Malignant diffuse-type tenosynovial giant cell tumors: a series of 7 cases comparing with 24 benign lesions with review of the literature.
ABSTRACT Malignant diffuse-type tenosynovial giant cell tumor (D-TSGCT), an unusual sarcoma with concurrent or previous benign D-TSGCTs, poses challenges to diagnosis and prognostication.
We described the radiologic, clinicopathologic, and immunophenotypical findings of 5 primary and 2 metachronous malignant D-TSGCTs and reviewed published cases to better delineate their morphologic spectrum and behavior. Twenty-four benign D-TSGCTs were also statically compared to analyze the diagnostic values of various variables.
The 7 malignant cases affected 4 females and 3 males aged 45 to 78 (mean, 60.9) years, which included 1 intraarticular and 6 extra-articular lesions. These tumors were 5 to 17 cm (mean, 9.4) and located within or near the large joints of extremities. Magnetic resonance imaging revealed expansile or infiltrative masses with frequent lobulation and heterogeneous signals. Histologically, areas of benign D-TSGCTs blended abruptly or gradually with frank sarcomas composed of pleomorphic, spindle, or enlarged oval cells, forming malignant fibrous histiocytomalike (n = 4), fibrosarcomatous (n = 1), myxosarcomatous (n = 1), or giant cell tumorlike (n = 1) patterns. One patient experienced recurrences twice, and another 3 developed metastases to the lymph nodes (n = 2), lung (n = 1), or vertebrae (n = 1), with 1 dying from disseminated diseases. An older age (P = 0.003), a larger size (P = 0.036), tumor necrosis (P < 0.001), atypical mitoses (P < 0.001), and Ki-67 overexpression (P < 0.001) appeared preferentially in malignant lesions, but these parameters had overlap between few benign and malignant tumors.
Malignant D-TSGCTs are a distinct sarcoma with considerable morphologic variability, metastatic propensity, and lethality. Altered architecture with anaplastic cells represents an important distinguishing feature, while abnormalities of other parameters should not be directly equated with malignancy.
[Show abstract] [Hide abstract]
ABSTRACT: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a relatively rare mesenchymal tumor. It is a locally aggressive but virtually non-metastasizing neoplasm, and thus regarded as benign. Only a few D-TGCTs with benign histology have been reported to metastasize. Here we report an extremely rare case of benign D-TGCT in which multiple metastases developed 9 years after surgery for the primary tumor. The present case suggests that conventional D-TGCT has the potential to form distant metastases, albeit exceptionally rarely, and that this probable implantation phenomenon can be managed conservatively.Human pathology 11/2014; 45(11). DOI:10.1016/j.humpath.2014.06.025 · 2.81 Impact Factor
Targeted Oncology 02/2013; 9(1). DOI:10.1007/s11523-013-0267-8 · 3.46 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Background Malignant tenosynovial giant cell tumors (GCTs) are extremely rare, and their etiology is unknown. However, this type of malignancy is associated with high metastasis and mortality rates. Therefore, the treatment of choice is wide excision. Case Description A 66-year-old man complained of tingling and loss of sensation in the left hand, caused by a tumor that compressed the median nerve. The tumor was excised. Histopathologic examination revealed a ganglion cyst. Two years later, the patient visited our clinic with recurrent and similar complaints of the left hand. This time, however, the lesion turned out to be a malignant tenosynovial GCT and was treated by amputation of the forearm. Literature Review Since 1979, only 37 malignant tenosynovial GCTs have been reported in literature. Follow-up of these patients showed that 11 patients died of the disease, 4 patients were still living with the disease, and 14 patients had no evidence of disease after treatment. The other seven patients were lost to follow-up, and one patient died of other causes. In these 37 patients, a high incidence of lymph node metastasis (41%) and a high mortality rate (30%) were seen. Clinical Relevance Although this malignant tenosynovial GCT is very rare, high mortality rates have been observed because of the high incidence of lymph node metastases. Therefore, more awareness has to be created, to recognize and treat this tumor timely.08/2013; 2(3):271-5. DOI:10.1055/s-0033-1350087