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.

Histiocytoid eccrine sweat gland carcinoma of the eyelid: report of a case.

This report describes the 12th documented case of histiocytoid, diffuse or signet-ring cell eccrine sweat gland carcinoma of the eyelid, a rare neoplasm that characteristically affects older men and mimics distant metastasis. Clinically, the patient was thought to have a colonic metastasis. Histologically, the neoplasm comprised sheets and cords of polygonal, eosinophilic cells, with dispersed larger, rounded single cells resembling histiocytes, and cells with cytoplasmic lumina or a signet-ring appearance. Occasional duct formation was also seen. The features were reminiscent of both the histiocytoid and lobular variants of breast carcinoma. Ultrastructurally, the presence of nonintestinal type microvilli with a length : width ratio in excess of 16 were consistent with an eccrine origin, but also raised the possibility of metastatic mesothelioma. However, a metastatic lesion was excluded by clinical and imaging investigations. There has been no recurrence 3 years after primary excision.

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.

Ultrastructural morphology of a middle ear ceruminoma.

The ultrastructural morphology of a ceruminous gland adenoma in the middle ear was examined electron microscopically. The epithelial tumor cells displayed apocrine caps, microvilli, cell junctions, secretory granules, vacuoles, lipid droplets and siderosomes, which are the characteristic ultrastructural features of apocrine glands. Concentric membranous bodies of the endoplasmic reticulum, phagocytic activity of the tumor cells, intracytoplasmic lumina, ciliated cells and also spiny collagen in the tumor stroma could be seen. The myoepithelial cells are an important tumor marker in the differential diagnosis between ceruminomas and adenomas of the middle ear. The ectopic origin in the modified apocrine ceruminous glands, the specific localization, the clinical features and the extremely rare occurrence of the ceruminoma makes this tumor a unique neoplastic entity.

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.

Hyaline cell-rich chondroid syringoma: case report and review of the literature.

Hyaline cell-rich chondroid syringoma (HCRCS) is a rare benign cutaneous neoplasm composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. To the best of our knowledge, only eight cases of this entity have been reported so far, and none of them was submitted to a large panel of myoepithelial markers. We report on a case of a previously healthy 29-year-old male patient who presented with a slowly enlarging flesh-colored nodule on the palmar aspect of the tenar region of his left hand, measuring 2 cm in maximum diameter. The nodule was "shelled-out" and submitted to light microscopy, immunohistochemistry, and ultrastructural examination. Histopathologic analysis disclosed a lobulated neoplasm composed of hyaline cells with plasmacytoid features showing ovoid nuclei, with occasional invaginations, finely granular chromatin, and discrete nucleoli; the cytoplasm was deeply eosinophilic with occasional dot-shaped paranuclear hyaline inclusions. On immunohistochemical evaluation, hyaline cells were strongly and diffusely positive for S-100 protein, vimentin, pan (CAM 5.2) and high molecular weight (34betaE12) cytokeratins; these cells were focally positive for GFAP, maspin, neuron-specific enolase, and cytokeratin 14. Alpha-smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, collagen IV, Gp100 (HMB-45), and p63 were negative in neoplastic hyaline cells. Ultrastructural analysis disclosed cells with ovoid nuclei showing occasional invaginations and nuclear pockets; the cytoplasm was rich in meshworks of non-bundling intermediate filaments and a variable amount of rough endoplasmic reticulum cisternae. Based on our findings and those previously reported, hyaline cells of HCRCS might posses an aberrant myoepithelial differentiation. Most importantly, pathologists need to be aware of the histologic and immunohistochemical features of HCRCS to avoid a misdiagnosis of highly malignant neoplams, such as malignant melanoma and extra-skeletal myxoid chondrosarcoma.

Apocrine adenocarcinoma in a golden hamster.

An apocrine adenocarcinoma was observed in the subcutis of the abdomen of golden hamster. Histologically, the tumor cells irregularly formed multiple layers of cysts and some detached cells were presented in the cystic space. PAS stain with alpha-amylase digestion revealed PAS-positive alpha-amylase-resistant granules in the cytoplasm. Immunohistochemically, cytokeratin was demonstrated in the tumor cells. By electron microscopy, the tumor cells had an oval nucleus with invagination, abundant cytoplasmic organelles and microvilli protruding into the intercellular spaces.

Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature.

Malignant chondroid syringoma, or mixed tumor of the skin, salivary gland type, is an uncommon neoplasm believed to originate in sweat glands. This neoplasm occurs mostly in women and is typically seen in the extremities and torso. A case of recurrent malignant chondroid syringoma of the right foot in a man aged 34 years is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology, immunohistochemistry, and transmission electron microscopy. The malignant chondroid syringoma showed microscopic dermal satellite tumor nodules. Immunohistochemical staining was positive for keratin and S100 and negative for actin and p53. Ki-67 showed <10% positive staining. Ultrastructurally, the neoplasm was composed of epithelial cells with tonofilaments, cell junctions, and electron-dense amorphous keratin-like substance in the intercellular spaces. No evidence of myoepithelial differentiation was noted. Given the tumoral size, acral location, and histologic findings, the neoplasm was classified as a malignant chondroid syringoma. After reviewing the literature, it became apparent that wide surgical excision, adjuvant radiation therapy as well as patient education are critical in facilitating long-term survival.

Clear cell syringoid carcinoma: an ultrastructural and immunohistochemical study.

