Author's Reply.

Sir,

I appreciate the interest shown[1] by the esteemed reader in the article titled “acquired lymphangiectasis following surgery and radiotherapy of breast cancer.” Published in esteemed journal, Indian Journal of Dermatology as E-case report.[2]

The issue of terminology raised by the esteemed reader is pertinent. The terminology used in the article is based on pathological changes consequent to surgery and/or radiotherapy. There is controversy regarding two different pathological changes; lymphangiectasia and benign vascular proliferations.

The origin of the papules in benign lymphangiomatous papules of the skin following radiotherapy is debatable, some observers think they are associated with disruption of the flow of lymphatic fluid with resultant dilation of the existing lymphatic ducts[3] whereas others believe that they are a reactive proliferation of lymphatic vessels after damage by either operation or radiation.[4] It is definite that there is damage to the lymphatics by surgery/radiotherapy but whether consequent dilation or proliferation of lymphatics leads to papule formation is uncertain. In such circumstances the usage of the term acquired lymphangictasia is not incorrect. Furthermore, histopatholiogically absence of plump endothelial cells protruding into the vascular lumen and absence of prominent nuclei of endothelial cells protruding into lumen of vessel in hobnail pattern and absence of dense lymphocytic infiltrate close to the dilated lymphatic vessels in the index case do not substantiate usage of the term benign angiomatous proliferation.[56] Hence, the term acquired lymphangiectasis was used aptly for the index case. Moreover, the term lymphangiomatous papules may be reserved when the above histopathological features are seen.

Regarding the development of angiosarcoma: Acquired lymphangiectasia patients with the chronic lymphedema following mastectomy and/or radiotherapy may develop angiosarcoma.[7]

These angiosarcomas are characterized immunohistochemically by expression and amplification of myelocytomatosis (MYC) gene, which differentiates post radiation angiosarcomas from benign vascular proliferations following radiotherapy.[8] In addition, immunohistochemical staining for MYC not only helps in the diagnosis but also helps in mapping the neoplasm.

It is clear that acquired lymphangiectasia and chronic lymphedema are the pathological process involved (following surgery and/or radiotherapy), which subsequently may develop angiosarcoma which is MYC expressive. Conversely, angiosarcoma from benign vascular proliferations (induced by radiation) is MYC nonexpressive. Lymphangiectasia and benign vascular proliferations are distinct pathological processes and usage of these terms should be based on histopathological findings.