealed multiple liver cysts, particularly in the left hepatic lobe, with characteristics similar to simple hepatic cysts. Multiple cysts were also found in the kidneys and the anterior surface of the pancreas  were located at the right hepatic lobe Figure . There wAfter completion of the resection, the tourniquet was opened and the remaining cut surface carefully inspected for residual bleeding or nonoccluded bile ducts. The cut surface was plain and brownish; biliary leaks or persistent bleeding were easily detected and sutured with 4-0 polypropylene. The ultrasound scalpel was applied to the cyst cavities exposed on the peritoneum in order to attempt ablation of the fluid-producing epithelial cyst lining. To avoid bile leakage and haemorrhage, fibrin glue was spread over the raw surface of the liver. Cysts in the pancreas and kidneys were not treated. Two wide-bore closed suction fluted drains (30 F) were brought out through a separate stab wound; one placed in the subhepatic space and one in the right subdiaphragmatic space. Postoperatively, the patient remained well and without complications. The drain tubes were removed on the third postoperative day, when the drainage had decreased to less than 30 mL in 8 hours. Symptomatic relief and reduction in abdominal girth were achieved. Histologic examination showed von Meyenburg's complexes. The patient was followed up at clinic - special data included hepatic and renal function, symptomatic relief, the patient's working capacity and CT scans. The follow-up showed post-resection hypertrophy of the spared liver and lack of clinically significant cyst progression. Four years after the procedure, the patient had an improved quality of life and functional status without deterioration in her hepatic or renal function.With the widespread use of sensitive imaging techniques, the frequency of non-parasitic hepatic cysts is reported more often. Adult polycystic disease is the most common cystic disease. Liver cysts in patients with polycystic kidney disease are generally asymptomatic, but in a few patients, hepatomegaly from numerous large cysts may cause symptoms ,4.de novo after treatment . Th. Th10]. Handling of the instrument, cutting and coagulation quality were satisfactory and safe. To achieve a better and more effective coagulating effect, the portal structures were occluded by a tourniquet which did not last longer than 30 minutes, together with lowering of the central venous pressure during resection. The liver resection using the ultrasound scissors allowed quick parenchyma dissection under haemostatic conditions with safe coagulation of small vessels and bile ducts of up to 2 to 3 mm in diameter. Larger vessels and biliary ducts were divided with clamps and sutured with 4-0 polypropylene. The major advantage of the ultrasound scalpel was the modest trauma that it produced and the controlled dissection of the tissue. Especially in the periphery, the UltraCision was an ideal dissection instrument: with the absence of large vessels and bile ducts, nearly all of the parenchyma was easily divided without causing bleeding, bile leakage or trauma. Moreover, use of ultrasound scissors on the cyst cavities exposed on the peritoneum was attempted to facilitate ablation of the secretory epithelium and reduction of postoperative continual peritoneal fluid losses. However, we also covered the cut cystic cavities exposed to the peritoneum surface of the liver with fibrin glue ,12. FibrAscites is the commonest complication specific to surgery for PLD, occurring in all patients undergoing resection ,2. We atSurgical management of patients with PLD remains a challenging issue for physicians. The aim of the present study was to investigate the ability of the UltraCision instrument, fibrin glue and closed suction drainage in hepatic resection combined with fenestration for PLD. This method appears to be an advantageous new technique. This case report is not sufficient to draw any final conclusions. Therefore, the benefits of this surgical approach should be further evaluated. However, our initial experience is promising, and we believe that it may become a valuable means in surgery for PLD.CT: computed tomography; MRCP: magnetic resonance cholangiopancreatography; MRI: magnetic resonance imaging; PLD: polycystic liver disease; SGOT: serum glutamic oxaloacetic transaminase; SGPT: serum glutamic pyruvic transaminase; US: ultrasound.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.CK, CE and EF performed the operation and together with GA and SL contributed to the conception and design of the manuscript. JP and KP analyzed and interpreted the patient regarding the polycystic disease. GA and SL were major contributors in writing the manuscript. All authors read and approved the final manuscript.


