Ductal eccrine carcinoma of the axilla: a diagnostic pitfall.

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. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

[Malignant cutaneous adnexal neoplasms of the face and scalp: Diagnostic and therapeutic update]. / Les tumeurs cutanées malignes à différentiation pilaire de la face et du cuir chevelu : mise au point diagnostique et thérapeutique.

INTRODUCTION: Malignant cutaneous adnexal neoplasms are rare and have been characterized only recently. They can occur at any age but preferentially in elderly. There are 3 of them: trichoblastic carcinoma, trichilemmal carcinoma and malignant pilomatricoma. The aim of our study was to make a diagnostic and therapeutic update about these tumors when located at the face or at the scalp. MATERIALS AND METHODS: A bibliographic research was made on PubMed using following keywords: appendage skin carcinoma AND pathology AND/OR therapeutic. Articles published before 2000 were considered outdated and were excluded. RESULTS: Twenty-five articles met the inclusion criteria. Clinical presentation was non-specific. Histological examination only allowed for diagnosis. Lesions were locally or loco-regionally aggressive. Lymphatic or hematogenous metastasis were reported. No consensus about treatment was found. When surgery was used, it consisted in resection with safety margins ranging from 0.5 to 3cm depending on the teams. In case of metastasis, treatment consisted in chemo- and/or radiotherapy. A quarterly medical monitoring was recommended. DISCUSSION: Malignant cutaneous adnexal tumors are rare. There is nowadays no treatment consensus. An initial staging by mean of a head and neck, chest, abdominal and pelvic CT-scan is mandatory. Treatment has to be decided in a multidisciplinary cancer committee. In the absence of metastasis, the reference treatment is surgical resection, possibly by Mohs micrographic technique, with large safety margins. In case of metastasis or if the loco-regional extension does not allow for a complete excision, chemotherapy and/or radiotherapy may be proposed. A close monitoring is essential.

Ductal eccrine carcinoma of the axilla: a diagnostic pitfall

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. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

Microcystic adnexal carcinoma: successful management of a large scalp lesion.

Microcystic adnexal carcinoma (MAC) is a rare, malignant cutaneous neoplasm. It is important for us to become more aware of it, as it is often misdiagnosed, either clinically or histopathologically. We report a case of an extensive scalp lesion that was successfully treated using a combination of resection and adjuvant radiotherapy.

Increasing incidence and survival of a rare skin cancer in the Netherlands. A population-based study of 2,220 cases of skin adnexal carcinoma.

BACKGROUNDS AND OBJECTIVES: The aim of this study was to describe the epidemiology and treatment of skin adnexal carcinoma (SAC) in the Netherlands and to identify prognostic factors for survival in patients with SAC. METHODS: We used population-based nationwide data from the Netherlands Cancer Registry with cases diagnosed during 1989-2010 and followed up to February 1st, 2012. RESULTS: A total of 2,220 SACs were diagnosed during 1989-2010 (age-standardized incidence rate 5.3 per million). Incidence increased by 2.7% and 1.7% annually in males and females, respectively. Fifteen different morphological types were registered. The 5-year relative survival rate increased from 80% in 1989-1994 to 91% in 2006-2010. The majority of all patients (91%) received surgery. Adjuvant radiotherapy and/or lymph node dissection was performed in only a minority of cases. The risk of death was significantly higher in patients who did not receive surgery. CONCLUSIONS: The rising incidence of SAC together with the predilection for the head and neck region suggests a role for UV radiation in the carcinogenesis of SAC. Furthermore, we found an improved survival of SAC in the Netherlands between 1989 and 2010. In view of the low proportion of patients receiving adjuvant therapy there may be further room for improving survival.

Nonmelanoma skin cancer.

Nonmelanoma skin cancer (NMSC) is the most common form of malignancy in humans. The incidence of NMSC continues to increase despite increased awareness and sun-protective measures. If neglected or mismanaged, NMSC can cause significant morbidity and even death. The most common forms of NMSC on the head and neck include basal cell carcinoma, squamous cell carcinoma, sebaceous carcinoma, eccrine porocarcinoma, Merkel cell carcinoma, atypical fibroxanthoma, and microcystic adnexal carcinoma. Surgery is the mainstay of treatment (standard excision, Mohs micrographic surgery, curettage); however, other modalities exist, including radiation, topical immunomodulators, photodynamic therapy, and new systemic medications.

Uncommon cutaneous neoplasms of the head and neck.

