Cases reported "Sweat Gland Neoplasms"

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1/27. Pigmented hidrocystoma of the eccrine secretory coil in the vulva: clinicopathologic, immunohistochemical and ultrastructural studies.

    A case of pigmented hidrocystoma of eccrine secretory coil is presented. A 47-year-old woman had developed a bluish black small nodule in the anterior portion of the labium minor a few years before entry. Microscopically, the cyst was lined by eosinophilic columnar epithelium with abundant brownish granules. There was a vague suggestion of decapitation secretion focally in the epithelial layer of cuboidal cells. This layer expressed distinct reactivity against CA19-9 with no reactivity for human milk fat globule-1 (HMFG-1). These features demonstrated that the cyst was not of apocrine nature but of eccrine derivation. In addition, positive immunoreaction for cytokeratin (CK)7, CK8 and CK19 defined the cyst as originating from the secretory coil of the sweat gland. Ultrastructurally, melanosomes in various stages were identified in most of the epithelial cells. These findings suggest that the present case was a hidrocystoma of eccrine secretory coil with abnormal melanin accumulation.
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2/27. Sweat gland carcinoma of the lips.

    Sweat gland carcinomas are extremely rare tumors with a fully malignant potential. Two cases of primary sweat gland carcinoma of the lips are presented. Because of the unique nature of this lesion, the histopathology and biologic behavior are discussed, and the pertinent literature is reviewed.
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3/27. Malignant eccrine spiradenoma: a case report and review of the literature.

    BACKGROUND: Eccrine spiradenoma is a well-differentiated benign tumor of the sweat glands. Malignant change arising within eccrine spiradenoma is rare. OBJECTIVE: We describe a patient with malignant eccrine spiradenoma exhibiting both carcinomatous and sarcomatous differentiation. methods: Case report and literature review. RESULTS: A 37-year-old woman noted enlargement of a left axillary tumor that had been present for 20 years. The tumor was resected and the specimen, measuring 3.0 cm x 1.5 cm, revealed an encapsulated benign eccrine spiradenoma as well as an undifferentiated carcinoma possessing both carcinomatous and sarcomatous components. A transition zone was evident between the benign eccrine spiradenoma and the undifferentiated carcinoma, suggesting that the latter had arisen from the benign tumor. The malignant areas consisted principally of undifferentiated carcinoma (70%), although squamous cell carcinoma (10%), adenocarcinoma (10%), and chondrosarcomatous (10%) components were also present. Numerous mitotic figures were noted within the areas of malignant change, suggesting that the tumor was aggressive in nature. The patient died of systemic metastases 7 months after diagnosis. CONCLUSION: Although eccrine spiradenomas are usually benign, they can, on rare occasions, undergo malignant transformation. This case report describes one such occurrence of malignant transformation of a benign eccrine spiradenoma that unfortunately resulted in the patient's death from systemic metastases 7 months after diagnosis.
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4/27. 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.
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5/27. Microcystic adnexal carcinoma of the breast: a very rare breast skin tumor.

    BACKGROUND: Microcystic adnexal carcinoma is a rare, slowly progressing, malignant tumor of sweat gland origin. OBJECTIVE: A case of microcystic adnexal carcinoma of the breast with a history of 20 years is presented. methods: On initial examination, the ulcerative cutaneous mass was fixed to the underlying breast tissue and chest wall. Both the macroscopic appearance of the tumor and its location suggested a glandular breast carcinoma. However, an incisional biopsy revealed the nature of the tumor as microcystic adnexal carcinoma of the breast skin. Following the diagnosis, a wide excision of the tumor was carried out for the final treatment. RESULTS: To our knowledge this is the first case of microcystic adnexal carcinoma arising in the breast skin presented in the literature. CONCLUSION: This case demonstrates that microcystic adnexal carcinoma can occur on the breast skin and should be treated with wide excision due to its locally aggressive behavior.
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6/27. Mohs micrographic surgery of a papillary eccrine adenoma.

    BACKGROUND: Papillary eccrine adenoma (PEA) is a rare benign sweat gland neoplasm first described by Rulon and Helwig in 1977. Although these lesions typically behave in a benign fashion, PEA's on the volar surfaces may demonstrate more aggressive biologic behavior. Additionally, aggressive digital papillary adenomas (ADPA) may histologically simulate peas and behave in a more malignant fashion. OBJECTIVE: To present a case report of a patient with an incompletely excised PEA that was successfully extirpated using Mohs micrographic surgery (MMS). methods: A 51-year-old black woman was evaluated for the treatment of an incompletely excised PEA located on the dorsum of her left hand at the base of the thumb. Mohs micrographic surgery was felt to be the ideal treatment choice because of incomplete prior resections, ill-defined clinical borders, the need for conservative surgical excision to preserve sensory and motor function of the left hand, and the previously reported more aggressive nature of this tumor when located on volar surfaces. The patient underwent a two-stage, six section micrographically controlled excision using the fresh tissue technique. RESULTS: Complete resection of the PEA without significant damage to neurovascular structures. CONCLUSION: This case demonstrates the increasingly important role MMS is playing in the surgical management of a wide variety of cutaneous tumors. To our knowledge, this is the first time MMS has been used in the resection of a PEA.
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7/27. 'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?

