Pediatric Segmental Eccrine Spiradenomas: A Case Report and Review of the Literature

Abstract: Eccrine spiradenoma is a rare benign adnexal tumor that usually occurs as a solitary nodule, although there are reports of multiple eccrine spiradenomas occurring in a segmental, linear, blaschkoid, or zosteriform pattern. Segmental eccrine spiradenomas have rarely been reported with onset occurring in childhood or adolescence. We describe a case of segmental eccrine spiradenomas occurring on the neck and mandible of a child.     

Multiple linear eccrine spiradenomas with eyelid involvement

Eccrine spiradenoma is an uncommon tumour of the sweat gland and usually occurs as a solitary small painful nodule. Very rarely, it may present as multiple tumours arranged in a linear/zosteriform fashion. We present the case of a 23-year-old female who had multiple spiradenomas arranged linearly from the midline of forehead to the left corner of the mouth and a few lesions also on the margin of the left eyelid. The lesions were probably along the Blaschko's lines. Tumours were excised surgically in stages. Literature search revealed only 13 reported cases of linear/zosteriform/naevoid multiple spiradenomas and only four cases of solitary spiradenoma of the eyelid. The review of reported cases suggests that arrangement of tumours in a linear/zosteriform pattern is an important diagnostic clue for multiple (naevoid) spiradenomas.     

Multiple vascular eccrine spiradenomas: a case report and published work review of multiple eccrine spiradenomas

Eccrine spiradenoma (ES) usually occurs as a solitary small nodule. It presents rarely as multifocal or multiple localized tumors arranged in a linear, zosteriform or nevoid distribution. We present a rare case of a 55-year-old woman who had a 48-year history of multiple vascular eccrine spiradenomas (VES) localized on the left side of the submandibular region and neck. All five tumors were skin-colored or pinkish-purple, and ranged in size 1.5-2.5 cm. Histologically, each tumor was composed of two characteristic cell types and many dilated vascular spaces were noted in the stroma. Contrast-enhanced computed tomography showed irregularly shaped, enhanced areas at the center of the tumors. A published work search revealed 35 cases of multiple ES, but multiple VES was extremely rare. We summarized the features of previously reported multiple ES and discuss the clinical and histological classification of ES.     

Multiple eccrine spiradenomas on the hand,forearm and head

The first case of multifocal eccrine spiradenomas on the hand, forearm and scalp is described. This case is unusual in that the tumors located on the little finger included the nail matrix and occurred in a linear/zosteriform distribution, resulting in a nail deformity. The nodules on the forearm and scalp were in a random distribution. Histologically, each tumor was highly cellular and composed of two cell types: small darkly staining basaloid cells and larger, pale cells. Ultrastructural observations often showed an intracellular lumen with numerous microvilli in larger pale-staining cells.     

Malignant eccrine spiradenoma. A clinicopathologic study.

Malignant eccrine spiradenomas (MES) are exceedingly rare and their immunohistochemical and ultrastructural features have not been fully characterized. We studied two cases, one of them immunohistochemically and electron microscopically. Patient 1 had a 25-year history of multiple exophytic tumors involving the scalp, the skin of the face, and the torso. Of the lesions removed, ten were spiradenomas, two with malignant changes, and three were cylindromas. The malignant areas showed loss of tubular and nesting patterns, lack of two cell populations, and contained anaplastic cells with high mitotic rate. The immunohistochemical findings were consistent with eccrine differentiation. Patient 2 had a cystlike mass of long duration in the right groin. Histologically, the mass consisted of nodules of benign eccrine spiradenomas adjacent to a ductal-cystic mass lined by anaplastic cells, but areas of squamous and glandular differentiation were also present. Conclusions: (a) Case 1 is probably the first reported MES associated with multiple spiradenomas and cylindromas. (b) Cytodifferentiation in MES is variable, sometimes with almost complete loss of eccrine differentiation. (c) Identification of adjacent spiradenomas may be required for definite diagnosis of MES. (d) Clinical history of longstanding lesions with recent fast growth warrants tissue diagnosis.     

