Cases reported "Sweat Gland Neoplasms"

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1/21. Detection of human papillomavirus type 10 dna in eccrine syringofibroadenomatosis occurring in Clouston's syndrome.

    Syringofibroadenomatosis is often associated with an underlying condition such as diabetes mellitus or hidrotic ectodermal dysplasia. By reason of these associations, a reactive or hamartomatous cause is suspected. We report a case of a 71-year-old woman with Clouston's syndrome in whom progressive multiple palmoplantar syringofibroadenomas developed over a 10-year period. The syringofibroadenomas formed flat-topped papules simulating verruca plana; the widespread distribution and chronic progressive course resembled epidermodysplasia verruciformis. Contiguous with the syringofibroadenoma's characteristic epithelial-stromal proliferation were epidermal changes of verruca plana. Evidence of human papillomavirus (HPV) infection was verified by immunolabeling with antibodies to bovine papillomavirus type 1 and detection of HPV 10 viral dna by means of polymerase chain reaction. Rather than a hamartomatous process, these findings suggest that syringofibroadenomas occurring in the setting of Clouston's syndrome could represent an HPV-induced epithelial proliferation.
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2/21. Metastatic malignant acrospiroma of the hand.

    We present the case of a 37-year-old man with multiple pulmonary metastases of a primarily unknown primary tumour. Thorough revision of the medical history yielded that he had already passed three resections of a right palmar mass, which had been described as a benign tumour. Clinical examination showed a thickened scar with a suspicious palpable mass in the right hand. Excision of this scar and the tumour mass with histopathological examination now revealed a malignant acrospiroma. Resection of the pulmonary metastases histologically also confirmed a malignant acrospiroma. The following radical resection of the metacarpals II and III with the index and middle finger under the assumption of a wide compartment resection achieved tumour free margins and proved to be efficient with the patient being relapse free for 4 years from this operation. Although the prognosis of this tumour is generally unfavourable this particular case demonstrates the value of a radical surgical resection as the mainstay of treating such highly malignant sweat gland tumours of the hand. copyright Harcourt Publishers Limited.
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3/21. 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.
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4/21. 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."
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5/21. Eccrine mucinous metaplasia associated with an apocrine cystadenoma.

    Mucinous metaplasia occurs uncommonly in cutaneous pathology, usually at specialized anatomic locations (genitalia, palms, and soles) and within restricted pathologic contexts (inflammation and trauma). Here, we report a unique case of eccrine mucinous metaplasia associated with an apocrine cystadenoma. A 13-year-old girl had an asymptomatic, 4-mm nodule on the chest. Histopathology demonstrated a typical apocrine cystadenoma in the upper and middle dermis. Adjacent to this lesion was a cluster of coiled eccrine secretory glands, of which the inner layer was almost entirely replaced by benign-appearing cells containing abundant, non-sulfated acid mucopolysaccharides. At 10 months' follow up, there was no recurrence. Our case demonstrates that, very uncommonly, mucinous metaplasia may be associated with a pathogenetically separate, adjacent proliferative adenomatous lesion, in this instance, an apocrine cystadenoma.
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6/21. A case of Schopf-Schulz-Passarge syndrome.

    Schopf-Schulz-Passarge syndrome (SSPS) is a rare ectodermal dysplasia characterized by hypodontia, hypotrichosis, nail dystrophy, palmoplantar keratoderma, and periocular and eyelid margin apocrine hidrocystomas. Several other skin tumours have been described in association with this syndrome, in particular, multiple palmoplantar eccrine syringofibroadenoma (ESFA). We report a case of SSPS with diffuse palmoplantar hyperkeratosis, which was shown by histology and immunocytochemistry to be due to ESFA.
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7/21. 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.
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8/21. Eccrine syringofibroadenomatous hyperplasia in a patient with bullous pemphigoid: a case report and review of the literature.

    A 76-year-old man with multiple erythematous lesions on his palms and soles which appeared following bullous pemphigoid (BP) is the subject of this case report. The lesions were not raised above the normal skin level, and there were no nodules on the erythematous lesions. The lesions had the histologic appearance of eccrine syringofibroadenoma. This condition is considered to be not a true tumor but a hyperplasia of eccrine sweat ducts following recurrent subepidermal blister formation in BP.
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9/21. Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.

    In two brothers and their sister, ectodermal defects including hypodontia, onychodystrophia, trichodysplasia and palmoplantar keratosis were observed. In addition to the ectodermal dysplasia a diffuse palmoplantar eccrine hyperplasia was noted as well as tumours and cysts of eccrine origin. This seems to be the first report of familial occurrence of eccrine tumours.
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10/21. Apocrine hidrocystomas of the lids, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy. A new variant of ectodermal dysplasia.

    A fourth case of a newly recognized variant of ectodermal dysplasia is reported. This syndrome is characterized by bilateral apocrine hidrocystomas of the eyelid margins, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy. To our knowledge, this syndrome, with its striking ocular manifestations, has not been previously documented in the ophthalmic literature.
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