    BACKGROUND: Syringomas have traditionally been categorized as benign neoplasms of the eccrine gland ductal epithelium. However, the variety of clinical presentations reported in the literature and some cases recently observed by the authors cast doubt upon the neoplastic nature of eruptive syringomas. Our goal is to challenge the traditional notion that eruptive syringomas are neoplastic lesions. RESULTS: We observed two patients who presented with an eczematous process, which resolved leaving residual lesions. Biopsies of the late lesions showed features of eccrine syringoma. Yet a biopsy obtained from an incipient lesion in one of the cases showed a lymphocytic inflammatory reaction of the superficial portion of the eccrine duct resulting in tortuous hyperplastic changes. CONCLUSION: Based on our observations, some of the so-called 'eruptive syringoma' may represent a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. We proposed the term 'syringomatous dermatitis' for such cases.
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8/27. Eccrine angiomatous hamartoma: a multiple variant.

    A case of multiple eccrine angiomatous hamartoma present in a boy since birth is reported. Clinically, this condition must be differentiated from other neonatal angiomatoses. Sometimes the clinical findings are nonspecific, whereas histologic examination may exclude angiomatoses with visceral involvement. In our case the hamartomatous nature of this tumor is documented also by the presence of pilar structures intimately related to the eccrine-angiomatous complex in one of two lesions histologically examined. Therefore, the histologic classification of eccrine angiomatous hamartoma into subgroups seems to be excessive.
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9/27. High-frequency ultrasound imaging of periocular hidrocystomas.

    BACKGROUND: High-frequency ultrasound has improved our ability to manage and diagnose anterior segment tumors. Hidrocystomas are unusual cystic tumors that can affect the conjunctiva and eyelid. When they appear pigmented and rapid in onset, it is important to differentiate them from malignant tumors, such as melanoma. case reports: Three patients were referred to The new york eye Cancer Center for evaluation of pigmented lesions on their eyes. The tumors affected the conjunctiva or eyelid and were of recent onset ranging from 1 week to 6 months. The hidrocystomas displayed hypoechoic high-frequency ultrasound patterns, which corresponded to the cystic nature revealed through histopathological analysis. Differential diagnoses are discussed and include benign and malignant tumors. CONCLUSION: Cystic tumors are a common finding of the eye; however, pigmented hidrocystoma is an uncommon diagnosis. Although histopathological study is often required for definitive diagnosis of these lesions, much information can be gained through pre-surgical evaluation, particularly high-frequency ultrasound. Intra-lesional cyst formation was characteristic in these three tumors. Further study of the high-frequency ultrasound characteristics of pigmented eyelid and conjunctival tumors will help determine if these ultrasound characteristics are diagnostic.
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10/27. Milium-like syringoma: a case study on histogenesis.

    BACKGROUND: Milium-like syringoma is a variant of syringoma first described in 1987. Only few cases have been reported in the literature. It may be misleading clinically, and its histogenesis has not been clarified. CASE REPORT: We present a case of periorbital milium-like syringoma, with studies on the histopathologic, histochemical, and immunohistochemical features. RESULTS: histology showed a large keratin-filled cyst in the upper portion of the lesion approximating the epidermis. Serial sections revealed that the cyst connected with the underlying syringomatous epithelial strands. Melanin was absent in the wall of the cyst, as demonstrated by Fontana-Masson stain. Cytokeratin 7 was expressed neither in the milia nor in the solid epithelial parts. carcinoembryonic antigen (CEA) reactivity was seen in the luminal cells of the keratinous cysts. However, in the largest keratin-filled cyst clinically suggesting a milium, only the lower half of the cyst was positive for CEA. These results proved that the milia were part of syringoma with eccrine duct differentiation. Fusion of the upper half of the largest cyst with the epidermis may explain the absence of CEA positivity in this part, analogous with eccrine duct milia. review of the literature indicated that this variant of syringoma occurs more often in Asians. CONCLUSION: We present evidence, and propose the histogenesis of milium-like syringoma, that it is a variant of syringoma with a prominent cystic component showing features of eccrine duct milia. Recognition of its nature is of therapeutic significance. Further studies are required to verify its clinical characteristics as compared with ordinary syringomas.
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