Ullah Hidayat, Khan Imran, Khan Aria, Tangri Apoorva, Lamichhane Saral, Maqbool Shahzaib, Nashwan Abdulqadir J
Consultant Hematology/Oncology, Bolan Medical Complex Hospital Quetta, Quetta, Pakistan.
Resident Internal Medicine, NYCH+H/Woodhull, Brooklyn, NY, USA.
Case Rep Oncol. 2024 Jul 12;17(1):741-746. doi: 10.1159/000539771. eCollection 2024 Jan-Dec.
Vascular malignancies are categorized into benign hemangiomas, malignant angiosarcomas, and tumors of intermediate malignancy. Retiform hemangioendothelioma (RH) is one of the rare diagnoses belonging to the class of intermediate malignancies that was initially diagnosed.
In this case report, we have reported a rare case of RH, a locally aggressive, vascular tumor of malignant potential presenting at an unusual site. The most observed locations of this soft tissue tumor are skin and subcutaneous tissues of extremities; however, this case of RH was observed in an unusual site called pleura, which is a rare occurrence related to this tumor. The presence of cell surface markers like CD31 and ERG of vascular and endothelial origin and the presence of hobnail appearance of endothelial channels on the biopsy profile supported the vascular origin of the tumor; the clinical features and imaging studies further confirmed the diagnosis of RH.
In this case report, a rare case of RH with an unusual site of origin was highlighted. RH is a vascular neoplasm; commonly observed sites of this vascular tumor were skin and subcutaneous tissue of the extremities; however, in this case, the site was unusually different, which was the pleura of the left lung.
血管恶性肿瘤分为良性血管瘤、恶性血管肉瘤和中间型恶性肿瘤。网状血管内皮瘤（RH）是最初诊断出的属于中间型恶性肿瘤类别的罕见诊断之一。
在本病例报告中，我们报告了一例罕见的RH病例，这是一种具有局部侵袭性、有恶性潜能的血管肿瘤，出现在一个不寻常的部位。这种软组织肿瘤最常出现的部位是四肢的皮肤和皮下组织；然而，该例RH出现在一个名为胸膜的不寻常部位，这与该肿瘤相关的情况罕见。活检结果中血管和内皮来源的细胞表面标志物如CD31和ERG的存在以及内皮通道的鞋钉样外观支持了肿瘤的血管起源；临床特征和影像学研究进一步证实了RH的诊断。
在本病例报告中，突出了一例起源部位不寻常的罕见RH病例。RH是一种血管肿瘤；这种血管肿瘤常见的部位是四肢的皮肤和皮下组织；然而，在本例中，部位异常不同，是左肺的胸膜。
