Vulvar apocrine hidradenocarcinoma arising in a hidradenoma papilliferum-A case report.

Hidradenoma papilliferum (HP) is a benign adnexal neoplasm of the vulva that typically presents as a unilateral, flesh-colored papule in the labium majus in middle-aged Caucasian women. It is considered to be a close counterpart of the intraductal papilloma of the breast. Malignant transformation is rare with few reports in the literature. We present a case of vulvar mammary-type apocrine hidradenocarcinoma arising in an HP.

Matrical carcinoma with melanocytic hyperplasia mimicking nodular melanoma in an elderly Mexican male.

Matrical carcinoma with melanocytic hyperplasia (MCMH), previously referred to as malignant melanocytic matricoma, is a rare variant of the uncommon pilomatrical carcinoma, occurring most often on the head/neck and upper backs of middle-aged men. Nodular lesions may resemble pigmented basal cell carcinoma or melanoma clinically. We present a case of MCMH in a Hispanic patient with history of melanoma. Histopathological clues to appropriate diagnosis include basaloid cells, numerous atypical mitotic figures, matrical differentiation, shadow cells, strong diffuse nuclear and cytoplasmic expression of ß-catenin, and interspersed pigmented dendritic melanocytes.

Squamoid Eccrine Ductal Carcinoma: An Aggressive Mimicker of Squamous Cell Carcinoma.

Squamoid eccrine ductal carcinoma (SEDC) is an uncommon cutaneous adnexal malignancy that has the potential for an aggressive clinical course. The authors present a case of SEDC that resulted in widespread metastases and death. The clinical, histological, and immunohistochemical features of SEDC and several entities on the differential diagnosis are reviewed.

p63 and smooth muscle actin expression in low-grade spiradenocarcinomas in a case of CYLD cutaneous syndrome.

Low-grade spiradenocarcinoma is a rare skin tumor, with fewer than six reported cases, arising in the context of CYLD cutaneous syndrome (CCS; syn: Brooke-Spiegler syndrome [BSS]). We report two independent cases of spiradenocarcinoma arising in a 50-year-old man with CCS. The tumors grew rapidly, prompting clinical excision. The histologic features in our cases corresponded to the salivary gland type basal cell adenocarcinoma-like pattern, low grade (BCAC-LG), that has been previously recognized as a recurrent finding in CCS. We performed genetic testing of the patient and found a novel mutation in CYLD. Recognition of low-grade spiradenocarcinoma can be difficult for the pathologist as the features can be subtle, especially in those cases where the transition from benign to malignant tumor is gradual. We examined p63 and smooth muscle actin (SMA) expression patterns in BCAC-LG and compared it with the expression pattern in the precursor spiradenoma. Our report provides information on two rare tumors in the context of CCS and suggests that the pattern of p63 and SMA staining may aid pathological detection of the BCAC-LG subtype of spiradenocarcinoma.

Microcystic Adnexal Carcinoma of the Nipple.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare infiltrative cutaneous tumor, but with its locally aggressive nature, high rate of recurrence, and rare metastatic potential, it is an important clinical entity to consider when faced with a nipple lesion. These tumors are frequently misdiagnosed based on superficial biopsy alone. CASE REPORT: This case of a 15-year-old girl with MAC of the nipple demonstrates the importance of having a high index of suspicion in order to obtain a diagnosis, to determine the extent of disease, and to achieve adequate resection before reconstruction. Close clinical observation is recommended due to the possibility of future recurrences. CONCLUSIONS: Between the confusing nomenclature, indolent course, and inadequacy of superficial biopsy to achieve an accurate diagnosis, MAC is a clinical entity that the plastic surgeon must be aware of in order to facilitate proper diagnosis and treatment.

Utility of Immunohistochemistry in Distinguishing Primary Adnexal Carcinoma From Metastatic Breast Carcinoma to Skin and Squamous Cell Carcinoma.

