Background: Musculoskeletal tumors are rare, but their descriptive data in any region are important to reduce mortality rate and improve their management. Materials and Methods: Retrospectively, 426 pathologic reports from 1997 to 2008 were reviewed in Shiraz University Orthopedic Hospitals which are the main referral centers for musculoskeletal tumors in south of Iran. We collected and analyzed data on age, gender, anatomical site, and histopathologic types of musculoskeletal tumors. Results: Of the 426 cases, 60.1% were men and 39.9% were women. The commonest malignant bone tumors were osteosarcoma (89; 50.6%), metastasis (30; 17.0%), Ewing's sarcoma (28; 15.9%), and chondrosarcoma (14; 8.0%). The most frequent benign bone tumors were osteochondroma (136; 63.9%), enchondroma (23; 10.8%), giant cell tumor (21; 9.9%), and osteoid osteoma (14; 6.6%). The femur was the most commonly involved site in musculoskeletal tumors. It was followed by the tibia in benign tumors and the humerus in malignant ones. Metastasis (28; 32.6%), soft tissue tumors (18; 20.9%), osteochondroma (10; 11.6%), and osteosarcoma (9; 10.5%) were the most diagnosed bone lesions in patients older than 40. Conclusion: There are no significant changes in epidemiology of musculoskeletal tumors in Shiraz, south of Iran, in comparison with other parts of the world.

Context: Computerized tomography (CT) guided fine needle aspiration cytology (FNAC) of lung lesions has rapidly emerged as a less-invasive, cheap, rapid and fairly accurate diagnostic aid in lung lesions. Aims: The purpose of this present study was to evaluate the effectiveness of CT-guided FNAC in the diagnosis of pulmonary mass lesions (both benign and malignant) and to determine the complication rate of this procedure. Settings and Design: We conducted an institution-based, prospective study on 127 patients who presented with pulmonary mass lesions. Materials and Methods: After proper consent was obtained, CT-guided transthoracic fine needle aspiration was done and their diagnoses were confirmed by appropriate methods. The results were analyzed statistically. Results: Out of 127 cases selected for the study, 59.8% were males while the rest were females. Cough was the most common symptom present in 71.2% cases, followed by weight loss (62.4%). 21.2% cases were cytologically benign. Adenocarcinoma (54.2%) was the commonest malignant tumor. FNAC provided at least 96% sensitivity and 100% specificity in diagnosing lung tumors. Among the benign lesions, specific diagnoses were obtained in 48.1% cases. Thus, altogether a specific diagnosis was obtained in 109 of 127 cases, i.e. 85.8%. No major complication was noted. Conclusions: CT-guided FNAC is an extremely valuable and fairly accurate diagnostic aid of intrathoracic mass lesions, with a reasonable rate of complication.

Background : The uniqueness of adolescent and young adult (AYA) non-Hodgkin lymphomas (NHL) with respect to biology and treatment have largely remained unanswered due to marked heterogeneity in treatment, paucity of prospective, or retrospective studies and poor representation of AYA in clinical trials. This audit attempts to put forward the clinicopathological attributes and treatment outcomes of AYA NHL treated with both pediatric and adult protocols from a single centre in a developing country. Patients and Methods: Hospital records of all consecutive NHL patients registered in lymphoma clinic from January 2007 to May 2010 were reviewed for information on demography, clinical features, histology subtype, staging, treatment regimen, response rates, toxicities, and follow up. Two-year progression-free (PFS) and overall survival (OS) were calculated with Kaplan-Meier method. Results: AYA NHL constituted 4% of all lymphomas. Diffuse large B-cell (DLBL) was the most frequent subtype. Following were the 2-year PFS and OS - DLBL 64%, 76.9%, Burkitt's lymphoma: 56%, 56%, lymphoblastic lymphoma: 33.2%, 44%. Our results did not show any improvement in outcome of DLBL with the use of Burkitt's lymphoma like regimen. Conclusions: This study highlights some of the key features of AYA NHL occurring in developing world.

Inflammatory pseudotumor (IPT) is a rare lesion of unclear etiology reported in various organs. Although mostly benign, these tumors may pose a therapeutic challenge in cases of recurrence. We report the case of a young male who presented with a clinical and radiological picture suggestive of a malignancy in the thorax and upon evaluation was noted to have IPT of the lung. Complete surgical resection was done with no evidence of tumor recurrence. We review the literature and discern the epidemiological, clinical, pathophysiological and management aspects of IPTs.

Ovarian involvement as primary or secondary lymphomatous process is extremely uncommon. In most cases, the diagnosis is usually not suspected initially and is confirmed only after detailed histopathological evaluation. We report a patient with primary ovarian diffuse large B-cell lymphoma (DLBCL) and associated auto-immune hemolytic anemia (AIHA) who achieved complete remission after treatment with Rituximab-cyclophosphamide-doxorubicin-vincristine and prednisolone (R-CHOP) chemotherapy. This patient was a 50 year old female, who presented with fever, abdominal pain, vomiting, weight loss and anemia. Computed tomography scan of the abdomen and pelvis revealed a large left ovarian mass with bilateral hydronephrosis. We performed exploratory laparotomy and partial resection of the mass was done due to the adhesions. Histopathology confirmed the diagnosis of DLBCL. After six R-CHOP chemotherapy cycles, patient achieved complete response with correction of anemia. To our knowledge, this may be the first case report till date of primary ovarian DLBCL with AIHA treated with R-CHOP chemotherapy who achieved complete remission in terms of primary disease as well as hemolytic anemia.

