Paraganglioma of the urinary bladder is a rare tumor and may be misdiagnosed as urothelial cancer especially on TURBT, but a high index of suspicion, careful search for the characteristic histological features and supportive immunohistochemical studies should lead to a correct diagnosis.

Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors. They usually involve the cervix and ovary, and are seen very rarely in the endometrium. The overwhelming majority of endometrial NECs are of conventional small cell type (up to 60 cases). Only seven cases of large cell type NEC of the endometrium have been reported. We report a case of large-cell neuroendocrine carcinoma (LCNEC) of the endometrium in a 70-year-old female. The case is described for its rarity and shows that a high index of suspicion can help the pathologist to use immunohistochemistry and in turn help in selection of appropriate chemotherapy.

A 17-year-old male patient presented with lower back pain and weakness of lower limbs. On radiological investigation, a destructive bony lesion involving the third lumbar vertebra was seen associated with soft tissue extension and an extradural component. The lesion was surgically excised in view of the rapidly progressing symptoms. The histologic diagnosis was histiocytic sarcoma. Further investigations did not reveal any other lesion elsewhere. The case is being presented in view of the uncommon occurrence of a primary extranodal histiocytic sarcoma of the lumbar spine, which simulated a primary bone tumor.

Context.—Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs). Objectives.—To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death. Design.—This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded. Results.—Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (>60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (>60%) was a significant risk factor in univariate analysis, which is the unique feature in this study. Conclusions.—Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.

We report a study of 84 extranodal natural killer/T cell lymphomas (ENKTCLs) from India to understand the disease pattern. Sixty-one patients had nasal disease and three had Waldeyer's ring tumors, while 20 had extranasal sites of presentation, namely: cervix (n = 1), oral cavity (n = 3), orbit (n = 3), liver (n = 1), ileum (n = 1), and soft tissue and lymph nodes (n = 9). Staging of patients with extranasal disease revealed that either they had disseminated disease or the nasal mass was missed during the initial staging. Extranasal presentation, performance status (PS), international prognostic index (IPI), Korean prognostic index, marrow involvement, radiotherapy and type of chemotherapy affected overall survival, while lymphopenia, performance status, radiotherapy and type of chemotherapy impacted disease-free survival. In the multivariate analysis, IPI and response to chemotherapy emerged as significant prognostic factors. Thus most patients with extranasal ENKTCL have a nasal lesion or disseminated disease and pure extranasal disease is uncommon in non-endemic regions.

Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi, which commonly affects young men and predominantly involves the mucous membranes of the nose, nasopharynx and eyes. Involvement of extranasal sites including bone has been reported, but rarely. We present a case of a 32-year-old farmer, who presented with a non-tender, fixed swelling over his right shoulder. Radiography and CT imaging showed an expansile, lytic, destructive lesion involving the lateral end of the right clavicle with soft tissue extension. The clinicoradiological impression was that of a primary bone tumor. Biopsy and wide excision of the lesion revealed features of rhinosporidiosis. There were no mucocutaneous lesions. Involvement of bone without associated mucocutaneous lesions is exceedingly rare in rhinosporidiosis. Involvement of the clavicle, as seen in the present case, has not been reported in the English language-peer reviewed literature, to the best of our knowledge.

Phosphaturic mesenchymal tumor (PMT) is a rare and distinctive tumor almost always associated with oncogenic osteomalacia. This tumor is often misdiagnosed due to its widely varied histomorphological spectrum. More recently the term PMT or its variants has been used whatever the histomorphological features. Herein, we present a case of a 32-year-old woman who presented with a 2-year history of oncogenic osteomalacia. The clinical examination failed to detect any tumor. The F-18 FDG PET scan and subsequent MRI revealed a tumor in the soft tissues of the right popliteal fossa. The histological features resembled a chondromyxoid fibroma (CMF), a feature documented only in two cases so far.

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