8) Although chondro sarcoma is the most frequent musculoskeletal malignancy encountered in Maffucci syndrome, malignant transformation of skeletal lesions into fibrosarcomas and of vascular lesions into hemangiosarcomas, hemangioendotheliomas
, and lymphangiosarcomas have also been reported.
The majority of consumptive hypothyroidism cases have been described in children with hemangioendotheliomas
, but cases have also been reported in adults with massive vascular and fibrous tumors (6-8).
They include hemangiomas, littoral cell angiomas, splenic hamartomas (SHs), lymphangiomas, hemangioendotheliomas
, angiosarcomas, and Sclerosing Angiomatoid Nodular Transformation (SANT).
Malignant tumors of blood vessels: Angiosarcomas, hemangioendotheliomas
, and hemangiopericytomas.
Hemangiosarcomas are malignant neoplasms that arise from the vascular endothelium and are also known as angiosarcomas or hemangioendotheliomas
The soft tissue abnormalities in Maffucci's syndrome are usually cavernous or capillary hemangiomas and, less frequently, lymphangiomas and epithelioid hemangioendotheliomas
The initial pathologic specimens were reviewed and considered to be epithelioid hemangioendotheliomas
many hemangioma subtypes such as hobnail, microvenular, sinusoidal), and malignancies (four hemangioendotheliomas
, several types of angiosarcoma, and Kaposi's sarcoma).
The wide expression of CD10 in healthy tissue is matched by its expression in a growing list of neoplasms, often with a high percentage (20%-100%) stained, including in cutaneous adnexal neoplasms, (18) malignant mesotheliomas, (19) epithelioid hemangioendotheliomas
, (64) ovarian clear cell carcinomas, (33) urothelial carcinomas, (22) prostatic adenocarcinomas, pancreatic adenocarcinomas, hepatocellular carcinomas, colonic adenocarcinomas, malignant melanomas, spindle cell sarcomas, lung carcinomas, ovarian carcinomas, endometrial stromal sarcomas, and pancreatic solid pseudopapillary tumors.
The idea that spindle-cell hemangioendotheliomas
are nonneoplastic was advocated by Fletcher et al, who reported that these tumors arise in association with a local acquired or congenital focal abnormality of blood flow at the affected site.
Since epithelioid hemangioendotheliomas
and angiosarcomas may express cytokeratins, even if a vasoformative tumor is keratin positive, the tumor should be stained with vascular markers.
The spectrum of these lesions varies from benign to malignant, including hemangiomas, hemangioendotheliomas
, and angiosarcomas.