Oral melanoacanthoma is a rare, benign pigmented lesion characterized clinically by the sudden appearance and rapid growth of a macular brown-black lesion and histologically by acanthosis of the superficial epithelium and proliferation of dendritic melanocytes.We present a case report of oral melanoacanthoma in a 24-year-old Asian Indian man. He presented with an intra-oral brown macular lesion on the left buccal mucosa with a duration of one and a half months. Microscopic examination revealed acanthosis of stratified squamous surface epithelium and dendritic melanocytes diffusely distributed in the epithelium; the Masson-Fontana silver impregnation technique was used to demonstrate the dendritic melanocytes. Based on the history, clinical features and histological presentation, the lesion was diagnosed as melanoacanthoma.This is the first reported instance of oral melanoacanthoma in the Indian sub-continent. This report details the course of the lesion from diagnosis to its resolution. Melanoacanthoma must be differentiated from other intra-oral pigmented lesions and biopsy may be required to rule out melanoma. Melanoacanthoma of the oral mucosa is a rare condition indicative of a reactive process . Oral meA 24-year-old graduate dental student presented with a complaint of intra-oral pigmentation of the left buccal mucosa with duration of one and a half months. The patient had initially noted a small round area of pigmentation of about 5 mm in size which, to his concern, had rapidly increased to the present size Figure . He did The brownish-black macular lesion on the left buccal mucosa was well demarcated from the surrounding mucosa with regular, well-defined borders. The lesion extended anteriorly from the region of the mandibular first molar (36) to the mandibular left canine region. It measured 25 mm antero-posteriorly and had a maximum width of 16 mm supero-inferiorly. The lesion was not tender, did not blanch under pressure and was not fixed to the underlying mucosa.Following incisional biopsy, the specimen was fixed in 10% neutral buffered formalin, routinely processed and paraffin embedded. Histopathological examination of the lesion revealed a stratified squamous surface epithelium exhibiting acanthosis, spongiosis, melanin pigmentation, inflammatory cell exocytosis and numerous dendritic melanocytes distributed diffusely in the suprabasal and spinous layers. A chronic inflammatory cell infiltrate was present in the subjacent connective tissue. The dendritic melanocytes were also demonstrated by Masson-Fontana silver impregnation stain Figure . Based oThe lesion characteristically appeared to regress following the biopsy procedure. A regular follow-up of the patient was carried out to observe the progress of the lesion Figure .The term melanoacanthoma refers to a lesion exhibiting a proliferation of dendritic melanocytes throughout the surface epithelium. Cutaneous melanoacanthoma is also known as pigmented seborrheic keratosis .Oral melanoacanthoma is a benign, reactive process and is unrelated to cutaneous melanoacanthoma. The reported age of presentation ranges from 9 to 77 years, with a mean age of 29 years ,4,12. ThIn our patient, the etiology of the lesion may be attributed to the incident of trauma during the restorative procedure. It may be safely assumed that GIC did not contribute to the cause of the lesion since the patient has multiple restorations with the same material and the adjacent sites did not exhibit any lesion.To the best of our knowledge, this is the first case of oral melanoacanthoma in the Indian subcontinent and the second case of melanoacanthoma reported in an Asian Indian. In the present instance, a biopsy was performed to alleviate the patient's anxiety and as reported, the lesion regressed following biopsy. Thus, melanoacanthoma must be considered in the differential diagnosis of rapidly progressing pigmented lesions of the oral cavity and requires a histopathological diagnosis to rule out melanoma.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.Both authors have made substantial contribution with individual input as follows:KR was responsible for identification, diagnosis of the case, drafting of manuscript and final correction of the version to be published. VL was involved in follow up of the patient, literature review and revising and submission of manuscript. The final version of the manuscript was approved by both authors


Clear cell neoplasms of the jaw are very infrequent and a review of the literature reports only isolated cases of metastatic renal clear cell carcinoma of the jaw.A 68-year-old man presented with an osteolytic lesion of the left hemimandible. The first diagnostic hypothesis was a third molar follicular cyst. Surgical treatment consisted of enucleating the lesion preserving the alveolar nerve and extracting of the retained tooth. Unexpectedly, the lesion presented as a solid.The authors report a case of a clear cell neoplasm involving the jaw in which histopathological exam presented an indeterminate histology. The histological characteristics of this tumor make it unique in the international literature. Clear cell neoplasms of the jaw are very rare and a review of the literature reports only isolated cases of metastatic renal clear cell carcinoma of the jaw -3. In thA 68-year-old man presented to our center for treatment of an osteolytic lesion of the left hemimandible. The patient was completely asymptomatic and became aware of the lesion following a panoramic radiography. The lesion had spread into the linguo-vestibular thickness of the left hemimandible from the second premolar to the impacted third molar. Upon panoramic Fig.  and