Sweat Gland Tumors Research Articles

Multifocal extramammary Paget’s disease-associated adenocarcinoma: a rare condition of flexoral skin of multiple sites

Extramammary Paget disease (EMPD) is a rare malignant neoplasm of apocrine sweat glands that is morphologically and histologically identical to Paget disease of the breast. The primary lesion is usually a solitary, well-demarcated, erythematous, scaly plaque that may contain crust, erosions, or ulcerations. The vulva is the most common site, but any area containing apocrine sweat glands may be involved. We present a case of triple extramammary Paget disease of the groin and bilateral axillae in a diabetic patient whose axillary lesions appeared consistent with acanthosis nigricans. This case demonstrates the need to consider EMPD in the evaluation of acanthosis of the axilla given its ability to mimic more common conditions.

Malignant Eccrine Spiradenoma with Squamoid and Chondroid Differentiation

Malignant eccrine spiradenoma (MES) is an extremely rare sweat gland tumor that arises in a pre-existing benign eccrine spiradenoma. We describe a case of malignant eccrine spiradenoma of a 41-year-old male with a history of swelling over nape of neck for 6 yrs and recent onset of increase in size since 6 months. Microscopically, the tumour showed benign component, which was composed of two nodules with interwining bands of 2 types of epithelium, inner differentiated cuboidal cells and peripheral undifferentiated cells. Malignant component was composed of large clear cells with areas of squamoid and chondroid differentiation, lymph nodes showed metastasis. With all these features, a diagnosis of malignant eccrine spiradenoma with Squamoid and Chondroid differentiation was made.

Treatment of eccrine porocarcinoma with Mohs micrographic surgery: a cases series and literature review.

Eccrine porocarcinoma (EPC) is a rare malignant tumor of the eccrine sweat gland. It is a potentially fatal neoplasm that is locally aggressive and commonly recurs. Wide surgical excision has traditionally been the treatment of choice and is curative in approximately 70-80% of cases. The disease is metastatic to lymph nodes and distant sites in 20% and 10% of cases, respectively. Metastatic EPC has not shown any great response to adjuvant chemotherapy or radiation. The purpose of this study was to evaluate the efficacy of Mohs micrographic surgery (MMS) for EPC as an alternative to wide local excision. Five patients diagnosed with EPS between 2011 and 2014 at the University of Louisville and treated with MMS were studied. Recurrence-free periods subsequent to the treatment of EPC with MMS were measured. The five patients with EPC treated by MMS remained recurrence-free for a mean of 11 months (range: 2-26 months). Mohs micrographic surgery is a highly effective treatment for EPC. Given the high rate of recurrence, propensity for lymph node metastases, and the often ineffective options for treating advanced disease, MMS should be considered in the treatment of all cases of EPC.

Malignant sweat gland tumours: an update

Cutaneous adnexal tumours can be a diagnostic challenge for the pathologist. This is particularly true in the case of tumours with sweat gland differentiation, due to a large number of rare entities, a multiplicity of names to designate the same neoplasms and consequent lack of consensus regarding their classification and nomenclature. In the traditional view, sweat gland tumours were divided into eccrine and apocrine. However, this has been challenged in recent years, and in fact many of these tumours may have both eccrine and apocrine variants. Some display more complex features and defy classification, due to the presence of other lines of differentiation, namely follicular and/or sebaceous (in the case of apocrine tumours, due to the close embryological relationship between apocrine glands, hair follicles and sebaceous glands). The present paper reviews and updates the basic concepts regarding the following malignant sweat gland tumours: apocrine carcinoma, porocarcinoma, hidradenocarcinoma, spiradenocarcinoma, cylindrocarcinoma, microcystic adnexal carcinoma and related entities, squamoid eccrine ductal carcinoma, digital papillary adenocarcinoma, primary cutaneous mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and primary cutaneous signet ring cell carcinoma. Particular emphasis is put in recent findings that may have implications in the diagnosis and management of these tumours.

Malignant sweat gland tumors: an update.

Sweat gland carcinomas represent an important and somewhat contentious group of tumors in diagnostic skin pathology. Although their overall incidence is rare, they show a wide range of histologic features, and reliable classification is often challenging. Awareness and recognition of these tumors is, however, important as they may be associated with significant morbidity and even disease-related mortality, especially if left untreated. According to their behavior, sweat gland carcinomas are traditionally separated into tumors with low-grade and high-grade malignant behavior. This article is aimed at increasing awareness and providing an overview of malignant sweat gland tumors with emphasis on recently reported and novel findings and diagnostically challenging and potentially underrecognized entities. It further aims to illustrate the wide morphologic range of these tumors and provides a discussion of the relevant immunohistochemistry, disease-specific behavior, and differential diagnosis.

