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Diagnosing and treating patients with persistent neuropathic pain associated with peripheral nerve lesions can be challenging. The authors report the rare case of a painful eccrine spiradenoma treated as a traumatic neuroma for many years because of a history of acute trauma, the presence of a tender palpable mass, and symptoms of allodynia. Surgical excision of the neoplasm completely relieved the pain and hypersensitivity that 2 prior surgeries and other nonsurgical treatments failed to resolve. The diagnosis of eccrine spiradenoma was not established until resection and histopathological analysis of the tissue. This case highlights the need to develop and consider an extensive list of differential diagnoses, including eccrine spiradenoma, for peripheral nerve lesions that fail to respond to treatment.

Hidradenoma papilliferum is an uncommon benign tumour that is located in the anogenital region of middle-aged women. They are usually only diagnosed after excision and are thought to arise from anogenital mammary-like glands. We present the case of a young woman who noticed a slow growing perianal lump which caused minimal symptoms., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Eccrine spiradenoma (ES) typically presents as a solitary tender lesion. Multiple ES is a rare variant of ES and can present in a segmental, linear, blaschkoid, or zosteriform pattern. The etiology of multiple ES is unknown, but several theories have been suggested including a multipotent stem cell origin. We report the case of a 30-year-old woman with multiple painful ES in a zosteriform pattern on the mid-back and abdomen. Skin biopsy of a representative lesion demonstrated a circumscribed tumor nodule encapsulated by a fibrous capsule with diffuse dense basophilic proliferation located in the dermis. The lesions were then excised on two separate sessions without recurrence.

Eccrine syringofibroadenoma (ESFA) is a rare benign lesion of ductal and secretory differentiation exhibiting multiple cutaneous polymorphic presentations with an unknown etiology. We present a case of ESFA that uniquely exhibited large, thick, verrucous-like hyperplastic growths as well as superficial shiny mosaic plaques and deep ulcerations in three different anatomical locations in the same patient. The diagnosis of ESFA was confirmed histologically after biopsies were performed on all of the affected areas. In addition to a case report and literature review, we also present classification, clinical, and histologic aspects of ESFA.

Malignant eccrine spiradenoma (MES) is an extremely rare but aggressive tumour, resulting in high mortality. We report the first case in the UK of metastatic MES on the scalp of a 37-year-old man who initially presented with a raised nodular mass which had grown rapidly over a few months. Excision biopsy of the lesion was performed and histology confirmed MES with evidence of cytological atypia and necrosis. A further wide local excision of the lesion with a 1 cm surgical margin was performed and full clearance was achieved. At 10-month follow-up another similar lesion had developed close to the previously excised area. A CT scan demonstrated metastatic lesions in the lungs, liver and pelvis, and subsequently chemotherapy was initiated. Although MES is uncommon, it has a high propensity to metastasise, therefore prompt identification, treatment and close follow-up of these tumours is essential., (2014 BMJ Publishing Group Ltd.)

Syringocystadenoma papilliferum is a rare adnexal tumor that often occurs as a solitary tumor in the head and neck region, although occurrences on other anatomical locations have been described. Linear configurations have been described, but an agminated form is a more rare and underreported variant of this tumor. We describe a case of a healthy 10-year old female with agminated syringocystadenoma papilliferum occurring on her left supraclavicular region, with the clinical appearance of grouped molluscum contagiosum papules.Case synopsis A healthy 10-year-old girl was referred for the treatment of a "collection of molluscum contagiosum" of the left supra clavicular region of several years duration. The lesions were asymptomatic and refractory to cryotherapy. The patient was a healthy girl with no significant systemic findings. Cutaneous exam revealed a clustered group of pink, dome shaped, umbilicated papules over a 1.5 x 1 cm area within the left supraclavicular fossa (Figure 1a). An excisional biopsy was performed. Routine H&E stained sections revealed cystic epidermal invaginations with papillary projections. The superficial portions of the cyst were lined by stratified keratinizing epithelium, whereas the deeper papillated portion exhibited a double layer of basal-like cells and luminal eosinophilic columnar cells with focal decapitation secretion. The papillary structures contained fibrovascular cores and lymphoplasmacytic infiltrates. A component of hamartomatous follicular growth was not identified (Figure 1b-d.). A diagnosis was made of agminated syringocystadenoma papilliferum.