The distinction between primary adnexal carcinoma (PAC) from metastatic breast carcinoma (BrCa) to skin and squamous cell carcinoma (SCC), particularly those with ductal differentiation (SCCDD), can be quite challenging, despite adequate history. The aim of the study was to determine the utility of an immunohistochemistry (IHC) panel to differentiate these entities and apply them to ambiguous tumors. Twenty-seven PAC, 7 metastatic BrCa, 28 SCC, and 16 ambiguous cases (SCCDD vs. PAC, n = 13 and metastatic BrCa vs. PAC, n = 3) were analyzed using CD23, PAX5, D2-40, P63, and CD117 immunohistochemistry. A total of 9 (33%) PAC were CD117 positive, whereas all metastatic BrCa and SCC were negative (P = 0.0002). D2-40 was expressed in 16 (59%) PAC and 16 (57%) SCC cases, but none of the metastatic BrCa cases (P = 0.0041). Of the 13 ambiguous tumors with a differential diagnosis of SCCDD versus PAC, all were positive for P63, 10 were positive for D2-40, and 1 was positive for CD117. Of the 3 ambiguous tumors with a differential diagnosis of PAC versus metastatic BrCA, 2 were positive for CD117, whereas none showed reactivity for D2-40 or P63. All cases were negative for CD23 and PAX5. Our study indicates that CD117 reactivity favors a PAC with a sensitivity and specificity of 33% and 100%, respectively. D2-40 and P63 expression highlighted both PAC and SCC and seems to be useful in excluding metastatic BrCa with a sensitivity and specificity of 58% and 100%, and 98% and 100%, respectively. Despite previous reports, CD23 and PAX5 do not seem to be useful.

[Microcystic adnexal carcinoma: A case report]. / Carcinome annexiel microkystique : à propos d'une observation.

We report a case of microcystic adnexal carcinoma in a 80-year-old woman. This is a rare malignant adnexal cutaneous tumor with glandular and follicular differentiation, rare, often asymptomatic, with late diagnosis and slow growth, locally aggressive and rarely metastatic. The distinction with other benign and malignant skin tumors is difficult.

Polymorphous Sweat Gland Carcinoma: An Immunohistochemical and Molecular Study.

Polymorphous sweat gland carcinoma is an uncommon low-grade malignant adnexal tumor with a marked predilection for the distal extremities. Histologically, the lesions are characterized by a cellular proliferation showing a combination of growth patterns, including trabecular, solid, tubular, cribriform, or adenoid cystic and pseudopapillary. The immunohistochemical and molecular profile of these tumors has not yet been properly addressed. We have studied 3 cases of polymorphous sweat gland carcinoma using a broad panel of immunohistochemical markers including cytokeratin AE1/AE3, CK5/6, MOC31, p40, p63, p16, chromogranin, synaptophysin, CD56, MIB-1, estrogen receptor, progesterone receptor, androgen receptor, BER-EP4, smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, CD117, S100 protein, HBME-1, DOG1, vimentin, and mammaglobin. We also examined for the MYB-NFIB fusion by fluorescent in situ hybridization (ISH) and for human papilloma virus by ISH. Our studies show that cytokeratin AE1/AE3, CK5/6, p40, p63, p16, chromogranin, and CD56 stains were positive in all 3 cases. All 3 cases were negative for MYB-NFIB fusion by fluorescent ISH which rules out adenoid cystic carcinoma. DNA ISH studies for high-risk human papilloma virus were negative in all cases. MIB-1 proliferation index was very high (30%-70% nuclear positivity), supporting a malignant phenotype. The positivity for chromogranin and CD56 suggests partial neuroendocrine differentiation. The differential diagnosis includes metastases from internal malignancies, basal cell carcinoma, and other benign and malignant adnexal neoplasms such as adenoid cystic carcinoma, ductal eccrine carcinoma, and microcystic carcinoma. Positivity for p16 in combination with chromogranin and CD56 may be potentially good markers for differentiating this tumor from other adnexal tumors.

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.

An Aggressive Microcystic Adnexal Carcinoma Infiltrating the Sternum.

Microcystic adnexal carcinoma is a rare cutaneous tumor that is often misdiagnosed and has the potential to be aggressive. Mohs surgery is the treatment of choice to prevent recurrences. We present a case of a large recurrent microcystic adnexal carcinoma on the sternum, initially diagnosed as a basal cell carcinoma. This tumor infiltrated the muscle and bone and was unresectable with Mohs surgery.

J Drugs Dermatol. 2017;16(2):180-181.

[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.

Melanocytic Matrical Carcinoma Mimicking Melanoma.

Melanocytic matrical carcinoma is a very uncommon malignant neoplasm with follicular differentiation. The lesion often presents as a solitary nodule on sun-exposed skin of elderly patients. Histopathologically, it is characterized by proliferation of atypical matrical cells admixed with an increased number of melanocytes, and some atypical matrical cells may have cytoplasmic pigment. When melanocytic proliferation or melanin pigment is prominent, melanocytic metrical carcinoma can mimic melanoma. Here, we present a novel case of melanocytic matrical carcinoma mimicking melanoma in an 80-year-old man with our findings in histopathologic and immunohistochemical study.