Mixed tumor of apocrine sweat gland in a terrier dog

A 4-year-old, female terrier dog was presented with complaint of a solid mass found incidentally on the left groin area. Touch of mass was painless. The skin overlying the mass was normal in appearance, with no ulceration. Complete physical examination, hematological tests, and radiology were done. No other abnormality was detected in clinical examination, and laboratory findings were within normal ranges. Radiographic surveys showed no local and distal metastasis. With excisional biopsy, the whole abnormal area was removed. It was loosely attached to the adjacent tissues. Grossly, the lesion was a 2.5 × 2.5 × 2, well-circumscribed, septate, and yellowish-white mass. At the time of excision, the surgical margin at the deep subcutis was separated by a thin capsule, but the border between the tumor and dermis was unclear. No areas of hemorrhage or necrosis were noted. Histopathological examination revealed a well-circumscribed tumor composed of both epithelial and mesenchymal components. Epithelial component was composed of well-differentiated apocrine glands containing one or two layers of epithelial cells with characteristic decapitation secretion. Mesenchymal component had myxoid tissues containing fusiform or stellate cells, and chondroid tissue. There was a lipomatous differentiation from small-sized adipocytes to vacuolated spindle cells. No osteoid tissue was observed. There were no cellular atypia and evidences for vascular invasion of neoplastic cells. Based on characteristic morphologic findings, and lack of clinical or radiographic features to support metastasis, the diagnosis of apocrine mixed tumor was made. Complete tumor removal was accepted as a recommended treatment.

Immunohistochemical distinction of primary sweat gland carcinoma and metastatic breast carcinoma: can it always be accomplished reliably?

Even with adequate history, the distinction of cutaneous metastatic breast carcinoma from primary sweat gland carcinoma can be difficult. Although previous studies have attempted to separate these tumors with various immunohistochemical panels, those series have been limited by small numbers of patients as well as the inclusion of benign sweat gland tumors. In this analysis, stains for p63, CK5/6, and D2-40 were included, as well as GATA3 and mammaglobin, in an evaluation of 21 primary sweat gland carcinomas and 33 examples of cutaneous metastatic breast carcinoma. Immunoreactivity for p63, CK5/6, D2-40, GATA3, and mammaglobin was respectively observed in 81%, 71%, 52%, 71%, and 5% of sweat gland carcinomas compared with 6%, 6%, 6%, 91%, and 45% of metastatic breast carcinomas. These differences were statistically significant for p63, CK5/6, and D2-40. For the diagnosis of metastatic breast carcinoma, GATA3 was the most sensitive marker (91%), but its sensitivity was substantially lower. Mammaglobin was 95% specific for breast carcinoma but again suffered from limited sensitivity (45%) in this context. These data suggest that p63 and CK5/6 are specific determinants for sweat gland carcinoma in the stated setting. In the absence of those analytes, metastatic breast carcinoma cannot always be identified to the exclusion of a primary tumor. This diagnostic scenario continues to require the procurement of a detailed clinical history regarding the number and duration of skin lesions in any given case.

Case Report Of Solitary Eccrine Spiradenoma Of Eyelid

Eccrine spiradenoma, a sweat gland tumor, is rarely found in the eyelid. We encountered a 73-year-old man with a nodular growth in his left upper eyelid, which had developed over the last 12 years. He had developed mild pain in the past. Excision was carried out under local anesthesia in a pentagon fashion with clear margins. The histopathology was suggestive for a benign eccrine spiradenoma.

Hidradenocarcinoma: A Rare Sweat Gland Neoplasm Presenting as Small Turban Tumor of the Scalp.

Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

Spiradenoma and cylindroma originate from the hair follicle bulge and not from the eccrine sweat gland: an immunohistochemical study with CD200 and other stem cell markers.

