ABSTRACT
INTRODUCTION AND OBJECTIVES: Primary cutaneous angiosarcoma is one of the most aggressive skin tumors and carries a very poor prognosis. Its initially indolent clinical presentation explains the frequently late diagnosis that, together with its typically multifocal pattern and poor delimitation, often makes surgery difficult. The low incidence of primary cutaneous angiosarcoma means that few large single-center series have been published. We review the clinical and pathologic characteristics of cutaneous angiosarcomas treated in our hospital, looking for prognostic factors and for possible diagnostic traits that could facilitate early diagnosis. MATERIAL AND METHODS: This was a retrospective observational study including all patients diagnosed with cutaneous angiosarcoma in Instituto Valenciano de Oncología in Valencia, Spain between January 2000 and December 2015. We recorded 16 clinical parameters, including age, sex, type of angiosarcoma, site, size, and time since diagnosis, and 8 histopathologic parameters. RESULTS: We identified 16 patients (11 women and 5 men) with cutaneous angiosarcoma. Their mean age was 67 years (median, 71 years). The most common site was the trunk (10 cases), followed by the head and neck (5 cases). The mean size of the tumor was 10cm (median, 6.5cm). Fourteen patients underwent surgical excision. Six of the 16 patients were alive at the end of the study, after a mean follow-up period of 42.5 months. CONCLUSIONS: The major determinants of survival among patients with cutaneous angiosarcoma are tumor size and patient age. Other characteristics associated with a poor prognosis were infiltration of deep planes (muscle), a predominantly solid histologic pattern, and a larger number of mitoses.
Subject(s)
Hemangiosarcoma/pathology , Skin Neoplasms/pathology , Adult , Aged , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/pathology , Breast Neoplasms/therapy , Cancer Care Facilities , Combined Modality Therapy , Early Detection of Cancer , Female , Follow-Up Studies , Head and Neck Neoplasms/pathology , Head and Neck Neoplasms/therapy , Hemangiosarcoma/diagnosis , Hemangiosarcoma/etiology , Hemangiosarcoma/therapy , Humans , Lymphedema/complications , Male , Middle Aged , Neoplasms, Radiation-Induced/diagnosis , Neoplasms, Radiation-Induced/etiology , Neoplasms, Radiation-Induced/pathology , Neoplasms, Radiation-Induced/therapy , Neoplasms, Second Primary/diagnosis , Neoplasms, Second Primary/pathology , Neoplasms, Second Primary/therapy , Prognosis , Radiotherapy/adverse effects , Radiotherapy, Adjuvant , Retrospective Studies , Skin Neoplasms/diagnosis , Skin Neoplasms/etiology , Skin Neoplasms/therapyABSTRACT
El angiosarcoma de mama es una entidad extremadamente infrecuente y de pronóstico infausto, que puede darse de forma primaria, o secundaria a radioterapia tras cirugía conservadora de mama. Puede presentarse como una masa palpable, o bien, como lesiones cutáneas violáceas que pueden simular cambios benignos derivados de la radioterapia. El diagnóstico precoz es esencial, tomando muestras para biopsia de dichas lesiones, y prestando especial interés a la inmunohistoquímica. El tratamiento de este tipo de neoplasias es un tema controvertido ya que, dada la rareza de su aparición, no existen protocolos definidos sobre la actitud a seguir, siendo de vital importancia las terapias asociadas a cirugía radical. Aquí se presenta un caso de una paciente de 62 años que desarrolla un angiosarcoma cutáneo de mama, secundario a radioterapia, clínicamente observado como lesiones papulares violáceas sobre la zona irradiada previamente, tras un carcinoma de mama tratado con cirugía conservadora, con una supervivencia de aproximadamente 12 meses entre el diagnóstico y el éxitus de la paciente.
Breast angiosarcomas are very rare malignancies with an unfortunate prognosis, which can occur in a primary form, or secondary to radiotherapy after conservative breast surgery. It can present as a palpable mass, or as violaceous cutaneous lesions that can mimic benign changes derived from radiotherapy. Early diagnosis is essential, biopsying such lesions, and paying particular attention to immunohistochemistry. The treatment of this type of neoplasias is a controversial issue since, given the rarity of its appearance, there are no defined protocols to be followed, being the therapies associated with radical surgery of vital importance. Here we present a case of a 62-year-old female patient who develops cutaneous angiosarcoma of the breast secondary to radiotherapy, clinically observed as violaceous papular lesions on the previously irradiated area, after a breast cancer treated with conservative surgery, and with a survival of approximately 12 months between the diagnosis and the patients success.
ABSTRACT
Comunicamos un caso de un paciente de sexo masculino de 54 años de edad, con un linfangiosarcoma símil síndrome de Stewart-Treves post-linfedema crónico, secundario a un traumatismo. El enfermo rechazó la amputación por lo que fue irradiado y luego recibió quimioterapia, interferón y talidomida; no obstante ello, fallece debido a metástasis pulmonares a los cuatro años de efectuado el diagnóstico. Se efectúa una revisión de los angiosarcomas en general, del linfangiosarcoma, como asimismo una nota conceptual sobre los llamados hemangioendoteliomas.
A 54 years-old man with a lymphangiosarcoma, associated to a post traumatic chronic lymphedema Stewart-Treves like, was reported. The patient refused the amputation of the superior left arm so radiation therapy was done. Despite the adjuvant chemotherapy and subcutaneous interferon, together with thalidomide the patient death four years after the diagnosis due to disseminated lung metastasis. A brief review of the cutaneous angiosarcoma and a conceptual nosological position of the so called hemangioendotheliomas, were made.
ABSTRACT
Comunicamos un caso de un paciente de sexo masculino de 54 años de edad, con un linfangiosarcoma símil síndrome de Stewart-Treves post-linfedema crónico, secundario a un traumatismo. El enfermo rechazó la amputación por lo que fue irradiado y luego recibió quimioterapia, interferón y talidomida; no obstante ello, fallece debido a metástasis pulmonares a los cuatro años de efectuado el diagnóstico. Se efectúa una revisión de los angiosarcomas en general, del linfangiosarcoma, como asimismo una nota conceptual sobre los llamados hemangioendoteliomas.(AU)
A 54 years-old man with a lymphangiosarcoma, associated to a post traumatic chronic lymphedema Stewart-Treves like, was reported. The patient refused the amputation of the superior left arm so radiation therapy was done. Despite the adjuvant chemotherapy and subcutaneous interferon, together with thalidomide the patient death four years after the diagnosis due to disseminated lung metastasis. A brief review of the cutaneous angiosarcoma and a conceptual nosological position of the so called hemangioendotheliomas, were made.(AU)
