Based on Immune Microenvironment and Genomic Status, Exploring Immunotherapy in Advanced Hidradenocarcinoma: A Retrospective Analysis.

There are no standard treatment guidelines for hidradenocarcinoma, and the immune microenvironment and genomic data are very limited. Thus, in this study the immune microenvironment and genomic indicators in hidradenocarcinoma was investigated, and immunotherapy for hidradenocarcinoma was initially explored. Forty-seven hidradenocarcinoma patients were retrospectively collected. Immunohistochemical staining was performed to identify CD3/CD8+ T cells and programmed death ligand-1 expression. In total, 89.4% and 10.6% of samples had Immunoscores of 0-25% and 25-70%. Tumour proportion score distribution was as follows: tumour proportion score < 1% in 72.4%, 1-5% in 17.0%, and > 5% in 10.6%. Combined positive score distribution was as follows: combined positive score < 1 in 63.8%, 1-5 in 14.9%, and > 5 in 21.3%. Next-generation sequencing revealed that TP53 (33%), PI3KCA (22%), and ERBB3 (22%) were the most frequently mutated genes. The PI3K-Akt signalling pathway, growth, and MAPK signalling pathways were significantly enriched. Five patients had a low TMB (< 10 muts/Mb), and 9 patients had MSS. Three patients treated with immune combined with chemotherapy achieved significant tumour regression, and the progression-free survival was 28.8 months. In conclusion, the hidradenocarcinoma immune microenvironment tends to be noninflammatory. Evidence-based targets for targeted therapy are lacking. Immunotherapy combined with chemotherapy may be better for most advanced hidradenocarcinoma patients with a noninflammatory microenvironment.

Cutaneous and mammary apocrine carcinomas have different immunoprofiles.

Often the distinction of cutaneous apocrine carcinoma from metastatic mammary apocrine carcinoma to the skin can be a diagnostic dilemma because both tumors share similar histologic features and have overlapping immunohistochemical profile. We compared the expression of adipophilin, cytokeratin 5/6, p63, GATA3, mammaglobin, androgen receptor, estrogen receptor, progesterone receptor, and HER2 by immunohistochemistry in 14 cutaneous apocrine carcinomas (11 primary tumors, 3 metastases) and 26 primary apocrine carcinomas of the breast. Whereas focal adipophilin staining was seen in 36% (5/14) of cutaneous apocrine carcinoma, strong and diffuse adipophilin staining was seen in 88% (22/25) of mammary apocrine carcinoma (P = .0013). Differences in estrogen receptor and progesterone receptor expression were also statistically significant (P = .018 and .043). Androgen receptor was strongly positive in all cutaneous and mammary cases. Although there was no significant difference in the frequency of expression of cytokeratin 5/6, p63, HER2, GATA3, and mammaglobin in cutaneous apocrine carcinoma versus mammary apocrine carcinoma, strong and diffuse cytokeratin 5/6 and/or mammaglobin expression were seen only in cutaneous apocrine carcinoma. In conclusion, cutaneous apocrine carcinoma is likely adipophilin- ER+ PR+/- HER2- and can exhibit strong and diffuse cytokeratin 5/6 and/or mammaglobin expression. On the contrary, a mammary apocrine carcinoma is likely adipophilin+ ER- PR- and often exhibit 3+ HER2 with corresponding HER2 gene amplification. A panel of adipophilin, ER, PR, HER2, cytokeratin 5/6, and mammaglobin may be helpful in distinguishing cutaneous apocrine carcinoma from mammary apocrine carcinoma.

Autoimmune acrosyringitis with ductal cysts: reclassification of case of eruptive syringoma.

Syringomas are architecturally complex tumors composed of small, cystically dilated segments of dermal eccrine duct. Syringomas typically form isolated flesh-colored periorbital papules, however, in a peculiar condition termed eruptive syringoma; scores develop simultaneously in near confluence over a large surface area. While traditionally regarded as a neoplasm, more recent observations indicate eruptive syringoma is a reactive proliferation secondary to autoimmune disruption of the acrosyringium. We present the case of a 44-year-old woman with eruptive syringoma of the labia majora and prominent lymphocytic inflammation in the acrosyringium. Immunohistochemical stains confirm that the infiltrate is composed of CD4+ and CD8+ T cells, without significant CD20 B cells or CD138 plasma cells. Sequential sections of the syringoma reveal a complex 3-dimensional architecture with functionally isolated cysts, not connected to adjacent cysts or ducts by a discernable epithelium. These findings support the conclusion that eruptive syringoma is a tortuous proliferation of dermal eccrine ducts and fibrous stroma secondary to autoimmune destruction of the acrosyringium. Conceptually, the disorganized expansion of an eccrine duct syringoma may be analogous to a peripheral nerve traumatic neuroma.