Spiradenoma and cylindroma have historically been described as sweat gland tumors and have often been considered to be of eccrine lineage. However, (a) associations with trichoepitheliomas in Brooke-Spiegler syndrome or with trichoepitheliomas and milia in Rasmussen syndrome, (b) neoplastic combinations with hair follicle tumors in solitary cases, and (c) anatomical considerations support a folliculosebaceous-apocrine lineage. Follicular stem cell markers may allow for further characterization of these neoplasms. A total of 97 tumors were examined for the expression pattern of follicular stem cell markers cytokeratin 15 (CK15), cytokeratin 19 (CK19), pleckstrin homology-like domain, family A, member 1 (PHLDA1), and CD200. The tumors were comprised of 27 spiradenomas, 30 cylindromas, 16 hidradenomas, 19 poromas, 4 dermal duct tumors and 1 hidroacanthoma simplex. All spiradenomas and cylindromas were CD200-positive whereas the other tumors classified as eccrine in lineage were all CD200-negative. CK15 also discriminated between spiradenomas and cylindromas and the remaining neoplasms but not to the degree of CD200. PHLDA1 and CK19 were noncontributory. It is concluded that both spiradenoma and cylindroma are not eccrine but follicular tumors. More specifically, it is proposed that both adnexal neoplasms are derived from the hair follicle bulge and as such represent one of the least differentiated follicular tumors.

Porocarcinome eccrine bowénoïde : un carcinome annexiel de diagnostic difficile

Eccrine porocarcinoma is a rare malignant sweat gland tumor, representing less than 0.01% of all cutaneous neoplasms, with eccrin differentiation. Its acrosynringeal origin and physiopathology still remain discussed. The prognosis of this carcinoma is held to be poor with a significant risk of lymph node metastasis and local recurrence. Also, this not specific tumor can be a challenging histological diagnosis, in particular, in Bowenoid variant. We report a case of Bowenoid and keratinizing variant of eccrine porocarcinoma of the left ring finger with pejorative evolution and initial diagnosis of infiltrating squamous cell carcinoma arising in Bowen's disease. The knowledge of these patterns and identification of eccrine differentiation of the tumor are essential for the diagnosis and for adapted therapeutic care.

Tumor suppressor expression and genetic characterization of benign and malignant chondroid syringoma

Chondroid syringoma is an uncommon epithelial and mesenchymal neoplasm of eccrine sweat glands. Most are benign, but some may exhibit aggressive behavior, including local recurrence and lymph node or distant metastases. Treatment is generally limited to radical surgical excision and possibly chemoradiation, although the latter is generally considered ineffective against these tumors. Histologic features of benign and malignant chondroid syringoma are similar, thus making morphology an unreliable marker for clinically aggressive behavior. Identification of biomarkers that predict clinical behavior would be potentially useful for selecting patients with malignant chondroid syringoma for targeted therapy. The purpose of this study was to investigate somatic mutations commonly involved in tumor pathways, and to determine if there is altered expression of tumor suppressors p16 and p53 in metastatic malignant versus benign chondroid syringoma. Five chondroid syringoma tissue samples were analyzed – 2 aggressive and 3 benign. Immunostaining for tumor suppressor genes p16 and p53 was performed. Genomic DNA was isolated and genetic analysis was performed using a custom Sequenom MassArray. Chondroid syringomas with clinically aggressive behavior showed strong nuclear p16 expression in contrast to those with benign behavior. Both the malignant and benign chondroid syringomas demonstrated positive p53 expression with varying intensity. No somatic mutations were identified in any of the chondroid syringomas.

Combined melanocytic and sweat gland neoplasm: cell subsets harbor an identical HRAS mutation in phacomatosis pigmentokeratotica

Phacomatosis pigmentokeratotica (PPK) is characterized by the co-existence of epidermal nevi and large segmental speckled lentiginous nevi of the papulosa type. PPK, previously explained as 'twin spot' mosaicism due to the postzygotic crossing-over of two homozygous recessive mutations, has recently been shown to derive from one postzygotic activating RAS mutation. Epidermal nevi, including those in PPK, are known to give rise to neoplasms such as trichoblastoma and basal cell carcinoma. Within speckled lentiginous nevi, Spitz nevi and melanoma have been well documented. We report a case of PPK with a combined melanocytic and adnexal neoplasm presenting where the nevi conjoined. Using next-generation sequencing techniques, we were able to identify the same HRAS G13R mutation within both components of the tumor, and to show the absence of additional mutated modifier genes in a panel of 300 cancer-related genes. Given the genetic findings in this rare tumor-type, we suggest that this case may be used as a model for understanding the development of biphenotypic neoplasia or intratumoral heterogeneity in some cases.

