ABSTRACT
Extraskeletal Ewing's sarcoma is rarely found in the head and neck regions. We report an unusual case of extraskeletal Ewing's Sarcoma of the parapharynx region in a 49-year-old man who presented with blindness. MRI examination showed marked enhancement of tumor thrombosis involving the superior sagittal sinus, straight sinus, transverse sinus, sigmoid sinus, and internal jugular vein. The final diagnosis was extraskeletal Ewing's sarcoma after biopsy of the internal jugular vein thrombosis by histopathological evaluation and immunohistochemical assay. In addition, the patient was diagnosed as having adenocarcinoma of the rectum by biopsy of the rectal mass. The patient was treated with systemic chemotherapy and showed improved response with durable remission. The patient's visual acuity, however, did not improve.
Subject(s)
Humans , Male , Middle Aged , Antineoplastic Agents/therapeutic use , Blindness , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Head and Neck Neoplasms/diagnosis , Sarcoma, Ewing/diagnosis , Vincristine/therapeutic useABSTRACT
BACKGROUND: Several studies from western countries have reported variable prognoses for patients with systemic anaplastic large cell lymphoma (ALCL) depending strongly on the expression of anaplastic lymphoma kinase (ALK). However, no prognostic significance of ALK expression in Koreans was reported in a single report regarding these patients, although the number of cases was limited in that study. METHODS: We analyzed the clinicopathological features of ALK+ ALCL and ALK- ALCL in 30 Korean patients diagnosed with primary systemic ALCL. RESULTS: ALK expression was detected in 60% of all ALCL patients (18/30), and there was no statistical significance to ALK expression in overall survival. Patients with ALK+ ALCL were younger in age and had negative bcl-2 expression; these differences were statistically significant. Tumors positive for ALK protein and granzyme B expression, and negative for bcl-2 expression with a null-cell phenotype tended to have better survival outcomes, althought this trend failed to reach statistical significance (p<0.2), probably due to the limited number of cases in this study. CONCLUSION: ALK protein expression and the absence of bcl-2 in tumor cells tend to result in better survival despite the failure of this trend to achieve statistical significance. Further studies that examine potential pathologic prognostic factors combined with the expression of ALK and apoptotic factors such as bcl-2 are needed. Additional larger-scale studies are also needed to conclude that ALK expression has no prognostic significance among Koreans.
Subject(s)
Humans , Granzymes , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Phenotype , Phosphotransferases , PrognosisABSTRACT
Primary urethral carcinoma accounts for less than 1% of the malignancies in males and adenocarcinoma accounts for 5% of all urethral carcinomas. A 36-year-old man with a history suggestive of urethral stricture was scheduled to undergo retrograde urethrography and visual urethrotomy. On urethroscopic examination, an abnormal polypoid mass was found in the proximal bulbous urethra, so transurethral resection biopsy was added to the urethrotomy. Histopathologic examination demonstrated urethral adenocarcinoma with no invasion to the corpus spongiosum and cavernosa on the magnetic resonance imaging. There was no evidence of metastasis and the search for the primary focus of adenocarcinoma revealed that the tumor originated from the urethra. The patient underwent segmental urethral resection and there has been no recurrence for 1-year follow-up.