Aggressive scalp and sternal lesion: A presentation of rare case of metastatic eccrine carcinoma

Abstract

Introduction Ductal Eccrine carcinoma (DEC) is a rare primary cutaneous tumor that exhibits both squamous and adnexal ductal differentiation. Due to its rarity in clinical practice we present as case of DEC and a literature review on the latest management of this rare disease. Case presentation We report a case 41 years old female presented with lesion on the scalp and sternal mass, increasing in size with itchiness and erythematous for 6 months duration. Further CECT scan of brain and neck shows features of malignant left frontal scalp lesion with poor plane with overlying skin and underlying skull bone and CECT of thorax shows a large, irregular heterogeneously enhancing mass with necrotic center noted at right hilar within superior segment of right lower lobe, encasing right middle and lower lobe bronchi. Wedge biopsy of scalp lesion showed an intradermal lesion extensively infiltrating by malignant gland accompanied by desmoplasia and the tumor cells are seen extending into the surgical margins suggestive of ductal eccrine carcinoma. Clinical Discussion:This case highlights the importance and challenges in achieving early diagnosis coupled with the scarcity of information on these leads to difficulty in managing this patient. Conclusion In managing Ductal Eccrine Carcinoma tumor, standard method of treatment for has not been established. However, wide surgical excision is the treatment of choice for localized lesions. Regarding prognosis, there is conflicting data published which we describe in this article.

Evaluating Rates of Positive Margins After Standard Excision of Cutaneous Adnexal Malignancies

“…9,29,30 Although eccrine adenocarcinoma and spiradenocarcinoma are very rare and limited data exist regarding their natural history and behavior, case reports have identified infiltrative growth patterns and perineural invasion in some of these tumors. [31][32][33] Positive surgical margins have several important clinical consequences. First, in cases where achieving a negative surgical margin is deemed necessary to treatment, positive margins can lead to the need for multiple excisions, leading to increases in cost of treatment, complexity of reconstruction, and burden to patients.…”

“…9,29,30 Although eccrine adenocarcinoma and spiradenocarcinoma are very rare and limited data exist regarding their natural history and behavior, case reports have identified infiltrative growth patterns and perineural invasion in some of these tumors. 31–33…”

Metastatic Ductal Eccrine Adenocarcinoma with Excellent Response to Immunotherapy

“…Eccrine carcinomas represent a subset of MCATs originating from eccrine glands, the most widely distributed sweat gland type. Eccrine carcinomas account for less than 0.01% of all cutaneous malignancies [1]. While the classification of eccrine carcinomas is complex and the nomenclature is often inconsistent, well-described subtypes include eccrine porocarcinoma, eccrine spiradenocarcinoma, and syringoid eccrine carcinoma [2].…”

“…Therefore, diagnosis is based primarily on morphological assessment. However, the diversity and rarity of eccrine carcinomas makes correct morphological classification challenging [1, 5]. In DEA, the appearance of marked ductal proliferation mimics metastatic adenocarcinoma from non-cutaneous sites such as the breast, further complicating the differential diagnosis [3].…”