Multiple eccrine spiradenomas in a linear nevoid pattern

A 16-year-old boy presenting with multiple slightly painful, bluish red papules and plaques on the left thorax is reported. The skin lesions were arranged in a linear and arched pattern following the lines of Blaschko. Clinical examination led to the suspicion of multiple leiomyomas, syringomas or glomus tumours. Histological examination revealed that the lesions were typical benign eccrine spiradenomas.     

Zylindrospiradenome bei Brooke-Spiegler-Syndrom

Brooke-Spiegler syndrome is a rare autosomal-dominant inherited disease, characterized by the development of multiple cylindromas and trichoepitheliomas. We report a case in which tumors were found on the head showing histological features of both cylindromas and spiradenomas. These changes were in continuity within the same lesions. Since cylindromas are thought to be apocrine in nature, whereas spiradenomas are eccrine, a contiguous growth within the same neoplasm indicates a conflict concerning their histogenesis. The available histological, ultrastructural and immunohistochemical studies have not produced any criteria to allow an unequivocal classification, suggesting instead that both tumors arise from similarly differentiated cells, perhaps from pluripotent epithelial cells. In our opinion, it seems impractical to strictly classify cylindromas and spiradenomas into tumors of apocrine or eccrine differentiation. Moreover, at least for those cases of Brooke-Spiegler syndrome, where both tumors are found in immediate vicinity to each other, we propose the term cylindrospiradenoma.     

MALIGNANT ECCRINE SPIRADENOMA ON THE LATERAL MARGIN OF NOSE AS AN INFREQUENT LOCALIZATION

Malignant eccrine spiradenomas are exceedingly rare tumors. They can arise from a preexisting eccrine spiradenoma or occur as a primary malignant tumor. Clinical features of these tumors may include a history of enlargement in a previously stable lesion. Tumor can be of low or high grade. Low-grade malignant eccrine spiradenoma has a better prognosis. We present a malignant eccrine spiradenoma arising from a preexisting eccrine spiradenoma, which has an infrequent localization between lateral edge of nose and medial canthus.     

Two cases of multiple eccrine spiradenoma with linear or localized formation

We report two rare cases of recurrent, multiple eccrine spiradenoma. Both cases presented with extensive lesions comprised of multiple red papules of various sizes and a soft blue-red nodule. The first case was a 30-year-old woman. Her lesions followed a linear arrangement on her chin, and extended down the right side of the neck with spontaneous pain. The second case was a 57-year-old woman with tumors in a localized group on the left occipital region without pain. A search of the literature revealed only 15 reported cases of linear/zosteriform/nevoid multiple eccrine spiradenoma. Both cases were treated by surgical excision. Most of the red papules displayed typical histological features including two cell types: large clear cells with low-density cytoplasm; and small dark cells with high-density cytoplasm. The large soft tumors exhibited a variable histological appearance. In the first case, the cystic tumors displayed an homogeneous structure comprised of eosinophilic material. In the second case, the cystic tumors included abundant interstitial tissue.     

Multiple Segmental Eccrine Spiradenoma with a Zosteriform Pattern: A Case Report and Literature Review

Eccrine spiradenoma usually occurs as solitary tender nodules. However, it rarely occurs as multifocal localized tumors or has zosteriform distribution. We report the case of a 32-year-old woman with a 20-year history of itchy lesions on the left side of the back and forearm with a zosteriform distribution. Before disease onset, almost no patients have inducing factors, but our patient received an injection beforehand; however, whether this was coincidental or causative remains unknown. The lesions became very itchy after perspiration or eating spicy food, which has never been reported. A literature search revealed 22 cases of multiple segmental eccrine spiradenoma; we summarized the clinical characteristic in order to aid diagnosis and treatment selection.     

