Retiform hemangioendothelioma: a case series and review of the literature

Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control. Results In our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up. Conclusion Owing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma.

Epithelial papillary angioepithelioma pada rongga sinus maksila wanita dewasa muda

Latar belakang: Epithelial Papillary Angioepithelioma (EPA) yang dikenal juga sebagai tumor Dabska adalah suatu tumor vaskular yang jarang terjadi pada rongga hidung dan sinus paranasalis. Tindakan bedah, radioterapi dan kemoterapi serta kombinasi ketiganya adalah pengobatan utama untuk tumor ganas sinonasal. Tujuan: Memberikan informasi mengenai diagnosis dan penatalaksanaan tumor Dabska. Kasus: Kasus langka ini ditemukan pada wanita usia 16 tahun dengan massa tumor pada rongga hidung dan sinus paranasal yang berekstensi hingga rongga mulut. Pemeriksaan histopatologi didapatkan sel tumor endothelial yang menunjukkan pola pertumbuhan papiler. Pemeriksaan imunohistokimiaCD34 positif. Penatalaksanaan: Radioterapi preoperasi 10 kali untuk mengurangi massa tumor yang progresif kemudian dilakukan maksilektomi infrastruktur dilanjutkan radioterapi postoperasi. Dilakukan juga pemasangan protesa palatum bars postoperasi dan protesa palatomaksilaris 3 bulan pasca operasi. Evaluasi pasca operasi tampak perbaikan, tidak didapatkan infeksi maupun tanda-tanda kekambuhan,dan secara anatomi fungsi kembali seperti semula. Kesimpulan: Diagnosis dan penatalaksanaan yang cepat dan tepat dapat meningkatkan prognosis pada tumor DabskaKata kunci: Tumor Dabska, maksilektomi infrastruktur, radioterapi, tumor sinonasal, protesa ABSTRACTBackground: Epithelial papillary angioepithelioma (EPA), also known as Dabska tumor, is a very rare vascular neoplasm in the sinonasal. Surgery, radiotherapy and chemotherapy, and the combination of those three are the primary treatment for malignant sinonasal tumors. Purpose: To inform about the diagnostic and treatment of Dabska tumor. Case: We present an exceptionally rare case of EPA of the sinonasal in a 16 year old female. A well defined, reddish tumor existed at the sinonasal that extended to oral cavity. Microscopic examination revealed the endothelioid tumor cells showing a papillary growth pattern with positive imunohistchemistry of CD34. Management: Ten consecutive radiotherapies was performed preoperatively and then continued with progressive infrastructure maxillectomy andreconstructions of the maxilla, followed by postoperative radiotherapies. Postoperative management also include the mounting bars palate prosthesis and palatomaxillary prosthesis 3-month after the operation. Postoperative evaluation showed improvement, there was no sign of any infection or recurrence, and the anatomical function returned to normal. Conclusion: Prompt diagnosis and the rightmanagement could improve the prognosis in Dabska tumors.Keywords: Dabska tumor, infrastructure maxillectomy, radiotherapy, sinonasal neoplasm, prosthesis

Strong ERG Positivity in Papillary Intralymphatic Angioendothelioma of the Testis of a 24-Year-Old Male: A Case Report

Papillary intralymphatic angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally or subcutaneously. We report the case of a 24-year-old male presenting with an isolated intratesticular palpable mass and symptoms of testicular pain. Preoperative ultrasound examination showed an irregular, heterogeneous mass. Subsequent surgery and pathologic assessment revealed a papillary intralymphatic angioendothelioma (PILA), formerly known as Dabska tumor within the lymphatic spaces.