Immunohistochemical and electron microscopic studies of Langerhans cells in a case of multiple eccrine spiradenomas.

Multiple eccrine spiradenomas are rare. In the present study, a detailed investigation of eccrine spiradenoma was performed, focusing in particular on the presence of Langerhans cells (LCs) in the tumor, and their immunohistochemical and ultrastructural characterization. The patient was a woman in her mid-forties who underwent resection of two tumors of the head that were 2.0 and 0.7 cm in size. They were diagnosed as eccrine spiradenoma and were composed of small and large tumor cells with a dense fibrous capsule in the dermis. Immunohistochemically, staining by antibodies to cytokeratins (AE1/AE3, CAM5.2) and CK 5/6 was diffusely positive in all tumor cells, although not in intermingled LCs, which harbored interdigitated nuclei. The cytoplasm of LCs was positive for S-100 protein and CD1a, and their nuclei were also occasionally positive for S-100 protein. Antibody to epithelial membrane antigen was positive for the surface of both intracytoplasmic and true glandular lumina. Fine structural examination revealed the presence of LCs among the tumor cells, extending fine irregular processes among the tumor cells. Birbeck granules were clearly demonstrated in the cytoplasm of LCs. Other fine structural findings included intracytoplasmic lumina with microvilli on their surfaces in some tumor cells. In these examinations of eccrine spiradenoma, LCs, approximately 15/HPF in the tumor, were distinctly detected even at light microscopic level as negative for various types of cytokeratin stains, although they were positive for S-100 protein and CD1a, whereas on ultrastructural examination Birbeck granules were demonstrated in their cytoplasm. Determination of the significance of these LCs in eccrine spiradenoma requires further investigation of a larger number of cases.

'Nuclear grooves' in nodular hidradenoma: frequency and significance of an unrecognized histopatological feature.

BACKGROUND: Nodular hidradenoma is a distinctive sweat gland neoplasm. In addition to the well-known histological and cytological features, we hereby describe for the first time nuclear grooving as a useful morphological feature to aid in its diagnosis. METHODS: All cases were analyzed for anatomic location, size, age, sex and histology, with attention focused mainly on the nuclear features. A semiquantitative method was used to estimate the percentage of nuclear grooves in five consecutive high-power fields (x40), with confirmation on x100. Electron microscopy was also performed to confirm light microscopic observation. RESULTS: Totally, 34 cases of nodular hidradenoma were studied. All the tumors showed two types of cells. One type of cell contained clear cytoplasm with small, round nuclei. The second type of cell, which was the predominant type, had elongated nuclei. In 30 (88%) of the 34 cases, about 30% of these cells at x40 and x100 magnification showed nuclear grooving. Electron microscopy on these samples confirmed nuclear grooves. CONCLUSIONS: In this study, we reviewed 34 cases of nodular hidradenoma. In addition to the classical features, both histologically and cytologically, we describe for the first time the nuclear grooves as a frequent finding in these neoplasms. This finding was also confirmed by electron microscopy. We believe that this newly described histological feature will aid the practicing pathologist to make this important diagnosis.

A case of canine apocrine sweat gland adenoma, clear cell variant.

A cutaneous mass at the base of the retroauricular region of a 4-year-old, female Golden Retriever was examined pathologically. Histologically, the mass formed multiple nodules consisting of a proliferation of large clear cells with abundant cytoplasm. Mitotic figures among the neoplastic cells were very sparse. The large clear cells were intensely positive for cytokeratins (AE1/AE4, cytokeratin 8 and 18) and moderately positive for lysozyme and contained periodic acid-Schiff-positive granules in the cytoplasm. In addition, small flat cells lined the islands of neoplastic large clear cells, and these were strongly positive for alpha-smooth muscle actin and vimentin, and some were positive for cytokeratin (AE1/AE4), suggesting they were myoepithelial cells. No local recurrence or metastasis has been recognized during the 18 months since surgical excision. On the basis of these findings, the present tumor was diagnosed as apocrine sweat gland adenoma, clear cell variant. There have been few previous reports of canine apocrine adenomas showing a clear cell morphology.

An immunohistochemical and ultrastructural study of syringocystadenoma papilliferum.

