Abstract:Ductal eccrine carcinoma (DEC) is a rare sweat gland carcinoma with ductular
differentiation. Clinically, it is characterized by a slowly growing, hardened
plaque or nodule predominantly located on the head and neck. Histologically, DEC
shares similar features to invasive breast carcinoma, thus causing great
diagnostic challenges. We report a 69-year-old woman who presented with a
hardened plaque on the axilla. A skin biopsy was performed and metastatic
invasive breast carcinoma could not be ruled out. Complet… Show more
“…Of these, the cerebral metastasis proved more overt clinically, resulting in significant symptom burden and requiring two operative decompressions and adjuvant stereotactic radiotherapy. In the previous literature on DEA, high rates of distant metastasis have been reported, occurring in 40–50% of cases [2, 4]. These figures are higher than, but generally comparable to, the prognostic data for other eccrine carcinomas and for MCATs more generally [6].…”
Section: Discussionmentioning
confidence: 68%
“…Eccrine carcinomas typically present as slow-growing nodules, plaques, or ulcerative lesions, with the most common sites of disease the being lower extremities, head and neck and trunk. DEA appears to have a predilection for the head and neck [2, 4]. The diagnosis of eccrine carcinoma relies on histopathological analysis of biopsy or excision specimens.…”
Section: Introductionmentioning
confidence: 99%
“…In addition, regional and distant metastasis rates as high as 30% have been reported for these tumours [6]. For DEA specifically, while data are limited, the prognosis appears particularly unfavourable, with a 70–80% risk of local recurrence and 40–50% risk of distant metastasis [2, 4].…”
Section: Introductionmentioning
confidence: 99%
“…This involves either wide local excision or Mohs micrographic surgery to achieve negative resection margins [5]. Regional lymph node dissection is recommended only if there is clinical suspicion of nodal disease [2]. Radiotherapy is often utilised as an adjunct to resection in high-risk cases such as large tumours, close resection margins, or lymphovascular or perineural invasion [5].…”
Eccrine carcinoma, a subtype of which is ductal eccrine adenocarcinoma (DEA), is a rare cutaneous malignancy. For metastatic eccrine carcinoma, there are very limited data to guide treatment. Conventional chemotherapy is of limited benefit and there is only a small body of evidence for the use of immunotherapy in non-DEA eccrine carcinomas. We report the first case of metastatic DEA treated with a multimodality approach including surgery, radiotherapy, and immunotherapy, with an excellent prolonged response to pembrolizumab, and provide a review of the literature on pathological and management aspects for this rare tumour subtype. A 60-year-old male with a history of pT1N0M0 left scalp DEA, managed 2 years prior with excision and adjuvant radiotherapy, represented with a symptomatic right pontine metastasis. Imaging demonstrated intracranial, pulmonary, and hilar disease; biopsy of the cranial and lung lesions showed metastatic adenocarcinoma, morphologically similar to the previously resected scalp DEA. The patient was treated with stereotactic resections of his pontine metastases and adjuvant cranial radiotherapy, then commenced on immunotherapy with pembrolizumab. The patient has completed 21 months of pembrolizumab with a significant radiological response of the pulmonary and hilar disease and nil evidence of intracranial recurrence or further metastases. In this case report, we provide the first evidence of efficacy of immunotherapy in metastatic DEA, demonstrating an excellent and prolonged response of metastatic DEA to pembrolizumab. Further research is required to better establish the role of immunotherapy within the management protocol for this uncommon but aggressive tumour subtype.
“…Of these, the cerebral metastasis proved more overt clinically, resulting in significant symptom burden and requiring two operative decompressions and adjuvant stereotactic radiotherapy. In the previous literature on DEA, high rates of distant metastasis have been reported, occurring in 40–50% of cases [2, 4]. These figures are higher than, but generally comparable to, the prognostic data for other eccrine carcinomas and for MCATs more generally [6].…”
Section: Discussionmentioning
confidence: 68%
“…Eccrine carcinomas typically present as slow-growing nodules, plaques, or ulcerative lesions, with the most common sites of disease the being lower extremities, head and neck and trunk. DEA appears to have a predilection for the head and neck [2, 4]. The diagnosis of eccrine carcinoma relies on histopathological analysis of biopsy or excision specimens.…”
Section: Introductionmentioning
confidence: 99%
“…In addition, regional and distant metastasis rates as high as 30% have been reported for these tumours [6]. For DEA specifically, while data are limited, the prognosis appears particularly unfavourable, with a 70–80% risk of local recurrence and 40–50% risk of distant metastasis [2, 4].…”
Section: Introductionmentioning
confidence: 99%
“…This involves either wide local excision or Mohs micrographic surgery to achieve negative resection margins [5]. Regional lymph node dissection is recommended only if there is clinical suspicion of nodal disease [2]. Radiotherapy is often utilised as an adjunct to resection in high-risk cases such as large tumours, close resection margins, or lymphovascular or perineural invasion [5].…”
Eccrine carcinoma, a subtype of which is ductal eccrine adenocarcinoma (DEA), is a rare cutaneous malignancy. For metastatic eccrine carcinoma, there are very limited data to guide treatment. Conventional chemotherapy is of limited benefit and there is only a small body of evidence for the use of immunotherapy in non-DEA eccrine carcinomas. We report the first case of metastatic DEA treated with a multimodality approach including surgery, radiotherapy, and immunotherapy, with an excellent prolonged response to pembrolizumab, and provide a review of the literature on pathological and management aspects for this rare tumour subtype. A 60-year-old male with a history of pT1N0M0 left scalp DEA, managed 2 years prior with excision and adjuvant radiotherapy, represented with a symptomatic right pontine metastasis. Imaging demonstrated intracranial, pulmonary, and hilar disease; biopsy of the cranial and lung lesions showed metastatic adenocarcinoma, morphologically similar to the previously resected scalp DEA. The patient was treated with stereotactic resections of his pontine metastases and adjuvant cranial radiotherapy, then commenced on immunotherapy with pembrolizumab. The patient has completed 21 months of pembrolizumab with a significant radiological response of the pulmonary and hilar disease and nil evidence of intracranial recurrence or further metastases. In this case report, we provide the first evidence of efficacy of immunotherapy in metastatic DEA, demonstrating an excellent and prolonged response of metastatic DEA to pembrolizumab. Further research is required to better establish the role of immunotherapy within the management protocol for this uncommon but aggressive tumour subtype.
“…Clinical benefit with tamoxifen in hormone receptor positive DEC has been documented. Regarding prognosis, local recurrence is common (up to 80%) while metastases are rare . Our patient underwent complete excision and 6 months of tamoxifen therapy, and after 9 months of follow‐up, there was no evidence of recurrence.…”
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.