Urbańczyk Katarzyna, Stachura Jerzy, Papla Bolesław, Karcz Danuta, Matłok Maciej
Department of Patomorphology, Collegium Medicum, Jagiellonian University, Kraków.
Pol J Pathol. 2007;58(3):207-14.
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.
作者报告了一例胃肠道多发血管球瘤（GTs）病例，该病例包括一例典型的胃血管球瘤以及具有肌周细胞瘤样特征的大肠血管肌脂肪瘤，患者为46岁女性，伴有原始细胞的多灶性血管周围增殖及肝脏受累。组织学上，多小叶的胃肿瘤及肝脏病变符合典型的血管球瘤，而位于横结肠、直径达7 cm的肿瘤表现为浸润性血管肌脂肪瘤（伴有肌周细胞瘤样灶），大肠黏膜内直径0.5 - 0.7 cm的卫星肿瘤为小血管肌脂肪瘤。此外，患者还表现出两种伴随的血管病变：1/ 以肿瘤栓子形式的血管内播散，阻塞了中等大小静脉的管腔；2/ 在主肿瘤附近及相邻脂肪组织中可见原始小细胞的显微镜下血管周围增殖。患者患病2.5年；接受了部分结肠切除术，切除了一段小肠袢、大网膜及右卵巢，随后进行了化疗。目前，她病情稳定，浸润——尤其是上腹部区域的浸润——已减轻。该病例可能证实了以下理论：多发血管球瘤与血管周围干细胞的多灶性增殖相关，以及它们侵入大血管管腔的能力导致了卫星肿瘤的产生，这些卫星肿瘤不一定是转移性的。目前看来，血管周围SMA +肿瘤组可能包括成人的婴儿型肌纤维瘤病、肌周细胞瘤、血管肌（周）细胞瘤、血管肌脂肪瘤、典型血管球瘤和血管瘤。很可能，一些以前归类为血管外皮细胞瘤的肿瘤也属于这一组。上述至少一些血管周围肿瘤的独特特征是它们在特定区域的同步或异时生长，以及它们以结节或实体带形式占据血管内空间的能力，这反过来可能导致卫星肿瘤的产生。与特定患者短生存期相关的多灶性病变显然支持转移性疾病的存在。在其余病例中，确定患者是否患有转移性疾病需要深入考虑并谨慎判断，在决定采用何种治疗方法时也是如此。