Eccrine porocarcinoma: a case with an obscure primary tumor diagnosed from lymph node metastasis.

Eccrine porocarcinoma (EPC), a rare malignant epidermal appendage tumor, is mainly seen in elderly patients. A long history is one of its main characteristics. Two types of EPC are known: juxtaepidermal and dermal. The juxtaepidermal type usually has a more aggressive behavior. Lymph node metastasis and high mitotic activity are associated with poor prognosis. A case of EPC with a long-standing but indolent primary site diagnosed from lymph node metastasis is presented here. There was also accompanying chronic dermatitis as a paraneoplastic syndrome. Diagnosing EPC prior to the regional lymph node involvement is the most valuable factor for a successful treatment. Persistent examinations and attempts to find the primary site(s) have to be made in such cases.

Multiple eccrine poromas in the setting of total body irradiation and immunosuppression.

Eccrine poromas are benign, adnexal tumors that most often occur as a solitary lesion on the palm or sole. The occurrence of multiple eccrine poromas is extremely rare. In this report, we describe the development of several eccrine poromas in an acral distribution in a 42-year-old man. Before the appearance of these tumors, the patient had received total body irradiation and allogeneic bone marrow transplantation for treatment of acute lymphocytic leukemia. As a complication of the bone marrow transplant, the patient developed chronic graft-versus-host disease, which was treated with immunosuppressive therapy. We discuss this patient and review the available literature regarding multiple eccrine poromas.

Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases.

There is uncertainty about the exact nosological relationship between mycosis fungoides, follicular mucinosis, syringolymphoid hyperplasia with alopecia (SLHA) and syringotropic cutaneous T-cell lymphoma (CTCL). We report the clinical, histological, immunophenotypic and genotypic characteristics of a series of five patients (three men and two women) with syringotropic CTCL. We also review the 15 cases of SLHA previously reported in the literature. We conclude that syringotropic CTCL is a distinct clinicopathological variant of mycosis fungoides which may present on its own with characteristic punctate erythema or more commonly in association with folliculotropic lesions. Syringotropic CTCL is characterized histologically by infiltration of sweat glands by atypical lymphocytes in association with syringolymphoid hyperplasia. Cases of SLHA represent a syringotropic form of CTCL in association with follicular involvement, and such cases need to be investigated using T-cell receptor gene analysis of both skin and blood. Only limited conclusions on prognosis can be derived from our preliminary data. However, a review of the literature suggests that the prognosis does not differ significantly from other types of mycosis fungoides of equivalent stage.

Clear cell eccrine porocarcinoma with extensive cutaneous metastasis and peripheral lymphocyte dysfunction.

An 81-year-old woman came to our clinic (Department of Dermatology, Kaohsiung Medical University, Kaohsiung, Taiwan) with multiple erythematous, indurated papules over the left side of her face. Two years earlier, the patient had a skin biopsy done at a similar anatomical area with the histopathological diagnosis of Bowen's disease. After reviewing surgical specimens and confirming no systemic involvement, clear cell eccrine porocarcinoma with extensive cutaneous metastasis has been diagnosed. In addition, the peripheral blood lymphocyte function of the patient was evaluated. The expression of interleukin-2 receptors on lymphocytes after stimulation is abnormal compared with the age-matched normal control and a patient with cutaneous squamous cell carcinoma. This clinical manifestation of eccrine porocarcinoma is exceptional, and lymphocyte dysfunction in this patient has probably contributed to extensive cutaneous metastasis.

Vulvar syringoma: a clinicopathologic and immunohistologic study of 18 patients and results of treatment.

