Xp11.2 translocation renal cell carcinoma (RCC) is rare and predominantly found in children and young adults. Because of the property of overexpressed transcription factor E3 (TFE3) fusion protein, immunohistochemical (IHC) staining with TFE3 antibody makes an excellent diagnostic tool. This study analyzed preliminary experiences of eight Xp11.2 translocation RCCs in our institution between 2007 and 2012. In four males and four females with a mean age of 28.4 years. Xp11.2 translocation RCCs were diagnosed. TFE3 IHC stain was positive in all tumor specimens. As the initial presentation, four patients suffered from abdominal pain, three cases had gross hematuria, and one case had hemoptysis caused by existing lung metastasis. The tumor was located in the right kidney (75%) with mean diameter of 5.85 ± 2.64 cm. Three cases (38%, 3/8) presented with lymph node metastasis at the time of diagnosis. In five cases (63%, 5/8), the initial diagnosis was Stage III and IV. Treatment included open surgery (one partial nephrectomy and five radical nephrectomies), cryoablation, immunotherapy, and target therapy. The mean follow-up time was 32 months. One patient died after 23.4 months of follow-up. The application of TFE3 IHC stain will improve the diagnostic accuracy for detecting XP11.2 translocation renal cell carcinoma. Surgery or cryoablation both had excellent prognosis in early stages. Although the disease is believed to be indolent, an increasingly aggressive clinical course should be kept in mind, especially for children and young adults.
Abstract. The current study describes a case of adenosquamous carcinoma originating from a thyroglossal duct cyst (TGDC). A 77-year-old man presented with an asymptomatic mass in the left mid-neck, which was soft and mobile on palpation. Fine-needle aspiration was performed, but cytology did not detect any malignant cells. Computed tomography demonstrated a single cystic lesion in the left lobe of the thyroid gland; therefore, surgery was performed on the suspected thyroid cyst. However, it was identified intraoperatively that the lesion was separated from the thyroid gland and instead adhered to an additional hyoid bone; therefore, the Sistrunk procedure was performed. Histopathological examination of the resected tumor confirmed the diagnosis of adenosquamous carcinoma originating from a TGDC. Carcinoma arising from a TGDC is rare, and accounts for 1% of all TGDC cases. The most common subtype of carcinoma associated with TGDC is papillary carcinoma, whilst adenosquamous carcinoma developing from a TGDC is extremely rare, with only one case currently reported in the literature. Although a consensus for the management of this disease has not yet been established, adequate surgical excision with long-term follow-up is currently the preferred treatment.
a b s t r a c tObjective: To assess the clinical characteristics, prognostic factors, and treatment outcomes of primary testicular lymphoma (PTL). Materials and Methods: We reviewed the medical records of PTL patients diagnosed between January 2000 and May 2012 at four hospitals in Taiwan. The following data were collected and analyzed: patient age at diagnosis, B symptoms (fever, night sweats, and/or weight loss), tumor localization (diffuse or testis alone), tumor site (left, right, or bilateral), stage of disease, histological pattern, International Prognostic Index, treatment method and outcome, relapse, and survival time. Kaplan Meier curves were constructed to assess the effects of various factors on patient survival. Results: Thirty patients with PTL were included in this study. The median age at diagnosis was 62.5 years (range: 19 89 years) and median overall survival (OS) was 44 months. The majority of the patients had unilateral lymphoma, stage I/II disease, and diffuse large B cell lymphoma. An absence of B symptoms, limited disease stage, lymphoma localized to testis only, and orchidectomy were considered to be significantly favorable prognostic factors of OS (p < 0.05). The PTL treatment regimen varied among patients and included orchidectomy, chemotherapy, and/or radiotherapy. Interestingly, although 50% patients achieved complete remission (CR) post treatment, relapse was observed in 42% patients during the follow up period. Nonetheless, patients who initially underwent orchidectomy appeared to have a significantly better survival rate, whereas chemotherapy proved more advantageous for those with the diffuse type PTL. Conclusion: PTL is characterized by unique clinical features and prognostic factors. Despite differing treatment regimens, 50% of patients achieved CR. Patients with testicular localization who underwent orchidectomy may achieve better outcomes, whereas chemotherapy proved to be beneficial in those with diffuse type PTL.
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