CAJ | 학술논문

目的探讨8例网状血管内皮瘤（ RH）的临床病理学特征，并扩展其瘤谱。方法回顾性复习8例RH的临床资料、病理学形态和免疫学表型，分析随访结果，并复习文献。结果患者平均年龄40岁（范围13～69岁），本组均为女性。肿瘤分别发生于头颈部和下肢皮肤（5例）、长骨（2例）和脾脏（1例）。皮肤病变表现为局部缓慢性生长的斑块或皮下结节，骨病变表现为患肢疼痛，脾脏病变表现为左季肋部隐痛不适。大体上呈灰黄或红褐色，平均直径3.0 cm，范围0.8～5.0 cm。镜下观察，肿瘤主要由内衬鞋钉样或火柴头样内皮细胞的细长分支状血管组成，形成特征性的网状结构，类似于睾丸网，部分区域内可见条索状或实性巢状排列的上皮样瘤细胞。除细长的网状血管外，3例于局部可见扩张的血管腔，腔内有乳头簇形成，表面被覆鞋钉样内皮细胞，轴心玻璃样变，形态上类似Dabska瘤。血管周围的间质多伴有淋巴细胞浸润，并可有胶原化。本组1例发生区域淋巴结转移。免疫组织化学标记结果显示，网状血管内皮细胞表达 CD31（8／8）、人凝血因子Ⅷ（5／8）、CD34（5／7）和D2-40（1／2）。随访6例（18～67个月），2例局部复发，无病例发生远处转移。结论RH是一种好发于成年人的中间型血管内皮瘤，以细长分支状血管和鞋钉样内皮细胞为特征，除皮肤外，部分病例可原发于骨和脾脏。部分病例兼有Dabska瘤样形态特点，提示与Dabska瘤有延续性，归属鞋钉样血管内皮瘤同一瘤谱。熟悉网状血管内皮瘤的形态学特点有助于与其他具有鞋钉样内皮细胞的血管肿瘤相鉴别。
목적탐토8례망상혈관내피류（ RH）적림상병이학특정，병확전기류보。방법회고성복습8례RH적림상자료、병이학형태화면역학표형，분석수방결과，병복습문헌。결과환자평균년령40세（범위13～69세），본조균위녀성。종류분별발생우두경부화하지피부（5례）、장골（2례）화비장（1례）。피부병변표현위국부완만성생장적반괴혹피하결절，골병변표현위환지동통，비장병변표현위좌계륵부은통불괄。대체상정회황혹홍갈색，평균직경3.0 cm，범위0.8～5.0 cm。경하관찰，종류주요유내츤혜정양혹화시두양내피세포적세장분지상혈관조성，형성특정성적망상결구，유사우고환망，부분구역내가견조색상혹실성소상배렬적상피양류세포。제세장적망상혈관외，3례우국부가견확장적혈관강，강내유유두족형성，표면피복혜정양내피세포，축심파리양변，형태상유사Dabska류。혈관주위적간질다반유림파세포침윤，병가유효원화。본조1례발생구역림파결전이。면역조직화학표기결과현시，망상혈관내피세포표체 CD31（8／8）、인응혈인자Ⅷ（5／8）、CD34（5／7）화D2-40（1／2）。수방6례（18～67개월），2례국부복발，무병례발생원처전이。결론RH시일충호발우성년인적중간형혈관내피류，이세장분지상혈관화혜정양내피세포위특정，제피부외，부분병례가원발우골화비장。부분병례겸유Dabska류양형태특점，제시여Dabska류유연속성，귀속혜정양혈관내피류동일류보。숙실망상혈관내피류적형태학특점유조우여기타구유혜정양내피세포적혈관종류상감별。
Objective To further elucidate the clinical and pathologic features of retiform hemangioendothelioma ( RH) and its clinical spectrum.Methods Eight cases of RH were reviewed.The clinicopathologic profiles , immunophenotypes and outcome data were investigated.Results All 8 cases occurred in females with a mean age at presentation of 40 years (range, 13-69 years).Five tumors arose in the skin of the head and neck region and lower extremities , two in the long bones and one in the spleen.Clinically , the patients presented with a slowly growing cutaneous plaque or subcutaneous nodule , pain of the upper arm, and dull pain and discomfort in the left hypochondrium respectively.Grossly, the tumor appeared as a non-encapsulated gray-yellowish to tan-brown mass with a mean diameter of 2.6 cm (range, 0.8-5.0 cm).On histology, it was composed of delicate branches of elongated vessels lined by a layer of hobnail or matchstick endothelium , exhibiting a retiform pattern with close resemblance of the normal rete testis.Cords or solid nests of epithelioid cells were noted adjacent to the well-formed vessels.In three cases, dilated vascular spaces with formation of intravascular papillary tufts were observed , features overlapping with Dabska tumor.There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases.One case had regional lymph node metastasis.By immunohistochemistry , all cases consistently expressed endothelial markers , including CD31 ( 8/8 ) , human coagulation factor Ⅷ(5/8), CD34 (5/7) and D2-40 (1/2).Two of six cases with follow-up information (18 -67 months) developed local recurrences , but distant metastasis was not identified.Conclusions RH is a distinctive vascular tumor of adulthood characterized by retiform growth of vessels with striking hobnail endothelium.Although the tumor occurs predominantly in the skin , the long bones and the spleen can be occasionally affected.The presence of Dabska tumor-like areas in RH may suggest a morphologic continuum between these two entities , comprising the family of hobnail hemangioendothelioma.Familiarity with the characteristic features of this vascular tumor of intermediate malignancy will help in the differential diagnosis of vascular neoplasms with hobnail endothelium .