The objective of this study was to review the natural history of extrapulmonary small cell carcinoma (EPSCC) with specific emphasis on clinical features, response to treatment and survival. The records of all patients (n=34) with EPSCC treated at Yeungnam University Medical Center and Catholic University of Daegu Medical Center between 1998 and 2005 were retrieved and reviewed. The primary sites of tumor were the esophagus and thymus in 6 patients (17.6%) each, pancreas and stomach in 5 patients each (14.7%); other sites included were the cervix, abdominal lymph nodes, abdominal wall, bladder, colon, maxillary sinus, nasal cavity, ovary, parotid gland and liver. Twenty three patients out of 34 had limited disease. The median survival of all patients was 14 months. Independent prognostic factors included stage and primary tumor location. The prognosis for the patients with extensive disease and in the gastrointestinal group was unfavorable. EPSCC is a non homogeneous disease entity. As a result of its frequent recurrence, multimodal therapy has a better outcome even in cases of limited disease. Combination chemotherapy plays a central role for treatment of extensive disease in EPSCC. Further multicenter studies are now needed to determine more details regarding disease sub-class and optimal treatment modality.
Thymus Neoplasms/mortality/therapy ; Survival Rate ; Stomach Neoplasms/mortality/therapy ; Pancreatic Neoplasms/mortality/therapy ; Middle Aged ; Male ; Humans ; Female ; Esophageal Neoplasms/mortality/therapy ; Combined Modality Therapy ; Carcinoma, Small Cell/mortality/*therapy ; Aged, 80 and over ; Aged ; Adult

OBJECTIVEDiffuse panbronchiolitis, a distinct clinical entity of unknown etiology, has been reported originally and primarily in Japanese and rarely in non-Japanese populations. Macrolide therapy is effective for this once dismal disease. Diffuse panbronchiolitis complicated with thymoma is uncommon; only 2 cases have been reported to date. The aims of this study were to describe the clinical profiles, assess the response to macrolide therapy, and to discuss the possible pathogenesis of diffuse panbronchiolitis in this setting.

METHODSThe clinical profiles, macrolide therapy response of diffuse panbronchiolitis complicated with encapsulated thymoma in 2 histologically confirmed cases were described and discussed with the 2 cases reported in the literature: one complicated with encapsulated thymoma, another with invasive thymoma.

RESULTSOf the 2 cases, both had negative PPD skin testing and abnormal serum levels of various immunoglobulins, 1 had positive anti-nuclear antibody, but none had elevated cold hemagglutinin titers, and both had an excellent response to macrolide therapy. Of the 2 cases reported in the literature, both had negative PPD or tuberculin skin testing, 1 had severe hypogammaglobulinemia, 1 had elevated IgA, 1 had positive anti-DNA, 1 had elevated cold hemagglutinin titers, but both died of respiratory failure in spite of macrolide therapy in 1 case.

CONCLUSIONSPrognosis for diffuse panbronchiolitis complicated with thymoma may depend on the nature of the thymoma and on the disease course. Macrolide therapy is also effective if administered early in the disease course and if the thymoma is cured. Immunological factors may play an important role in the pathogenesis of diffuse panbronchiolitis in this setting.

Adult ; Bronchiolitis ; complications ; drug therapy ; mortality ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Thymoma ; complications ; Thymus Neoplasms ; complications

OBJECTIVETo study the diagnosis and treatment of thymoma and to assess its prognostic factors.

METHODSThe clinical data of 116 patients with thymoma were collected. A retrospective analysis was performed, by comparing the survival rate calculated by the Kaplan-Meier method with the rate of recurrence or metastasis.

RESULTSThe standard posteroanterior and lateral chest radiographs were reliable means of detection of most thymomas. Myasthenia gravis was the most commonly paraneoplastic disease (25.0%, 29/116). The extensive radical resection was beneficial for reducing the rate of recurrence of stage I or stage II thymomas (chi(2) = 4.941, P = 0.0219). The survival time could be prolonged by postoperative radiotherapy and chemotherapy. There was a strong correlation between the clinical stage and the histological classification (according to MH classification), through which the invasive behavior of thymoma could be predicted (chi(2) = 19.76, P = 0.007, RR = 1.47). The 3- 5- and 10-year survival rates were 81.2%, 67.9%, and 40.5%, respectively. Statistical analysis showed a significant negative correlation between the stage and the survival rate (chi(2) = 29.73, P = 0.0000, RR = 0.15).

CONCLUSIONThe prognosis of thymoma depends mainly on the histological classification, clinical stage and multimodality treatment rather than on the paraneoplastic diseases.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; etiology ; Prognosis ; Retrospective Studies ; Thymoma ; diagnosis ; mortality ; therapy ; Thymus Neoplasms ; diagnosis ; mortality ; therapy

BACKGROUNDThis study was designed to analyze the accuracy and reliability of several prognostic indicators for long-term survival of thymoma patients.

METHODSData from 142 patients treated for thymoma at the Tianjin Medical University Cancer Hospital from January 1954 to January 2001 were retrospectively analyzed. The Kaplan-Meier method and Cox's proportional hazards model test were used for single and multi-variable analyses respectively. The Log-rank test was used to compare survival between groups.

RESULTSThe sizes and extent of the tumors were classified as I - IV according to the Masaoka clinical staging scale. Respective 5- and 10-year survival rates were: 93.8% and 79.2% in stage I, 79.3% and 55.2% in stage II, 53.1% and 34.4% in stage III, and no survivors in stage IV. Among 30 patients with associated myasthenia gravis, 19 had generalized myasthenia gravis and 11 had ocular myasthenia gravis; 5- and 10-year survival rates were 83.3% and 60.0%, respectively. The 5- and 10-year survival rates for 112 patients without myasthenia gravis were 53.6% and 42.0%, respectively. Eighty-four patients had radical resection and nine had palliative resection. Eighty-nine patients had radiotherapy and 55 patients had postoperative radiotherapy. Single or multi-variable analyses showed that the main prognostic indicators are Masaoka clinical staging, thymoma-associated myasthenia gravis, and the treatment method.

CONCLUSIONSThe most important indicators of long-term survival in thymoma are Masaoka clinical staging and the completeness of resection. The primary treatment method for thymoma should be wide tumor resection. Pre- and/or postoperative radio- and/or chemotherapy should be given according to individual treatment requirements.

Adolescent ; Adult ; Aged ; Combined Modality Therapy ; methods ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Survival Rate ; Thymoma ; mortality ; radiotherapy ; surgery ; Thymus Neoplasms ; mortality ; radiotherapy ; surgery ; Treatment Outcome ; Young Adult