The Ewing's Sarcoma Family of Tumors of Urinary Bladder: A Case Report and Review of the Literature.

BACKGROUND: Only 15 cases of Ewing's Sarcoma (EWS) family of tumors of urinary bladder have been documented in the literature to date. CASE REPORT: We presented here a 38 year-old female with primary urinary bladder EWS with no distant metastases. She had presented with macroscopic hematuria and had undergone transurethral resection of the tumor within the following week. Microscopic examination revealed a tumor diffusely infiltrating the lamina propria and muscularis propria under an intact urothelium, which was composed of small round blue cells with scant cytoplasm, monotonous round or oval nuclei, stippled chromatin and small nucleoli. Immunohistochemistry showed strong vimentin, synaptophysin and membranous CD99 expression by the tumor. Fluorescent in situ hybridization analysis displayed the EWSR1 rearrangement. Radical cystectomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy, extended lymph node dissection, and ileal conduit were performed. As adjuvant chemotherapy, she received vincristine, doxorubicin, cyclophosphamide and mesna, alternating with courses of etoposide, iphosphamide and mesna. She is alive and well with no evidence of disease 14 months after the surgery. CONCLUSION: Surgery supported with chemotherapy should be considered as an option, especially in advanced Ewing's sarcoma family of tumors of urinary bladder.

A T-cell prolymphocytic leukemia case with central nervous system involvement.

T-cell prolymphocytic leukemia (T-PLL) is an aggressive mature T cell neoplasm that typically involves peripheral blood, bone marrow, lymph nodes and spleen. It is a rare disease that comprises 2-5% of mature lymphocytic leukemia in adults. Here we present a T-PLL patient with CNS involvement. A 74-year-old man admitted to a hospital in April 2014 with vomiting. He was diagnosed as chronic lymphocytic leukemia (CLL) and R-CVP (Rituximab, cyclophosphamide, vincristine and prednisolone) chemotherapy protocol was started. After the first two cycles of chemotherapy, the patient's mental functions improved. However after the 3(rd) cycle of chemotherapy was given in July 2014 the general situation of the patient deteriorated and ptosis of the left eye and facial paralysis developed. Then the patient was referred to our medical center. An MR of the brain revealed linear contrast enhancement around the bilateral 3(rd), 7(th) and 8(th) cranial nerves which indicated cranial involvement by the lymphoproliferative process (Figure 1). Cerebrospinal fluid cytological examination confirmed the diagnosis. Based on these and bone marrow aspiration and biopsy findings a diagnosis of T-PLL was rendered (Figure 3). In September 2014 the patient died suddenly due to a cardiac arrest. Differential diagnosis is very important in T-PLL. Both T-PLL and chronic lymphocytic leukemia (CLL) may present with splenomegaly and lymphocytosis as well as circulating prolymphocytes in blood. Typical CLL cells are like mature lymphocytes with dense nucleus and aggregated chromatin. To conclude, CNS involvement in T-PLL is a rare finding and differential diagnosis of T-PLL is very important.

Conjunctival extranodal natural killer/T-cell lymphoma, nasal type.

PURPOSE: To report a rare case of a patient with isolated primary conjunctival extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) without nasal involvement. METHODS: The clinical course, magnetic resonance imaging, positron emission tomographic and immunohistopathological features of the patient were evaluated. RESULTS: A 57-year-old man presented with rapidly progressing swelling and redness in his right eye for 2 months. A salmon-colored mass was present under all parts of the bulbar conjunctiva. Magnetic resonance imaging demonstrated a mass anterior to the globe without any sinus involvement. Positron emission tomographic study did not show other disease sites. Immunohistopathological studies on incisional biopsy specimen demonstrated ENKTCL with positive CD2, CD7, CD56, bcl-2, and T cell-restricted intracellular antigen, and negative CD20, CD8, CD123, and terminal deoxynucleotidyl transferase staining. Epstein-Barr virus (EBV)-encoded mRNA was also diffusely positive. Ki-67 index was more than 90%. The patient received cyclophosphamide, vincristine, hydroxydaunorubicin, cisplatin, and prednisone chemotherapy with 4500 cGY radiotherapy in 25 fractions to the right orbit, resulting in total resolution of the conjunctival tumor. He developed intracranial and gastrointestinal tumors and died of cardiopulmonary failure 11 months later. CONCLUSIONS: Extranodal natural killer/T-cell lymphoma, nasal type may primarily arise in the conjunctiva without nasal or paranasal sinus involvement. Despite initial successful local tumor control by systemic chemotherapy and local irradiation, the overall prognosis is poor due to systemic dissemination.

Nasal type natural killer T-cell lymphoma involving nasooropharynx and larynx.

Lymphoma is one of the malignant non-squamous tumors involving the head and neck. Lymphomas in this region are mostly B-cell type in origin and usually seen in Waldeyer's ring. In this article, we report a 45-year-old female case of primary natural killer T cell lymphoma-nasal type involving the nasooropharynx and larynx. This is a very rare entity with poor prognosis.