Syringoid carcinoma (syringoid "eccrine" carcinoma or eccrine epithelioma) is a rare cutaneous tumor with some controversy regarding its correct definition. It may also be difficult to differentiate from its benign counterpart (syringoma), other adnexal carcinomas, and cutaneous metastasis from adenocarcinomas. We present a case of a syringoid carcinoma of the clear cell variant complemented with an immunohistochemical and ultrastructural study, the latter revealing cytoplasmic accumulation of glycogen and presence of intercellular and intracellular lumina in clear tumor cells, as well as diverse hallmarks of malignancy (i.e., perineural invasion, tumor necrosis, and deep invasion). Clear tumor cells showed cytoplasmic and membranous immunoreactivity to epithelial membrane antigen, carcinoembryonic antigen, keratins, and S-100. Our ultrastructural and immunohistochemical results support the ductal differentiation of the glycogen-filled clear cell tumor population.

Bone marrow relapse in primary mucinous carcinoma of skin.

Primary mucinous carcinoma of skin is a rare adnexal tumor arising from the eccrine sweat gland. The tumors grow slowly and have low rates of local recurrence and rare chances of distant metastasis. The authors report a 70-year-old man with primary mucinous skin carcinoma who had a relapse in bone marrow 19 months after initial treatment.

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.

Papillary eccrine adenoma: an ultrastructural and immunohistochemical study.

A 45-year-old African American man presented with a small, solitary, nonulcerated cutaneous lesion of the right thigh of many years duration, which was excised. Light microscopically, the lesion consisted of an oval, well-circumscribed, intradermal proliferation of tubules, which were often dilated and lined by a double layer of tumor cells with conspicuous intraluminal papillations. Ultrastructurally, there was evidence of both intradermal eccrine duct and eccrine secretory coil differentiation. Although the majority of tumor cells resembled cells of the intradermal eccrine duct, occasional myoepithelial tumor cells and rare tumor cells having secretory granules and resembling dark mucous cells were indicative of eccrine secretory coil differentiation. The positive immunohistochemical staining for S-100 protein, CEA, EMA, and vimentin supported these ultrastructural findings.

Atypical mixed tumor of the skin: histologic, immunohistochemical, and ultrastructural features in three cases and a review of the criteria for malignancy.

Three cases of cutaneous mixed tumor with atypical features are described. These biphasic tumors comprise cords and nests of often plasmacytoid cells with areas of tubule formation, set in a chondroid stroma. The tumor cells show immunohistochemical positivity for both CAM 5.2 and S-100 and ultrastructural features of myoepithelium. The atypical histological features of an infiltrative margin, satellite tumor nodules, and tumor necrosis, which were present in these cases, have been described in malignant chondroid syringoma; however, a review of the small number of previous case reports demonstrates that histological appearances did not always correspond with apparent malignant potential in this tumor. The term atypical mixed tumor is recommended for tumors in which there are histological features of malignancy, especially local invasion, without proven metastases. Complete excision of the tumor and careful follow-up are advised, as at present the malignant potential of these tumors cannot be reliably predicted from their histological appearance.

Small cell sweat gland carcinoma in childhood.

Sweat gland carcinomas are rare skin tumors that typically occur in older patients. The spectrum of their clinical and pathologic features is broad, and many different types of sweat gland carcinomas have been described, ranging from fairly indolent to highly aggressive neoplasms. We present two cases of sweat gland carcinoma with a predominant small cell morphology. Both tumors occurred in children. One lesion developed in an 8-year-old girl as an asymptomatic papule on her left forearm, which ultimately was evaluated using biopsy because of rapid growth and change in color. The other lesion occurred on the hand of a 12-year-old boy. Both tumors were pandermal and extended into fat. They were composed of monotonous cuboidal cells with scant cytoplasm that formed tubules and grew in anastomosing cords and trabeculae. The tumor cells were immunoreactive for cytokeratins but not for cytokeratin 20. Ultrastructural analysis (available in one case only) showed that the tumor cells lacked neurosecretory granules. This variant of sweat gland carcinoma needs to be distinguished from other small cell neoplasms of the skin, especially Merkel cell carcinoma, its closest mimic.

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.

Adenomyoepithelioma of the skin: a case report with immunohistochemical and ultrastructural observations.

AIMS: The histological, immunohistochemical and electron microscopic features of a primary adenomyoepithelioma of skin, a rare sweat gland tumour, are reported. METHODS AND RESULTS: The tumour occurred on the back of a 92-year-old woman. It was composed of well-formed tubules lined by epithelial cells surrounded by clear or spindled myoepithelial cells. Immunohistochemically, the epithelial cells exhibited strong cytokeratin (CAM5.2) and weak carcinoembryonic antigen positivity. The myoepithelial cells showed diffuse positivity for smooth muscle actin and focal positivity for S100 protein. Ultrastructurally, the myoepithelial cells contained myofilaments with focal densities and hemidesmosomes. They were limited by well-formed basal lamina. The tumour was associated with a small eccrine spiradenoma. CONCLUSION: We predict that the tumour will behave in a benign fashion. There is no evidence of recurrence or metastasis 28 months later.

Apocrine gland tumor of the eyelid in a dog.

A 13-year-old male Shetland Sheepdog with progressive exophthalmos had a neoplastic mass in the ocular adnexa. Histologically, this neoplasm was composed of duct-forming epithelial cells with decapitation secretion. Tumor cells invaded the globe through the tunica conjunctiva and replaced the vitreous body. The cornea, iris, ciliary body, and retina were extensively destroyed. Both the epithelial and spindle-shaped myoepithelial cells showed nuclear atypia and mitotic activity in the globe. The primary tumor was diagnosed as adenocarcinoma, probably originating from apocrine sweat glands of the eyelid, and the infiltrating intraocular neoplasm was diagnosed as a malignant mixed tumor.

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.