Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome

Abstract

Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. The biopsy specimen showed an ill-defined lesion composed of a proliferation of irregular and sometimes anastomosing vascular structures in the dermis, lined by endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no mitotic figures. As the lesion grew within deeper dermis, these proliferating vessels dissected dermal collagenous bands, occasionally arranged in low-papillary projections and/or characteristic hobnail cytomorphology. We first interpreted it as low-grade angiosarcoma following chronic lymphedema due to the operation, i.e., the so-called Stewart-Treves syndrome. Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well. Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%. Therefore, we finally made a diagnosis of BL of the skin. MIB-1 labeling index might be useful and adjunctive aids for reaching the correct diagnosis of cutaneous BL, especially in case of small or inadequate specimens.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_197

Surgical Treatment for Benign Lymphangioendothelioma After Two Incomplete Excisions: A Case Report and Literature Review

“…We also searched cited cases prior to the official recognition of the terms “APL” and “BL” and identified a total of 83 patients in 40 reports ( Table 3 ), including 27 cases diagnosed with BL after radiotherapy for breast carcinoma. 6 , 7 , 11 , 12 Recurrences were observed only in cases with incomplete excision, 6 , 8 , 12 with only one lesion progressing to an angiosarcoma eight years later. 12 …”

“… 6 , 7 , 11 , 12 Recurrences were observed only in cases with incomplete excision, 6 , 8 , 12 with only one lesion progressing to an angiosarcoma eight years later. 12 …”

“…As shown in Table 3 , significant quantities of endothelial cells were only observed in eight of the 83 cases. 6 , 7 , 37 Usually, extravasated red cells and hemosiderin deposition, as well as marked inflammation, are rarely observed, indicating a predominant involvement of lymphatic channels. This is further supported by positive immunohistochemistry for lymphatic-specific markers such as D2-40.…”

Feasibility study of combined dynamic imaging and lymphaticovenous anastomosis surgery for breast cancer-related lymphoedema

“…It can impair quality of life significantly, have a profound psychological impact, and be a reminder of cancer diagnosis. In rare instances, it can predispose to developing lymphangiosarcoma and retiform lymphangioendothelioma. Although physiological lymphatic surgery such as LVA may offer a therapeutic benefit, the impetus is on the surgeon to select only those patients who may benefit and, above all, avoid interventions that may worsen BCRL.…”

Embryonal rhabdomyosarcoma arising from the uterine corpus in a postmenopausal female: a surgical case challenging the genuine diagnosis on a cytology specimen

“…All immunohistochemical stainings below were carried out using Dako Envision kit (Dako Cytomation Co., Glostrup, Denmark) according to the manufacturer’s instructions [ 8 , 9 ]. Immunohistochemically, the atypical tumor cells were specifically positive for desmin (Dako, diluted 1:150) (Fig.…”