compThe mandibular canal appeared to be eroded by the lesion, such that the alveolar nerve was circumscribed, even though the patient did not refer paresthesia. No intraoral or extraoral swelling was present.The first diagnostic hypothesis was a third molar follicular cyst. A biopsy was not taken. Surgical treatment consisted of enucleating the lesion preserving the alveolar nerve and extracting the wisdom tooth. Unexpectedly, the lesion presented as a solid, sheet-like mass. At 6 months after resection, all teeth involved maintained vitality.The histological specimen was sent to the Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, USA for additional consultation. The lesion was classified as a clear cell tumor of undefined origin. It appeared as a grayish-white soft mass with a diameter of 3.8 cm. The tumor was composed of a monotonous, sheet-like proliferation of uniform cells having a clear cytoplasm and small nuclei; neither atypia nor pleomorphism could be detected and the mitotic index was within normal limits (2 mitoses/50 HPF).The possibility of a rare intraosseous form of meningioma  was consThe possibility of a metastatic renal lesion was considered, but both total-body CT scan and magnetic resonance were negative. A specimen was sent to an international specialist who also could not identify the origin of this neoplasm, and concluded that it should be considered an 'unclassified clear cell neoplasm'. The clinical course was to be carefully followed and a wait-and-see policy was adopted since no overt morphologic signs of malignancy were present.Clear cell neoplasms of the jaw are very rare. In a review of the literature, only isolated cases of metastatic renal clear cell carcinoma of the jaw have been described -3.In the maxillo-facial area, clear cell odontogenic tumors have also been documented that are potentially aggressive and capable of multiple local recurrences and both locoregional and distant metastases. Management of this type of tumor should include wide en bloc resection and long-term follow-up . MoreoveIn this patient, immunohistopathological exam presented an ambiguous pattern that did not permit a definitive diagnosis. Long-term follow-up will include clinical examination at 6-month intervals. In the follow-up, we decided to use a wait-and-see policy.As this is an unidentifiable kind of neoplasm, it might represent a new type of lesion. We suggest considering this lesion as malignant, as long as it is impossible to find evidence of its benignity. That is why we highly recommend a follow-up with physical exam every 3 months in the first year, every 2–4 months during the second year, every 4–6 months from the third to the fifth year and every 6–12 months from the fifth year. The radiological exams should consist of CT of the maxillo-facial complex and neck with and without contrast agent every 6 months during the first year, alternating with echography of the neck every 3 months for the first year. From the third year, one echography every 6 months and CT every year . We suggThe authors declare that they have no competing interests.All of the authors were involved in examination of the patient as well as in writing and reviewing the manuscript.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.


Report of a gastrointestinal stromal tumor in an 11-year-old girl who presented with a large lump in the upper abdomen. After complete surgical excision and histopathology, postoperative immunotherapy with imatinib led to an excellent outcome and a tumor-free survival of 3 years. Gastrointestinal stromal tumor (GIST) is an entity in the group of mesenchymal tumors. For many2 a day. The dose was then reduced to 200 mg/m2 as the child had jaundice, with a serum bilirubin of 2.2 mg/dl after the first month. This was continued for 6 months. The child was followed with ultrasonography scans at regular intervals and, at the 3-year follow-up, is well and tumor-free.An 11-year-old girl presented with complaints of low-grade intermittent fever for 6 months and a gradually increasing lump in the left upper abdomen of 15 days duration. On examination, a 10 cm × 5 cm nontender, firm, freely mobile, mass with ill-defined margins was palpable in the left hypochondrium. The investigations revealed anemia and raised Lactate Dehydrogenase. Alfa-fetoprotein and a chest radiograph were normal. A computed tomogram (CT) scan showed a large lobulated heterogeneously enhancing mass of size 11 cm × 6 cm × 7 cm, with central area of necrosis and calcification in left hypochondrium and lumbar region . The masGISTs are a type of mesenchymal tumors .2] With With2] WEven with advanced GIST where complete resection was not possible and/or extensive resection of the structures/organs involved, surgical excision used to be the main treatment modality. With the112, which was reduced to 200 mg/m2 in view of hepatic impairment. There is no consensus on the duration of treatment with imatinib and further research and study of a large number of cases will provide future guidelines.The enthusiasm of the use of this drug led to its utilization as neo-adjuvant therapy in adults. An Radiation Therapy Oncology Group trial in adults with large or marginally resectable tumors14 implemPediatric GISTs are still classified, risk stratified and treated according to the adult GIST protocols. As more and more patients will be managed and reported, the understanding of pediatric GISTs (which has a different behavior from its adult counterpart) will increase and thus expand the scope of multimodality treatment. Surgical excision is and shall continue to play a cardinal role in the management protocol of this uncommon pediatric tumor.