Parieto-occipital region is the most commonly involved site in posterior reversible encephalopathy syndrome (PRES). Cerebellar involvement has been reported with the predominant involvement of posterior cerebral regions, but isolated cerebellar involvement in PRES has been reported only once in English literature. We report here a 7-year-old boy with acute lymphoblastic leukemia who had PRES with isolated cerebellar involvement during induction chemotherapy. He presented with sudden onset headache, vomiting and hypertension followed by seizures, unconsciousness, and involuntary movements. Computed tomography scan revealed bilateral cerebellar hypodensities. He improved within few hours and reversibility of the lesions was documented on magnetic resonance imaging after 2 weeks. Awareness of atypical patterns in distribution of imaging abnormalities is important to recognize PRES more accurately and to avoid unnecessary diagnostic procedures and treatment.

Antenatal detection of Wilms' tumor is rare and only nine cases have been reported till now in the English literature. We report a case of a 7-month-old child with prenatally detected renal mass managed by nephroureterectomy associated with intraoperative spill. The child developed recurrence while on DD4A chemotherapy. Salvage chemotherapy was initiated but there was no response after two cycles and the child expired due to tumor rupture.

Ataxia telangiectasia (AT) is a rare multisystem, neurodegenerative genetic disorder. Due to its wide clinical heterogeneity, it often leads physicians to an incorrect or missed diagnosis, and insight into this rare disease is important. Here is a case report of two cousins from the same family who showed salient characteristic features of AT along with the incidental finding of co-inheritance of hemoglobin E trait. Though both of them were from the same family, they showed differences in the type of humoral immune deficiencies, laboratory findings, and their susceptibility to develop different types of malignancies. One of them developed T cell acute lymphoblastic leukemia, isolated immunoglobulin A deficiency, and normal serum carcinoembryonic antigen (CEA) and carbohydrate antigen 19.9 (CA 19.9) levels. He expired at the age of nine years. The other, though a year older, has still got normal blood counts, normal immunoglobulin levels, and elevated serum CEA and CA 19.9 levels. Thus, insight into this disease is very important as AT patients require protection from unnecessary exposure to ionizing radiation to prevent malignancies. Diagnosis of AT allows appropriate genetic counseling for the family.

Primary pulmonary neoplasms are uncommon in children and represent a wide spectrum of pathology from benign to malignant. They are quite different in their histopathologic distribution from that of adults. This study was done to analyze the histopathologic spectrum of primary lung tumors in children. All the resected specimens of lung in children over a period of 5 years were studied and only the cases of primary pulmonary neoplasms were further analyzed. There were two cases of inflammatory myofibroblastic tumor. The patients were boys aged 10 and 12 years, respectively. One case of bronchial carcinoid was diagnosed in a boy of 12 years. There were one case each of pleuropulmonary blastoma (PPB) in a girl of 9 years and pulmonary blastoma (PB) in a girl of 2 years of age. In our study, the two cases of inflammatory myofibroblastic tumor had excellent prognosis. However, the cases of PPB and PB were both associated with poor clinical outcome, whereas the case of bronchial carcinoid has been doing well on follow-up.

Peripheral T-cell lymphoma (PTCL) represents approximately 12% of lymphoid neoplasms. They are even rarer in children and represent only 1% of Non-Hodgkin's lymphoma in this age group. We report a case of PTCL in a 1-year-old female child for its rarity.

Splenic angiosarcoma is a rare neoplasm that often remains asymptomatic till the onset of massive intra abdominal bleeding that require emergency splenectomy. We report here a case of 60 year old male who presented with on and off fever and a splenomegaly was found on clinical examination suggesting a lymphoproliferative disorder. A contrast enhanced computerized tomography suggested splenic trauma with intracapsular bleed. A splenectomy was carried out that revealed splenic angiosarcoma. Splenomegaly and fever is a very rare presentation of angiosarcoma. Though rare it should be kept in mind when investigating pyrexia of unknown origin.

We present a patient with metastatic leiomyosarcoma of the prostate who achieved complete response with chemotherapy. A 70 years old male patient presented with urinary tract symptoms and a prostatic mass. After having been treated as carcinoma prostate, he presented with progressive pelvic mass, lung and bone metastases to our hospital. Repeat biopsy was suggestive of prostatic leiomyosarcoma. He was treated with chemotherapy with the ifosfamide-epirubicin regimen and achieved complete remission on positron emission tomography/computerized tomography after three cycles. He was given a total of six cycles of chemotherapy and continues to be disease-free after 8 months. Although adenocarcinomas are the commonest prostatic malignancies in the elderly, a careful evaluation is essential to rule out alternative diagnoses. Our case report illustrates the potential role of combination chemotherapy in the management of advanced leiomyosarcoma of the prostate. The literature on this entity is reviewed.

Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy in the presence of a rapidly enlarging vascular tumor. It usually presents in early infancy, but onset in early neonatal period, facial hemangioma, and vincristine use in neonates has rarely been reported. We, hereby, present a 6-day-old male child presenting with facial hemangioma and intracranial hemorrhage, and KMP responding well to steroids and vincristine. Pathophysiology of disease and various treatment options have been discussed.

Malignant ectomesenchymomas are rare tumors. This tumor affects predominantly young children. Most common site is head and neck. A multi modality approach should be appropriate for this soft-tissue tumor. We present a 43 year old female with malignant ectomesenchymoma of the nasal cavity.