Epidemiological study of Appendageal skin tumors in tertiary health centre in central India

Introduction: Cutaneous appendageal tumours are a large diverse group of tumours that are commonly classified according to their state of appendageal differentiation- eccrine, apocrine, follicular and sebaceous. Most appendageal tumors are relatively uncommonly encountered in routine clinical practice. Though some of the appendageal tumors (e.g. syringoma, nevus sebaceous) can be diagnosed clinically with ease but most of them have non-specific morphological appearance and their diagnosis is mainly based on histopathological characteristics. Material and methods: It was a cross sectional descriptive study conducted in the department of dermatology, Chirayu medical college and hospital, Bhopal. A total of 90 patients attending department of dermatology were screened out for appendageal tumors and those 60(66.6%) who were confirmed by histopathological examination of lesional biopsy were included in the study. Results: On the basis of differentiation of tumors, sweat gland tumors were the most common appendageal tumors in our study (79.8%) followed by follicular tumors (11.6%), sebaceous were 8.3%. On individual tumor basis, syringoma constituted maximum numbers of cases (56.6%) followed by multiple trichoepitheliomas and hidrocystoma (both 11.6%). Face was the commonest site of predilection 40(66.6%) followed by scalp 12(20%). Conclusion: Appendageal tumors are relatively uncommon and constitute very small proportion of dermatological disorders. Though clinical appearance might help in diagnosing common appendageal tumors but histological examination remains the gold standard method in diagnosing clinically suspicious lesions

Anti-CD117 in sweat gland tumors

Anti-CD117 in sweat gland tumors

Chondroid Syringoma: Report of a Case with Uncommon Location

‘‘The mixed tumor’’ of the skin was originally defined by Billroth in 1859 as an entity having the same histopathologic properties of the mixed tumors of the salivary glands [1]. The term ‘chondroid syringoma’ was first used by Hirsch and Helwig in 1961 to describe this sweat gland tumor, because of the presence of sweat gland elements which are set in a cartilaginous stroma [2]. The reported incidence of CS among primary skin tumor is low, ranging between 0.010.098% [3]. This uncommon eccrine sweat gland tumor clinically presents as a slow-growing, painless, subcutaneous or intracutaneous nodule located usually in the head and neck region, and it affects middle-aged or older men [3,4]. Less commonly, this tumor can develop in the axillary region, penis, vulva, and scalp. We report a rare case of a chondroid syringoma with an atypical location on the back.

Chondroid Syringoma–An Unusual Presentation

Chondroid syringoma is a rare mixed tumour of the skin. It was first described by Hirsch and Helwig. The tumour is composed of a proliferation of epithelial cells present in a myxoid and chondroid matrix. Although chondroid syringoma was predominantly benign, sweat-gland tumour, the usual presentation is of an asymptomatic, slowly growing mass, typically located on the head and neck region. The present case was located in right leg, lateral aspect and rare.

Eccrine spiradenoma in knee.

Eccrine spiradenoma is an uncommon benign adnexal tumor of the eccrine sweat glands. Although it can occur at any age, it is most common in young adults without any sex predilection. Malignant transformation is rare, presenting as rapid increase in size of a long-standing lesion. Here, we report a case of eccrine spiradenoma in a 35-year-old man who presented with swelling over the right knee, with cytological atypia but no recurrence until date.

Nodular hidradenoma over the chin: A rare presentation

Nodular hidradenoma is an uncommon benign adnexal tumor arising from the eccrine sweat glands. It occurs as a slow growing, nodular, solid or cystic, protruding cutaneous mass, which may exhibit serous discharge or ulceration. The lesions are commonly seen in women on head, neck, and limbs. This is a case report of an 85-year-old lady who presented with a slowly progressive nodular protruding mass over the chin. Nodular hidradenoma is an uncommon benign tumor of sweat glands. The clinician and the pathologist should have a strong index of suspicion to detect the lesion. Complete surgical excision is warranted with a close follow-up.

Mucoepidermoid Carcinoma of the Eyelid -A Rare Case Report

we describe here a 43 year old woman who came to eye opd with a chief complaint of a mass in left lower eyelid since 4 years. On local examination the mass was non-tender, well defined margin, smooth, mobile and non-adherent to underlying tarsal plate. Other ocular examinations were normal. General investigations were done. The mass was excised by simple excision under peribulbar local anaesthesia and sent for histopathological examination. The report showed a well encapsulated malignant tumour composed of intermediate cells in sheets admixed with spindloid cells. Many polygonal cells with oncocytic cytoplasm were also seen in the sheets. Abnormal mitosis was noted. The features were suggestive of mucoepidermoid carcinoma- spindloid oncocytic variant. Mucoepidermoid carcinoma of eyelid is a rare malignant sweat gland neoplasm. To our knowledge this is the 1 st report of mucoepidermoid carcinoma of eyelid here in our Institute.