Recurrent primary cutaneous adenoid cystic carcinoma of the scrotum

Abstract

A 60‐year‐old man had asymptomatic erythematous nodule on the right inguinal area, which had progressively grown for 2 years. Excision was performed at the Department of Urology of our hospital. Two months later, there was local recurrence on the excision site, and it extended locally to make several tender erythematous nodules on the right inguinal area and firm plaque on the scrotum (Fig. 1). Histopathologic examination of the excision specimen revealed large cell masses with tubular or cribriform pattern and many small, solid epithelial islands unattached to the overlying epidermis. The tumor was confined to the dermis and subcutaneous tissue. Round spaces formed by malignant epithelial cells and containing amorphophilic basement membrane‐like material occured inside the tumor masses. The tumor cells were basaloid cells with scanty cytoplasm and deeply stained round‐to‐oval nucleus (Fig. 2). There was clear perineural involvement by the tumor cells. A substance in the pseudoglandular spaces, cystic spaces and the stroma was stained strongly with periodic acid‐Schiff (PAS) and alcian blue (pH 2.5). A PAS‐positive substance was digested by diastase pretreatment. These findings suggested the substance to be a neutral mucopolysaccharide. Immunoperoxidase staining for S‐100 protein and cytokeratin were strongly positive with the tumor cells but not with carcinoembryonic antigen. A histopathologic diagnosis of ACC was made. Salivary glands, bronchi, esophagus, and other organs were examined, but there was no evidence of neoplasm in other organs. A diagnosis of primary cutaneous adenoid cystic carcinoma was made but the patient refused all the treatment, including surgery. 1 Several erythematous nodules on the right inguinal area and indurated plaque on the adjacent scrotum 2 The characteristic tubular and cribriform pattern of basaloid cells with round spaces containing mucinous material in the dermis (H&E, × 400)

Primary cutaneous adenoid cystic carcinoma with MYB aberrations: report of three cases and comprehensive review of the literature

“…The PubMed‐based search revealed a total of 114 PCACC published cases the majority of which were part of case reports, with only a couple of small series and four relatively large series first authored by Seab & Graham, Urso et al, Ramakrishnan et al and North et al, which included 10, 5, 27 and 19 PCACC cases, respectively. After including our three cases, complete clinical information was found in 116 cases with 1 case that was reported as part of an immunohistochemical study did not include any clinical data .…”

Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival, and associated cancers, 1976 to 2005

“…In a recent review including 50 cases of PCACC, the largest series reported in the English literature, mean age at diagnosis was 59 years, with a slight female predominance 9. Although information on race is not uniformly specified in clinical series, reports of PCACC have originated from institutions in North America, South America, Europe, and Asia 12-14, 16, 17, 19, 25, 28, 33-35…”

Malignant adnexal neoplasms

“…[167][168][169][170] The prototypic adenoid cystic carcinoma is a major or minor salivary gland tumor; accessory minor salivary gland-like tissue is seen in the lacrimal apparatus, the upper and lower respiratory tract and rarely in the breast, uterine cervix, thymus and prostate gland. As a primary cutaneous neoplasm, this tumor is rare, with an exceptional case seen in the head and neck, particularly the scalp, and also occasionally on the trunk.…”