Gastric solid glomus tumor and multiple glomangiomyomas of the large bowel with intravascular spread, multifocal perivascular proliferations and liver involvement.
The authors present a case of multiple glomus tumors (GTs) of the gastrointestinal tract, representing the type of a gastric glomus tumor proper and large bowel glomangiomyomas with myopericytoma-like features, observed in a 46-year old female, with multifocal perivascular proliferations of primitive cells and hepatic involvement. Histologically, the multilobular gastric tumor and hepatic lesions corresponded to a typical glomus tumor, while the tumor situated in the transverse colon, up to 7 cm in diameter, presented as a glomangiomyoma infiltrative (with myopericytoma-like foci), and satellite tumors in the large bowel mucosa, 0.5-0.7 cm in diameter, represented small glomangiomyomas. In addition, the patient demonstrated two types of concomitant vascular lesions: 1/ intravascular spread in the form of neoplastic plugs that obliterated the lumen of medium-size veins, and 2/ microscopic perivascular proliferation of primitive, small cells seen in the vicinity of the main tumor and in the adjacent adipose tissue. The patient has been ill for 2.5 years; she has been subjected to a partial colectomy with a resection of the small intestinal loop, greater omentum and the right ovary, followed by chemotherapy. At present, she is stable, and the infiltration--especially in the epigastric region--has decreased. The picture may confirm the theory that multiple GTs develop in association with multifocal proliferation of perivascular stem cells, as well as that their ability to penetrate into the lumen of large vessels gives origin to satellite tumors, which are not necessarily metastatic. It seems that at present, the group of perivascular SMA+ tumors may include infantile-type myofibromatosis in adults, myopericytoma, glomangio(myo)pericytoma, glomangiomyoma, glomus tumor proper, and glomangioma. Most likely, also some tumors previously classified as hemangiopericytomas belong to this group. The distinctive feature present in at least some of the above listed perivascular tumors is their synchronous or metachronous growth in a particular region and their ability to occupy intravascular space as nodules or solid bands, which in turn may give origin to satellite tumors. Multifocal lesions associated with a short survival in a given patient will obviously support the presence of metastatic disease. In the remaining cases, determination whether the patient has metastatic disease requires deep consideration and caution, also while deciding on treatment to be employed.
Urbańczyk
Katarzyna
K
Department of Patomorphology, Collegium Medicum, Jagiellonian University, Kraków.
Stachura
Jerzy
J
Papla
Bolesław
B
Karcz
Danuta
D
Matłok
Maciej
M
D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D005918:Glomus Tumor / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007420:Intestine, Large / Q000473:pathology*; D008113:Liver Neoplasms / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D013274:Stomach Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy
D014408:Biomarkers, Tumor