Multiple linear eccrine spiradenoma associated with multiple trichoepithelioma

A 37-year-old woman had multiple trichoepithelioma of the face which showed typical clinical and histological features, together with multiple papular lesions of 30 years' duration. The lesions were arranged in a linear fashion on her right breast and right upper arm. Microscopic examination of a biopsy specimen revealed typical eccrine spiradenomas. An ultrastructural study of the tumor on the breast revealed that it was composed of two main types of cells: one was a clear cell with low-density cytoplasm and the other was a dark cell with high-density cytoplasm. Some of the clear cells had secretory granule-like structures and/or tono-fibrils, while others had intracytoplasmic cavitations. Neither myoepithelial cells nor Langerhans cells were observed.     

Immunohistochemical differentiation of four benign eccrine tumors

Background:  The histogenesis and differentiation of eccrine tumors, including cylindroma, poroma, spiradenoma and syringoma, remains controversial. This controversy may be because of sporadic and incomplete studies of these neoplasms.
Methods:  Ten examples each of normal eccrine structures and of four benign eccrine tumors are analyzed with antibodies to cytokeratin (CK) 7, CD34, CK6, CK10, smooth muscle actin (SMA) and CD10. These markers represent two different immunohistochemical stains for each part of the eccrine structure; CK7 and CD34 stain the secretory coil, CK6 and CK10 stain the straight duct and SMA and CD10 stain the myoepithelial cells. This redundancy in staining is performed on four benign eccrine tumors to better interpret the existing literature.
Results:  We find that CK7 is a sensitive marker for the secretory coil; both cylindromas and spiradenomas express CK7. We also find that CK6 is a marker for the inner ductal cells, while CK10 is a marker for the middle ductal cells; syringomas express both these markers. SMA appears to be a more specific marker for myoepithelial cells surrounding normal eccrine coils, and none of the studied tumors express SMA or CD10.
Conclusions:  Our studies suggest that syringomas are tumors of the eccrine duct, while cylindromas and spiradenomas are tumors of the secretory coil.     

Familial multiple eccrine spiradenomas with cylindromatous features associated with epithelioma adenoides cysticum of Brooke

Four cases of rare familial multiple eccrine spiradenomas showing features of dermal cylindromas and associated with epithelioma adenoides cysticum of Brooke are reported. Skin biopsy specimens were obtained from three generations of this family and routine histochemical and immunoperoxidase stains were used. The eldest affected family member had multiple disfiguring facial and scalp tumors, which precipitated episodes of depression. Unlike other cutaneous genetic disorders, such as neurofibromatosis and tuberous sclerosis, the cutaneous adnexal tumors occurring in these patients continue to erupt and grow during their lifetimes.     

呈带状分布的小汗腺螺旋腺瘤

报告1例多发性小汗腺螺旋腺瘤。患者女,23岁．右侧上肢紫红斑10余年,皮下结节7年,皮肤科检查示右侧躯干、上肢泛发米粒至黄豆大紫红色结节．呈带状排列,组织病理检查：真皮下部可见多个瘤细胞团块,瘤组织由2种细胞组成,一种细胞核大、淡染,位于细胞团块中央,另一种细胞核小、深染,排列在瘤细胞索周边。     

Eccrine porocarcinoma.

We describe a 78-year-old woman with eccrine porocarcinoma presenting a zosteriform appearance on the lateral upper part of the buttock. Erosion and bleeding were observed in the center of a 50 X 80 mm, erythematous indurated plaque with an elevated border. Posterior to the plaque, there was another erosive plaque, 50 X 20 mm in diameter, with peripheral induration overlaid by two reddish papules. Based on the previous reports of 21 patients with eccrine porocarcinoma in Japan, we discuss the relationship between the clinical pattern and lymph node metastasis. The pedunculated eccrine porocarcinoma has less tendency to metastasize to the regional lymph nodes than the nonpedunculated form.     

