Cutaneous Lymphangioma in a Post Mastectomy Female: Following Radiotherapy

Sir, Lymphangioma is a rare vascular tumor. They are usually primary, but secondary causes include postirradiation. Lymphangiomas are dilated lymphatic spaces with lymphoid aggregates lined by endothelial cells and are separated by connective tissue stroma.[1] This case report is of a postmastectomy female who developed cutaneous lymphangiomatous lesions restricted to the site of radiotherapy. No similar case has been reported in the Indian literature so far. A 38-year-old female with no chronic illness was diagnosed with a case of locally advanced breast carcinoma (right) in April 2021. After initial neoadjuvant chemotherapy for 6 months, she underwent a modified radical mastectomy in November 2021. She received radiotherapy of 50 Gy of radiation dose over 5 weeks with tangential beams to the right chest wall postmastectomy. She had up to grade 2 dermatitis due to radiation (CTCAE v5.0). This amount of dermatitis is common in patients receiving chest wall radiotherapy. After this, being hormone receptor-positive, she was started on hormonal therapy, which has continued to date. The patient was referred to the dermatology department for complaints of multiple asymptomatic raised lesions over the right chest wall in the irradiated area over the last 7 months. The lesions were insidious in onset about 3 years after the irradiation and persistent. The patient was anxious about whether it was a metastasis of her erstwhile breast cancer. Dermatological examination revealed multiple discrete firm nontender hypopigmented and hyperpigmented glistening nodules of varying sizes over the right side of the chest wall [Figures 1 and 2]. Histopathological examination from one of the lesions revealed multiple dilated vascular channels lined by endothelial cells (positive to CD240, CD34 on immunohistochemistry) enclosing predominantly proteinaceous fluid with few accompanying delicate capillaries in the dermis as shown in Figure 3. As the patient was reassured about the benign nature of the lesions, she did not opt for any treatment for the same. She has been advised to regular follow-up.Figure 1: Multiple firm nodular lesions varying in size the largest up to one cm over the area of irradiation of the patient’s right breastFigure 2: A closer view of the individual translucent nodules on the right chest wallFigure 3: Histopathological examination of the lesion (a) H and E stain (×2): Nodular skin lesion (b) H and E stain (×20): Epidermis with thin compact keratin layer. Dermis shows dilated spaces (c) H and E stain (×40): The dilated spaces are lined by endothelial cells, enclosing pale proteinaceous material, and few lymphocytes (d) Immunohistochemistry with Podoplanin (Clone D240, Pathnsitu) shows cytoplasmic expression in the endotheliumCutaneous lymphangioma is a rare and benign dilatation of lymphatic channels which account for 4% of the vascular tumors. Acquired lymphangiomas can be triggered by conditions which cause obstruction or damage to the lymphatic drainage such as surgery, postirradiation, chronic inflammatory conditions like Crohn’s disease, tuberculosis, and hidradenitis suppurativa. Radiation therapy is an approved treatment modality in the management of breast malignancy and an adjuvant treatment in postmastectomy cases. Radiotherapy leads to chronic inflammation and obstruction of the lymphatic channels, resulting in lymphedema. Breast cancer-related lymphedema is a known adverse effect of radiotherapy. Radiotherapy can also lead to various vascular proliferation, which may have a spontaneous occurrence or secondary to preexisting chronic lymphedema. Lesions which closely resemble lymphangiomatous lesions on the breast include cysts of fibrocystic disease, lymphocele, and hemangiomas. The French Sarcoma group has reported the occurrence of a variety of vascular lesions consequent to breast irradiation, which include hemangioma-like, lymphangioendothelioma lymphangiectasias, angiosarcomas, lymphangioma, and lymphangioma circumscriptum.[2] Diaz-Cascajo et al.[3] and Requena have proposed the nomenclature of benign lymphangiomatous papules after radiotherapy. Superficial cutaneous lymphangiomas are benign evolving lesions which are seen as well-circumscribed translucent lesions located usually in the superficial dermis or mid-dermis. Microscopically, lymphangiomas are composed of superficial lymphatic or vascular channels that are dilated and lined by a single layer of endothelial cells. Literature shows the occurrence of benign lymphangiomatous lesions in the irradiated skin [Table 1] with the time interval between the radiotherapy and occurrence of lesions varying from 4 to 12 years. In our case, lesions have occurred almost 3 years after irradiation.Table 1: Various case reports on post-irradiation - benign lymphangiomatous papulesThe treatment options for lymphangioma include surgical excision, electrodesiccation, sclerotherapy, and cryotherapy. The treatment is for cosmetic or functional reasons. Our patient presented with superficial cutaneous lymphangioma after undergoing a modified radical mastectomy followed by irradiation. In this situation, both the surgery and the irradiation have contributed to the onset of the lymphangiomas. It is reported for its rarity and may also be considered the first case of its type described in Indian literature to the best of our knowledge. Due to its rarity and unfamiliarity, this condition can be mistaken for cutaneous metastasis of breast malignancy by physicians. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.