Long-Term Survival of a Patient with Pulmonary Artery Intimal Sarcoma after Sequential Metastasectomies of the Thyroid and Adrenal Glands
A 62-year-old woman presented with a 4-week history of dyspnea on exertion and facial edema in November 1999. Her past medical history was unremarkable. Electrocardiography showed T wave inversion at leads V1-5, III, and aVF, right axis deviation, and right ventricular hypertrophy. Initial echocardiography revealed an echogenic mass in the main pulmonary artery with significant flow obstruction and right ventricular dysfunction suggesting pulmonary embolism. Chest computed tomography (CT) revealed an embolism-like mass in the pulmonary trunk and in both sides of the main pulmonary arteries (). Pulmonary artery endarterectomy with pulmonary valve replacement and pulmonary artery reconstruction were performed. The surgical specimen showed an ill-defined, protruding polypoid mass that was 4.8 cm in the greatest dimension. The pathology revealed pulmonary artery intimal sarcoma, which was supported by positive smooth muscle actin and negative desmin in immunohistochemical staining (). Involvement of the resection margin by the tumor was noted. She received radiation therapy (4,500 cGy fractionated) and chemotherapy (cyclophosphamide, doxorubicin, and dacarbazine) as adjuvant treatments. During follow-up, she was regularly checked by echocardiography and chest CT. There was no evidence of local recurrence on echocardiography. On initial follow-up chest CT (November 2001, 2 years after initial surgery), mild diffuse enlargement of the thyroid gland with multiple small low density lesions was discovered. It is hard to know if these findings existed before the initial operation, because the preoperative chest CT did not include the neck. She showed normal thyroid function tests. This lesion was just followed up with chest CT yearly and showed no change for 3 years.\nIn August 2004 (4.7 years after initial surgery), she was referred to an endocrinologist for abnormal thyroid function tests. There was a palpable nodule in the left thyroid gland. Cervical lymphadenopathy was not present. The serum thyroid stimulating hormone level was 0.12 mIU/L (normal, 0.4 to 5.0), and total T4 was 166 nmol/L (normal, 76 to 178). CT of the neck showed two low density nodules of 2.0×2.5 cm and 0.9×0.9 cm in both lobes of the thyroid glands, which was no different from previous studies (). Fine needle aspiration cytology was done and revealed atypical spindle cells, suggestive of metastatic intimal sarcoma (). She had no other metastatic sites in the chest and abdomen-pelvic CT. She underwent total thyroidectomy, and histology confirmed metastatic intimal sarcoma with clear resection margins. She was followed by neck and chest CT (covering the adrenal gland), and there was no evidence of local recurrence or distant metastasis until August 2005.\nA 10.0×8.5 cm sized round, heterogeneous mass replacing the whole adrenal gland superior to the right kidney was newly detected by chest CT in March 2006 (6.3 years after initial surgery), suggesting a metastatic lesion in the right adrenal gland (). An 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT scan showed a large hypermetabolic mass (max standardized uptake value, 6.8) with internal necrosis (). The lesion was suspected as a recurrence of intimal sarcoma. Right adrenalectomy was done without preoperative biopsy. The histopathologic diagnosis was metastatic intimal sarcoma. The tumor cells were positive for smooth muscle actin and were negative for inhibin, cytokeratin, chromogranin, and desmin, supporting the above diagnosis.\nDuring follow-up after right adrenalectomy, abdomen-pelvic CT and 18F-FDG PET/CT showed no evidence of recurrences or metastases, including the anastomosis site of the main pulmonary trunk, thyroid bed, and adrenalectomy site. She has been alive and well without any evidence of disease as of May 2012 (12.5 years after initial surgery).