BACKGROUND: Syringocystadenoma papilliferum is a benign hamartomatous tumour of the skin. The histogenesis of this tumour is still controversial. There have been few reports regarding immunohistochemical investigations using only a limited range of antibodies and ultrastructural studies on this rare tumour. OBJECTIVES: To elucidate the immunohistochemical and ultrastructural properties of this tumour. METHODS: We investigated the immunohistological patterns of 12 different anticytokeratin (CK) antibodies and several other markers in five cases of this tumour, comparing them with the patterns in adult sweat glands. One of these cases was also evaluated ultrastructurally. RESULTS: The luminal columnar cells of the tumour were mostly positive for CK7 and more than 70% were positive for CK19. These cells showed the heterogeneous expression of CK1/5/10/14, CK14 and CK5/8. These patterns were also observed in the luminal cells in the secretory or the ductal portion of the adult sweat glands. The basal cuboidal cells of the tumour almost constantly expressed CK1/5/10/14, CK5/8, CK14 and CK7 (except for one case), similar to the patterns of basal cells in the transitional portion and myoepithelial cells in the sweat glands. However, the basal tumour cells expressed CK19 and vimentin heterogeneously, and alpha-smooth muscle actin focally (three cases). Ultrastructurally, the constituent epithelial cells were mainly divided into three types: luminal cells, basal cells and clear cells. The luminal tumour cells bore features of the secretory or ductal luminal cells of sweat glands, although they were somewhat immature in appearance. The basal tumour cells were fundamentally basaloid in nature. The clear cells were undifferentiated or primitive in appearance, suggesting stem or progenitor cell properties. Transitional forms between the clear cells and the other two cell types were also identified. CONCLUSIONS: The tumour epithelium was composed of several cell types demonstrating various developmental stages from the primitive clear cells to the basal cells demonstrating a tendency to differentiate toward basal cells in the apocrine transitional portion or myoepithelial lineage, or luminal cells toward the ductal or secretory epithelium. These results support the classical concept that syringocystadenoma papilliferum is a hamartomatous tumour that arises from pluripotent cells.

Poroid hidradenoma. Report of a case with cytologic findings on fine needle aspiration.

BACKGROUND: Fine needle aspiration cytology (FNAC) can be used for establishing a diagnosis of cutaneous lesions, especially in cases with cyst formation. Poroid hidradenoma is eccrine neoplasm with both solid and cystic components. CASE: A 77-year-old female presented with a slightly elevated nodule in the skin on her left elbow. The tumor was well demarcated, 2.7 x 2.4 cm and soft, and overlying skin was slightly reddish. FNAC revealed two types of cell: one had abundant cytoplasm in which small to large, occasionally multinucleated nuclei with small but distinct nucleoli were evident. Chromatin was finely granular, and nuclear membrane was thin and almost smooth. Another type of cell had scanty cytoplasm and a round to oval nucleus with small but prominent nucleoli. Histologic diagnosis was poroid hidradenoma. CONCLUSION: FNAC can be useful for diagnosing intradermal cystic lesions before surgical resection.

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)

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

Malignant eccrine spiradenoma. Case report and review of the literature.

Malignant eccrine spiradenoma is an exceedingly rare tumor. A case of a 72-year-old women with this highly aggressive malignancy arising from a long-standing lower leg lesion is reported. Management during the course of disease included surgery, radiation therapy (RT), hyperthermic limb perfusion chemotherapy, and chemotherapy. The patient died of her disease, with widespread metastatic disease 20 months after the diagnosis. A review of the literature is presented, and treatment considerations are summarized.

Eccrine syringofibroadenoma surrounding a squamous cell carcinoma: a case report.

A 91-year-old man presented with a 9.0 x 7.0 cm exophytic mass on the dorsum of the right foot, surrounded by a scaling hyperkeratotic plaque-like lesion that had been present for many years. He had similar long-standing hyperkeratotic plaque-like lesions on both legs. Histopathologic examination of the exophytic mass revealed a well-differentiated squamous cell carcinoma surrounded by an eccrine syringofibroadenoma (ESFA). Histochemistry, immunohistochemistry and electron microscopy support this diagnosis. To our knowledge, this is the only reported case of ESFA being intimately associated with a malignant neoplasm.

Malignant eccrine spiradenoma with carcinomatous and sarcomatous elements.