BACKGROUND: Syringoma of the vulva has been rarely reported. No effective treatment modality has been documented. OBJECTIVE: The purpose of this study was to describe the clinicopathologic features of vulvar syringoma, to investigate the hormonal influence on its growth, and to establish an effective treatment modality. METHODS: A total of 18 cases of vulvar syringoma were selected from the surgical pathologic file at Chang Gung Memorial Hospital. In all, 15 formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for estrogen receptor and progesterone receptor. RESULTS: The age of first presentation at our vulvar clinic ranged from 21 to 60 years with a median age of 29.5 years. Of patients, 13 (72%) had vulvar pruritus and 7 noticed aggravation during summer or during menstruation. The most common clinical appearance was multiple flesh-colored or brownish papules on bilateral sides of the vulva (9 of 18). One third of our series was found to have coexisting eyelid syringoma and 4 of them also had a family history of periorbital syringoma. Immunohistochemical stainings for estrogen receptor and progesterone receptor were all negative on the 15 cases studied. Of our patients, 7 with intense pruritus were treated with carbon dioxide laser vaporization. Their lesions resolved and pruritus subsided. CONCLUSION: Vulvar syringoma is not very rare and should be considered in the differential diagnosis of vulvar pruritus. In our study, estrogen receptor and progesterone receptor were not detected in vulvar syringoma. Carbon dioxide laser was an effective therapeutic modality in treating patients with intractable symptoms.

Espiroadenoma ecrino maligno / Malignant eccrine spiradenoma

El espiroadenoma ecrino maligno es un tumor raro que se desarrolla generalmente sobre un espiroadenoma ecrino preexistente de muchos años de evolución, en el que se observa un rápido crecimiento con ulceración y dolor. Existen unos 31 casos descritos en la literatura, de los cuales varios de ellos fallecieron poco tiempo después del diagnóstico debido a las metástasis ocasionadas a partir del tumor primario. Presentamos el caso de un paciente de 51 años que desarrolló un espiroadenoma ecrino maligno a partir de una lesión benigna de más de 20 años de evolución, localizada en región sacrococcígea

Espiroadenoma ecrino maligno / Malignant eccrine spiradenoma

El espiroadenoma ecrino maligno es un tumor raro que se desarrolla generalmente sobre un espiroadenoma ecrino preexistente de muchos años de evolución, en el que se observa un rápido crecimiento con ulceración y dolor. Existen unos 31 casos descritos en la literatura, de los cuales varios de ellos fallecieron poco tiempo después del diagnóstico debido a las metástasis ocasionadas a partir del tumor primario. Presentamos el caso de un paciente de 51 años que desarrolló un espiroadenoma ecrino maligno a partir de una lesión benigna de más de 20 años de evolución, localizada en región sacrococcígea (AU)

CD44 distribution in sweat gland tumors suggests it has different functional roles in the various cell types.

CD44 is a polymorphic group of membrane glycoproteins with multiple functions that include cell adhesion. Since on normal sweat glands CD44 is expressed only in eccrine coil secretory cells, it has been considered as a possible marker of this type of differentiation. We have immunohistochemically investigated the distribution of CD44 in paraffin-embedded samples of 41 benign and malignant sweat gland tumors by using a monoclonal antibody directed against the standard isoform of CD44. CD44 was strongly expressed in epithelial cells at the peripheral row of syringomas and in cuticular areas of eccrine poromas. Apocrine tumors such as apocrine hidrocystoma, syringocystadenoma papilliferum, or hidradenoma papilliferum showed intense CD44 positivity in the portion of cells in contact with the neighboring stroma and focally on the luminal side of cells with apocrine secretion. Cylindromas and spiradenomas presented focal CD44 positivity, virtually limited to clear cells. Malignant neoplasms exhibited irregular CD44 staining, which was more intense in the less differentiated zones and tumors. Our results indicate that CD44 is not a useful marker for a specific form of sweat gland differentiation. Nevertheless, its characteristic patterns of distribution might reflect the variety of functional roles assumed by the different CD44 isoforms in each epithelial cell.

Malignant hidroacanthoma simplex. A case report and literature review.

A 73-year-old woman with a malignant hidroacanthoma simplex on her right lower limb is reported. The lesion was immunohistochemically studied. Carcino-embryonic antigen and epithelial membrane antigen were found in the luminal border of ductal structures within the aggregations of neoplastic cells. We review the literature on this subject.