Primary Pulmonary Malignant Fibrous Histiocytoma

Abstract

Malignant fibrous histiocytoma (MFH) is one of the most common adult soft tissue sarcomas. MFH is very aggressive and is most often found in the extremities and the retroperitoneum, but it can manifest at other sites. Though the lungs are the most common sites of metastasis, they rarely present there as a primary tumor. Our report describes a rare case of a primary MFH tumor in the lung. Careful diagnostic procedure should be followed to ensure the tumor does not have extrapulmonary origins. Though MFH is highly invasive and deadly, surgical excision of the tumor has been shown to be successful.

Primary undifferentiated pleomorphic sarcoma (Malignant fibrous histiocytoma) of the lung: A case report

“…In general, chest CT scan is one of the most useful examinations to determine not only location of the tumor but also tumor spread and mediastinal lymph node involvement. As previously reported, FDG PET/CT scan showed significantly increased uptake in the primary pulmonary UPS . It is still unclear how frequent pulmonary UPS shows high level of uptake by FDG PET/CT.…”

“…This suggests that complete surgical resection of the tumor should be considered for patients with primary pulmonary UPS if it appears to be resectable, and close follow‐up after surgery is important to find recurrence earlier. Although adjuvant chemotherapy or radiotherapy has been performed in some cases, their role remains uncertain for this malignancy …”

Primary Pulmonary Malignant Fibrous Histiocytoma

“…Better survival rates are mostly related to complete surgical resections with clear margins. In the same vein, survival rates are poor for patients with advanced-stage disease, incomplete resection, or tumor invasion to the mediastinum or chest wall, and the chances of recurrence and metastasis are higher [7]. Our patient had a three-year survival without recurrence.…”

“…Patients with MFH detected in the lungs should undergo an exhaustive diagnostic examination (including PET-CT and histopathological verification) to specify that the tumor is primary pulmonary MFH or a metastasis of extrapulmonary primary origin [7].…”

“…Although doxorubicin, dacarbazine, cyclophosphamide, and cisplatin might be used during chemotherapy, there are, in fact, no large-scale studies that report a favorable response to chemotherapy [7]. Close follow-up is advised due to the relatively high recurrence rates of MFH.…”