A case of a 60 year old man with malignant eccrine spiradenoma involving the perineum is described. Areas of typical eccrine spiradenoma were admixed with carcinomatous and sarcomatous elements. Immunohistochemical and ultrastructural analysis revealed no evidence of epithelial differentiation in the sarcomatous areas. The tumour qualified for the designation carcinosarcoma arising in eccrine spiradenoma. The clinical course was aggressive with rapid development of nodal and pulmonary metastases.

Fine-needle aspiration of nodular hidradenoma: a case report.

A case of nodular hidradenoma presenting on the forearm of a 36 year old woman is reported. The diagnosis was made on fine-needle aspiration biopsy (FNAB). The cytologic features of the lesion are described. This is the first case to be diagnosed cytologically.

Eccrine syringofibroadenoma (Mascaro). An ultrastructural and immunohistochemical study.

Eccrine syringofibroadenoma is a rare benign skin tumor, which usually develops on the extremities of elderly persons. We performed immunohistochemical and ultrastructural studies of a typical case of eccrine syringofibroadenoma that developed on the left heel of a 58-year-old man. The tumor consisted of anastomosing thin epithelial strands connected to the epidermis. There were many ductal or cystic structures, and their luminal cells were strongly positive to antibodies against carcinoembryonic antigen and epithelial membrane antigen. Filagrin and involucrin immunoreactivities were also detected in some cells surrounding the ducts. Keratins K1 and K10, co-expressed in the peripheral cells of normal acrosyringia, were colocalized in small cell clusters. Ultrastructurally, intracellular duct formation characteristic of developing acrosyringia was observed. Tumor cells containing globular keratohyaline granules with various electron densities were seen around some ductal structures. In these areas, keratinization took place without lamellar granule formation or prominent cornified cell envelope assembly. These results suggest acrosyringial differentiation of this tumor.

Eccrine syringofibroadenoma. Report of a case and immunohistochemical study of keratin expression.

We report a 49-year-old woman with an eccrine syringofibroadenoma. An asymptomatic nodule appeared on the right heel, and subsequently enlarged gradually. Histological examination revealed anastomosing strands of pale epithelial cells, with occasional duct formation and mucinous fibrovascular stroma. There were two types of strands: thick and thin. Immunohistochemical study, using a panel of monoclonal antikeratin antibodies, revealed that the immunoreactivity differed between the two types of strands. The thick strands showed a staining pattern similar to the uppermost portion of the intradermal duct, and the thin strands showed a pattern similar to the other portion of the duct. This observation suggests that the thick and thin strands differentiate towards the uppermost portion and the remainder of the duct, respectively.

A case of ductal sweat gland carcinoma connected to syringocystadenoma papilliferum arising in nevus sebaceus.

We report a case of a tumor arising in the preauricular region in a 50-year-old woman. The histopathological findings revealed it to be a ductal sweat gland carcinoma connected to a syringocystadenoma papilliferum (SCAP) arising in a nevus sebaceus. Mucinous stroma, considered to be deposition of hyaluronic acid, was also observed in the ductal carcinoma portion. The immunohistochemical and ultrastructural findings in the ductal carcinoma were compared with those in the SCAP. The proliferating cell nuclear antigen labeling index of the cells in the ductal carcinoma was higher than that of those in the SCAP. Both the ductal sweat gland carcinoma and SCAP showed findings compatible with the ductal segment of a sweat gland.

Is tubular apocrine adenoma a distinct clinical entity?

A 75-year-old Japanese man developed a tubular apocrine adenoma (TAA) (tubulopapillary hidradenoma with apocrine differentiation, a rare skin tumor), within a long-standing organoid nevus on the parietal area of his scalp. Histologically, the tumor consisted of dilated ductlike areas with some atypism and apocrine glandlike areas surrounded by myoepithelial cells. The superficial part of the tumor was connected to the epidermis and showed some of the characteristics of syringocystadenoma papilliferum (SCAP). The close relationship between TAA, SCAP, and papillary eccrine adenoma (PEA) is discussed. According to 19 reported cases of TAA, SCAP might occur together with TAA when they are preceded by an organoid nevus, and they might represent a spectrum of disease. Although TAA and PEA may represent another spectrum (designated as tubulopapillary hidradenoma), the relationship to SCAP should be considered in understanding and diagnosing an intermediate case.

