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May 2025 DOI 10.14302/issn.2379-8572.joa-25-5504
Hachemi MohamedCorresponding author
Introduction Angiosarcoma is a rare malignant tumour from fast-evolving and aggressive vascular endothelial cells that accounts for 1% of all sarcomas. The sinus location of angiosarcoma is exceptional. The clinical polymorphism of this tumour may cause a delay in diagnosis. Risk factors are not always understood. However, a history of radiation therapy for locoregional cancer and/or lymphedema is most commonly found. The prognosis remains bleak with an overall five-year survival of 35 to 40%. Objectives The authors discuss the diagnostic, therapeutic and prognostic modalities and difficulties of this tumour. Observation: The authors report a case of maxillary sinus angiosarcoma confirmed by radio-induced secondary CD34 immunohistological staining in a 40-year-old man having a history of hemangioendothelioma treated with conservative endoscopic surgery and adjuvant radiotherapy 15 months earlier. Discussion This lesion is rare in the ENT sphere and may be confused with a benign tumor in its early stages. Only histological examination with immunohistochemistry can confirm the diagnosis (factor VIII, CD34 and CD31 antigens). Treatment consists of a wide resection followed by radiotherapy, hence the need for early diagnosis in the metastatic phase, first-line treatments include doxorubicin or paclitaxel weekly. Conclusion Radio-induced angiosarcoma is a rare malignant tumour, it occurs in patients who have received conservative treatment and radiation therapy for nasosinusitis cancers. Angiosarcomas of the nasal passages remain a diagnostic and therapeutic challenge due to their local and general aggressiveness.
Oct 2022 DOI 10.14302/issn.2641-5518.jcci-22-4323
Y. Fernando GracieuxCorresponding author
Consultant, Section of Medical Oncology-Department of Internal Medicine, University of the Philippines-College of Medicine Philippine General Hospital, Manila
Introduction Primary sarcomas of the breast are <0.1% of all malignant tumours of the breast. To date, there are 13 major breast sarcoma series in English literature. This study adds to these series characterizing primary breast sarcoma among Philippine patients. Methods All breast biopsies from the pathology records of the University of the Philippines-Philippine General Hospital (UP-PGH) were searched for breast sarcoma cases from January 2000 to December 2010. Metaplastic carcinomas and phyllodes tumors were excluded. Results There were 52 patients (45 female, 7 male) ranging in age 25-83 years (median 46 years). Majority had lump, ten cases with pain. No history of previous cancer was given. No history of prior radiation was found. Histopathological diagnoses were spindle cell sarcoma (n=13), fibrosarcoma (n=6), liposarcoma (n=6), MPNST (n=5), stromal sarcoma (n=5), angiosarcoma (n=4), MFH (n=4), leiomyosarcoma (n=3), rhabdomyosarcoma (n=3), chondrosarcoma (n=2), and synovial sarcoma (n=1). Tumors were with grade 1 (n=18), grade 2 (n=8), and grade 3 (n=10). Necrosis was noted in 6 cases. Simple mastectomy was done in 19 cases (37%), MRM in 31 cases (59%), while 2 far advanced had no surgery (3%). None had adjuvant radiotherapy or chemotherapy. The duration of follow-up for 45 patients ranged from 1 – 117 months, excluding those who were lost to follow-up. All 15 deaths were due to progressive disease. Recurrences were observed in 9 patients. The disease-free survival (DFS) and overall survival (OS) was 73%and 75%, respectively. On multivariate analysis, DFS and OS were significantly correlated with size (HR=113.63; p=0.019 and HR=77.36; p=0.037), grade (HR=20.73 ; p=0.003andHR= 39.57; p= 0.004), and having a histology of angiosarcoma (HR=35.20 ; p=0.005and HR= 50.74; p=0.007), respectively. Conclusion Sarcoma remains an important clinical entity among primary breast cancers.
Sep 2020 DOI 10.14302/issn.2471-2175.jdrt-20-3511
Bajaj AnubhaCorresponding author
MD. (Pathology) Panjab University, Department of Histopathology, A.B. Diagnostics, A-1, Ring Road, Rajouri Garden, New Delhi, 110027, India.
Intravascular papillary endothelial hyperplasia is an exceptional, benign, inflammatory, vascular neoplasm delineating papillary configuration engendered from reactive proliferation of damaged endothelial cells, while being confined to a thrombus. Initially scripted by Pierre Mason in 1923, the tumefaction was denominated as an intra-luminal lesion within an ulcerated, haemorrhoidal vein and designated as “hemangio-endotheliome’ vegetant’ intravasculaire”(1). The neoplasm is additionally nomenclated as Masson’s tumour, Masson’s pseudo-angiosarcoma, endovascularite proliferante thrombopoietique, intravenous atypical vascular proliferation, intravascular angiomatosis, vascular angiomatosis, intravascular endothelial proliferation, reactive papillary endothelial hyperplasia or intravascular papillary endothelial hyperplasia. The papillary neoplasm is associated with deposition of fibrin and thrombotic substances within a painful, ulcerated.