An Unusual Case of Congenital Linear Eccrine Spiradenoma

Abstract:   Eccrine spiradenoma (ES) is a benign uncommon tumor of skin adnexa with a characteristic clinical and histopathological presentation. Typically, it presents as a painful, slow growing and solitary nodule on the head or upper trunk in adult patients. We report a child with linear ES which presented with asymptomatic papulonodular lesions in a blaschkoid distribution on the face. Cases reported in the literature of multiple spiradenomas are very rare and multiple linear lesions are even rarer. To date, 21 cases of linear/multiple ES have been reported. Of these, eight were in children or adolescents. We report an additional case of this rare clinical presentation and review the literature.     

Multiple linear spiradenoma with a linear distribution

We report the case of a 32-year old woman who presented with multiple nodules in the left arm and breast that had been present for 15 years. These nodules had a bluish colour and were occasionally painful. She had gone under excision of three other nodules on the left arm with the histopathological diagnosis of spiradenoma. All of the nodules were disposed in a linear arrangement and underneath a maculo-papular erythematous lesion which clinically seemed to be an epidermal nevus. The case described in the literature of multiple spiradenomas are very rare and multiple linear spiradenomas are even rarer.     

Expression of Bcl-x in normal skin and benign cutaneous adnexal tumors

BACKGROUND: Bcl-x appears to have an antiapoptotic role in the epidermis. Little is known about the expression of Bcl-x in cutaneous adnexal structures and benign cutaneous adnexal tumors. METHODS: Tissues from 31 cases of benign cutaneous adnexal tumors (five trichofolliculomas, five trichoepitheliomas, two sebaceous adenomas, five apocrine hidradenomas, five eccrine poromas, five eccrine spiradenomas, and four syringomas) were immunostained for Bcl-x. RESULTS: Strong staining for Bcl-x was seen in cells of the epidermal granular layer and inner root sheath of hair follicles. Sebaceous gland cells showed strong staining. Apocrine gland cells showed weak to moderate staining. No staining was seen in eccrine gland cells. The basaloid cells of trichofolliculomas and trichoepitheliomas showed no staining. In sebaceous adenomas, the sebaceous cells showed strong staining while the basaloid cells were negative. The cells of apocrine hidradenomas showed patchy weak staining. No staining was seen in eccrine poromas, eccrine spiradenomas, and syringomas. CONCLUSIONS: The degree of Bcl-x expression in cutaneous adnexal glandular structures appears to be related to their mode of secretion, being strongest in cells with apoptotic degradation of the entire cell (sebocytes). This pattern is recapitulated in the corresponding benign cutaneous adnexal tumors. Bcl-x may be useful in identifying cells with sebaceous differentiation in poorly differentiated adnexal tumors.     

Painful eccrine spiradenoma containing nerve fibers: a case report

An eccrine spiradenoma is a rare benign tumor most often seen in the head, neck and upper trunk of young adults. Although spontaneous pain or tenderness is a typical symptom of eccrine spiradenomas, the underlying mechanism has not been fully elucidated. Here, we report the case of a 47- year-old woman who had a spiradenoma in the subcutaneous tissue of her posterior neck accompanied by agonizing pain which was triggered by pressure. Multiple nodular lesions were excised and the typical histopathological findings of spiradenoma were seen. The histopathological architecture of a disorganized nerve fiber encasing the tumor nodules appeared to correlate with the unique clinical symptom of pain.     

Multiple eccrine spiradenomas in zosteriform distribution in a child

A 9-year-old boy presented with asymptomatic papulonodular lesions on the left half of the chin of 3-years duration. On examination he had small papular lesions on the left chin varying in size from 1 to 5 mm. An additional lesion on the margin of the lip was 14 mm in diameter. The overlying skin was normal. Lesions were firm, nontender, and fixed to the skin. Histopathologic examination of the biopsy specimen revealed characteristic features of eccrine spiradenoma. A literature search produced reports of 10 cases of linear/zosteriform spiradenoma. Of these, five were in children or adolescents. We report this patient because of the rare occurrence of this presentation.