Fine needle aspiration cytology of chondroid syringoma and syringocystadenoma papilliferum. A report of two cases.

Fine needle aspiration cytology of one case each of chondroid syringoma and syringocystadenoma papilliferum with histopathologic confirmation are reported. The aspirate of chondroid syringoma was characterized by the presence of two distinct components, a mesenchymal element with a chondroid appearance and an epithelial component. The chondroid element predominated in the smears. The aspirate from syringocystadenoma papilliferum showed papillalike clusters and dyshesive, monomorphic epithelial cells in a background of inflammatory cells. The clinical diagnosis and differential cytodiagnoses are discussed.

Chondroid syringoma with hyaline cell change.

Four cases of chondroid syringoma containing large numbers of hyaline or plasmacytoid cells are described. Three cases occurred in the hand and one in the foot. Hyaline cells are commonly seen in mixed tumours and myoepitheliomas of salivary glands and rarely in chondroid syringomas. The hyaline-cell change in three of the cases initially caused diagnostic difficulties and the possibility of sarcoma was raised in two cases. In addition to the characteristic hyaline cells, the presence of tubulo-glandulo-ductal structures, benign squamous epithelium and myxochondroid stroma aided diagnosis. Immunohistochemically, the hyaline cells exhibited positivity for vimentin, cytokeratin, S-100 protein, carcino-embryonic antigen, focal glial fibrillary acidic protein (3 cases), neuron-specific enolase (3 cases) and focal alpha-smooth muscle actin (2 cases). Occasional cells were Ber EP4 positive (2 cases). In some cells, a striking peripheral ring-like positivity for cytokeratin and S-100 protein was noted. Ultrastructurally, desmosomes, varying numbers of tonofibrils and non-bundling intermediate filaments were seen. Scanty fine filaments with vague focal densities were detected in some cells. Our studies suggest that the hyaline cells represent modified epithelial as well as myoepithelial cells. One of our cases also exhibited collagenous spherulosis.

Eccrine syringofibroadenoma (Mascaro): an ultrastructural study.

To confirm the eccrine acrosyringeal differentiation of eccrine syringofibroadenoma (ESFA) and to elucidate the histogenesis of its angiofibrotic stroma, a case of ESFA from a 45-year-old man was examined by light and electron microscopy. Histologically, the parenchyma featured anastomosing, slender epithelial cords containing small cuboidal cells and occasional duct-like structures. The stroma had increased numbers of mast cells, increased capillaries with swollen endothelial cells, and prominent fibrosis. Ultrastructurally, the following findings were characteristic of ESFA: a) abundant glycogen particles in epithelial cells, b) numerous intracytoplasmic and extracellular spaces lined with microvilli, c) intraepithelial duct formation, consisting of microvilli, vesicles, rod-shaped dense bodies, multivesicular dense bodies, and peripheral network of tonofilaments, and d) large numbers of mast cells, closely associated with fibroblasts, surrounding increased numbers of capillaries containing swollen endothelial cells. These ultrastructural features support the acrosyringeal differentiation of ESFA. We hypothesize that mast cell hyperplasia and degranulation may play an important role in the formation of the angiofibrotic stroma.

Eccrine syringofibroadenomatosis: a clinical and histologic study and review of the literature.

A 56-year-old man had an 11-year history of a psoriasiform eruption of the palms, soles, and shins. An examination revealed well-demarcated patches and plaques of erythematous, fissured, and hyperkeratotic skin with focal erosions. There was no clinical evidence of ectodermal dysplasia. On histologic examination these lesions proved to be eccrine hamartomas that consisted of anastomosing cords and strands of cuboidal epithelial cells with well-formed ducts and a fibrovascular mucinous stroma. Eccrine ductal origin was indicated by histopathologic, histochemical, immunopathologic, and electron microscopic evaluation. These multiple palmoplantar eccrine hamartomas, unassociated with ectodermal dysplasia, represent a sporadic hamartomatous condition that is best designated as "eccrine syringofibroadenomatosis."

Multiple linear cylindromas.

We report a case of multiple cylindromas with a linear arrangement on the lower right extremity. The patient had more than 100 tumors, but the scalp was spared. Some tumors showed overlapping features with eccrine spiradenoma. We believe this is the first report of multiple linear cylindromas.