it was first reported in 1923 in an autopsy case by mandelstamm , and since then , fewer than 250 cases have been described .
the prognosis of patients with pulmonary artery sarcoma is usually poor . in reported cases ,
the mean survival time was usually less than 2 months for those without surgical treatment and approximately 10 months for those treated surgically .
surgical resection , if possible , is known to be the best therapeutic option to prolong survival .
thyroid and adrenal glands are representative endocrine organs that have high vascularity , and we often encounter thyroid or adrenal metastases in autopsy cases with malignant diseases ; however , malignant metastasis to both endocrine organs during follow - up is not common in the clinical setting . as imaging techniques and fine needle aspiration skills
resection of metastatic disease has been shown to prolong survival of patients in a variety of cancers .
metastasectomy can be justified in liver metastases from colorectal cancer or neuroendocrine tumors and in lung metastases from colorectal cancer or from osteogenic sarcoma .
there has been a limited number of reports evaluating the role of thyroid and/or adrenal metastasectomy [ 6 - 10 ] .
here we report a case of a patient with pulmonary artery intimal sarcoma who showed long - term survival 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 .
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 ( fig .
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 ( fig .
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 .
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 .
this lesion was just followed up with chest ct yearly and showed no change for 3 years . in august 2004 ( 4.7 years after initial surgery ) , she was referred to an endocrinologist for abnormal thyroid function tests .
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 2.02.5 cm and 0.90.9 cm in both lobes of the thyroid glands , which was no different from previous studies ( fig .
fine needle aspiration cytology was done and revealed atypical spindle cells , suggestive of metastatic intimal sarcoma ( fig .
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 .
a 10.08.5 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 ( fig .
3a ) . an f - fluorodeoxyglucose positron emission tomography ( f - fdg pet)/ct scan showed a large hypermetabolic mass ( max standardized uptake value , 6.8 ) with internal necrosis ( fig .
the tumor cells were positive for smooth muscle actin and were negative for inhibin , cytokeratin , chromogranin , and desmin , supporting the above diagnosis . during follow - up after right adrenalectomy , abdomen - pelvic ct and f - 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 ) .
intimal sarcoma is a rare tumor that arises from large vessels , including the aorta and pulmonary artery .
it is characterized by insidious growth , but it usually presents with extensive local invasion and hematogenous metastases at the time of diagnosis .
metastases are most commonly found in the lungs , although deposits in the pancreas , kidney , brain , lymph nodes , and skin have been reported .
although endocrine organs have high vascularity , metastasis to endocrine organs in the clinical setting is not as common ; however , recent autopsy studies have shown that the incidence of thyroid metastases ranges between 1% and 24% in patients with a known history of neoplasm .
the prevalence of adrenal metastases in patients with extra - adrenal cancers ranges from 32% to 73% in different series .
breast cancer and lung cancer are the most frequent malignancies showing metastases to the thyroid . in the case of adrenal metastasis , lung , kidney , breast , and
gastrointestinal carcinomas are the most frequent primary malignancies . as ultrasonography - guided aspiration and various imaging modalities such as f - fdg pet / ct
have been more frequently used in clinical settings , more cases with metastases to the thyroid or adrenal gland are encountered . moreover , asymptomatic solitary metastatic lesions are more likely to be found before they become widespread .
thus , a thyroid or adrenal mass in a patient with a history of a previous malignancy should be regarded as potentially metastatic , even if the primary tumor was adequately treated many years before .
no standard therapeutic guidelines have been established for solitary metastatic disease to the thyroid or adrenal gland .
the efficacy of surgical treatment for a solitary metastasis as seen in our case is unknown .
numerous case reports have suggested that metastases to the thyroid or adrenal gland are associated with a poor prognosis , because most thyroid or adrenal metastases occur late in the course of disseminated cancers .
surgical treatment for metastases has not been widely adopted because of the concern about probable associated advanced diffuse metastatic diseases ; however , radical treatment for an isolated metastasis to the thyroid or adrenal gland can be curative or achieve long - term survival , and an aggressive surgical approach has been recommended by some authors [ 6 - 10,18 ] .
surgery has been advocated for metastatic disease , provided the disease is limited and/or amenable to complete treatment .
preoperative investigations should be as extensive as possible in order to detect any inaccessible metastatic sites or invasion hampering complete surgery .
the prognosis of patients who have a solitary thyroid or adrenal metastasis depends on multiple factors such as the site of origin , the histological cell type of primary tumors , antecedent history of distant metastasis or discovery of other synchronous distant metastasis , and extent of local disease .
there should be no extra thyroid or adrenal disease , and preoperative imaging studies should demonstrate disease that is likely to be completely resectable .
furthermore , as in our case , an additional metastatic site does not have to be an exclusion criterion if it seems to be curable .
we treated a rare case with intimal sarcoma of the pulmonary artery that metastasized to the thyroid and adrenal glands sequentially .
the patient developed a thyroid metastasis 4.7 years after initial surgery and an adrenal metastasis 6.3 years after initial surgery .
there should be more investigations regarding which cancers should be considered for metastasectomy of the endocrine organs with curative intent .
 cancer metastases to the thyroid or adrenal gland are uncommon . 
 furthermore , cases showing long - term survival after surgical resection of those metastatic tumors are rare . 
 we report a case of pulmonary artery intimal sarcoma with metastases to the thyroid and adrenal glands sequentially that was successfully treated with sequential metastasectomies . a 62-year - old woman presented with a 4-week history of dyspnea on exertion and facial edema in november 1999 . 
 echocardiography and chest computed tomography ( ct ) revealed an embolism - like mass in the pulmonary trunk . 
 pulmonary artery endarterectomy with pulmonary valve replacement was performed , and histopathology revealed pulmonary artery intimal sarcoma . 
 a thyroid nodule was found by chest ct in november 2001 ( 2 years after initial surgery ) . during follow - up , this lesion showed no change , but we decided to obtain fine needle aspiration cytology ( fnac ) in august 2004 ( 4.7 years after initial surgery ) . 
 fnac revealed atypical spindle cells suggestive of metastatic intimal sarcoma . 
 she underwent total thyroidectomy . during follow - up , 
 a right adrenal gland mass was detected by chest ct in march 2006 ( 6.3 years after initial surgery ) , and adrenalectomy was done , which also revealed metastatic sarcoma . she has been followed up without any evidence of recurrent disease until may 2012 ( 12.5 years after initial surgery ) . 
INTRODUCTION
CASE REPORT
DISCUSSION