This article concentrates on the less-common cutaneous malignancies such as merkel cell, atypical fibroxanthoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, microcystic adnexal carcinoma, and sebaceous carcinoma. The clinical and histopathologic descriptions of each, most current and emerging etiologies, diagnosis, staging, treatment, and prognosis are discussed.

Microcystic adnexal carcinoma: a diagnostic and therapeutic challenge.

Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm that is often diagnosed after having been present for a significant period of time. It appears bland on histologic evaluation despite its locally aggressive behavior. Actual skin involvement is significantly more extensive than can be determined clinically and because of this, therapy is challenging. Though metastasis is rare, there have been reports of both regional and distant metastatic disease. Several treatment modalities have been used to date, including standard excision (SE), Mohs micrographic surgery (MMS), irradiation, chemotherapy, and observation. There has also been discussion in the literature regarding techniques than can aid in assurance of clear margins with MMS. We review the literature on MAC, including the various therapeutic options, addressing when one modality may be preferable over others. In general, MMS offers the highest likelihood of clear margins and cure with the fewest procedures.

Brooke-Spiegler syndrome: report of a case of multiple cylindromas and trichoepitheliomas.

We report a case of Brooke-Spiegler syndrome, a rare autosomal dominant disorder caused by mutations of the cylindromatosis gene (CYLD) tumor suppressor gene resulting in multiple cylindromas and trichoepitheliomas. Treatment of these patients can involve surgical excision, laser therapy, or topical applications of aspirin derivatives.

Multiple microcystic adnexal carcinomas.

Microcystic adnexal carcinoma (MAC) is a relatively uncommon adnexal neoplasm that can demonstrate locally aggressive behavior; rare instances of metastatic lesions have been reported. We report a case of a 34-year-old black man with multiple primary MACs.

Sweat gland carcinoma with lung metastases.

Sweat gland carcinoma is a rare skin tumor. The tumor has propensity to spread to lymph nodes and distant metastases has been reported. Their exact incidence in the Indian setting is not known. Aspects related to treatment are also not clearly defined. Though surgery forms the initial treatment approach, adjuvant treatment has not been properly explored. We report here a case of sweat gland carcinoma with bilateral lung metastases.

[Microcystic adnexal carcinoma (malignant syringoma) of the nose: case report and review of the literature]. / Malignes Syringom der Nase: Fallvorstellung und Literaturübersicht.

A case of Malignant Syringoma (syn. = Microcystic Adnexal Carcinoma, Sclerosing Swat Duct Carcinoma) of the nose in a 44 year old female patient is reported. The tumor had been misdiagnosed as a Rhinophyma some 20 years before and had thus been treated under cosmetic aspects. Besides the appearance, the patient did not suffer any complaints and was referred to the ENT-department of the Klinikum Hannover for cosmetic reasons. Histopathological examination after removal revealed a malignant syringoma, which, due to its extensive size and subepithelial growth pattern, made a complete ablation of the entire nose and the adjacent soft tissue of the face necessary. Pathohistologically cellular atypia, invasive growth pattern, perineural and perivascular infiltration was characteristic. Quantitative DNA anaylsis revealed a tumor with a diploid stem line and only few aneuploid cells. Malignant syringoma is a rare differential diagnosis of face skin tumors. The present case is discussed based on a review of the literature.

Sclerosing sweat duct carcinoma of the orbit.

Sclerosing sweat duct carcinoma or microcystic adnexal carcinoma is a rare, aggressive and locally destructive sweat gland malignancy. It is difficult to diagnose clinically and histologically and as such is probably under-recognized. Late recognition is common and is associated with extensive tissue loss. Local extension into the orbit from periorbital cutaneous lesions is documented, necessitating exenteration for adequate tumor clearance. We report a case of orbital sclerosing sweat duct carcinoma without a clinically evident cutaneous lesion, which required exenteration in a patient with no identifiable risk factors. Inclusion of sclerosing sweat duct carcinoma in the clinical and histological differential diagnosis of orbital masses and reporting of cases will lead to increased recognition of this malignancy, add to our understanding of its natural history and improve patient outcomes.

Squamous cell and adnexal carcinomas of the skin.

The diagnosis and treatment of adnexal cancers continues to pose a challenge to a wide range of clinicians. The diseases are a diverse lot, owing to the wide range of skin structures and large surface area. Early recognition and treatment are key to improved outcomes. Education of the patients as to their role in their care, especially early detection, is also of crucial importance. Further study may yield information to improve diagnosis and treatment.