Malignant spiradenoma/cylindroma of the vulva is an extremely rare adnexal tumor. We report the clinicopathological features of a 58-year-old woman who presented with malignant spiradenoma/cylindroma originating in the vulva and metastasized to the inguinal lymph nodes. Surgical excision with adequate margins and lymph node dissection was performed. Sections from the case were stained with Periodic Acid Schiff stain before and after diastase. Immunohistochemical study of the case using antibodies to carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), cytokeratin5/6 (CK 5/6), p63, cytokeratin7 (CK 7), smooth muscle actin (SMA), and S100 were performed. Microscopic examination revealed that spiradenoma nodules were positive to EMA and CEA. However, the cylindroma lobules showed strong immunoreactivity to p63 and CK5/6, whereas both tumor components were negative to S100, SMA, and CK7. Malignant spiradenoma/cylindroma is a rare tumor with controversial histogenesis that should be considered in the differential diagnosis of primary adnexal carcinoma and secondary metastatic tumors in the vulva. Further studies on a wider cohort should be encouraged.

Hidradenoma papilliferum of the anogenital region was previously believed to originate from apocrine glands but has recently been accepted as originating from anogenital mammary-like glands. We describe a case of hidradenoma papilliferum with mixed features of syringocystadenoma papilliferum and mammary-like glands from the left labia majora of a 25-year-old woman. Histopathologically, the lesion showed an epithelial lining with apocrine secretion, and like syringocystadenoma papilliferum, the lesion extended from the epithelium as invaginations into the dermis. Adjacent to this lesion were ductal and glandular structures resembling normal mammary tissue. This review of the literature highlights the heterogeneity and complexity of lesions arising from anogenital mammary-like glands, and this case serves as further documentation of the association between anogenital mammary-like glands and hidradenoma papilliferum.

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., (Copyright © 2012 S. Karger AG, Basel.)

Carcinosarcoma is exceedingly rare in the vulva. We describe a case of carcinosarcoma in a 67-year-old female patient who presented with recent enlargement and pain of a vulval nodule noted for 15 years. The excised tumor showed intermixed carcinomatous (adenocarcinoma and anaplastic carcinoma) and sarcomatous elements (osteosarcoma, chondrosarcoma, and leiomyosarcoma), which focally merged with several lobules of typical eccrine spiradenoma. The inguinal lymph nodes showed metastasis of the carcinomatous component only. This case represents the first reported case of vulval carcinosarcoma of the skin adnexal origin, and has to be distinguished from sarcomatoid carcinoma of epidermal origin because of a probable more aggressive behavior.

A very rare case of nodular hidradenoma with an atypical clinical presentation of the face of a 70-year-old woman is presented. Although this rare benign tumor has the potential of local recurrence and malignant transformation, it is commonly reported in the dermatology, pathology, and oncology literature, with limited clinical information. The purpose of this report was to emphasize the clinical features of this lesion for plastic surgeons.

The presence of myoepithelial (ME) cells is considered an important feature in the vast majority of benign breast lesions. Recently, a case showing the absence of myoepithelium in a mammary duct with apocrine metaplasia was reported. To investigate the status of ME cells associated with apocrine metaplasia, the distribution of ME cells in 59 metaplastic and intraductal proliferative apocrine lesions was evaluated using immunohistochemical expression of p63 and Calponin. p63 showed a diminished number of ME cells and increased intermyoepithelial nuclear distance in ducts with all variants of apocrine metaplasia and proliferation compared with normal glands. In the majority of cases, Calponin showed a continuous ME layer. In 6 cases, including an apocrine papilloma, there were definitive ME gaps confirmed by both markers, in the absence of atypia and with preservation of the basement membrane. In all cases, there was frequent heterogeneity in the distribution of ME cells in ducts harboring apocrine cells and even in various papillae within papillary lesions. In summary, benign and noninvasive apocrine lesions can show reduction and occasional complete loss of ME cells. This observation is particularly important when evaluating apocrine papillary proliferations, in which the absence of ME cells may lead to overdiagnosis of atypia and/or malignancy. The observation suggests that at least 2 ME markers should be used when evaluating apocrine lesions, and that a malignant diagnosis should be based on features of the proliferating cells until more data become available on the significance, if any, of the absence of ME cells in apocrine lesions.

Hydrocystoma is a benign tumor whose development involves the sudoriferous eccrine or apocrine glands. We report the case of a giant apocrine hydrocystoma in a 70-year-old female diabetic patient. The tumor's natural progression over 10 years and its volume caused significant functional and aesthetic damage. This article discusses the radiological, clinical, anatomopathological, and therapeutic aspects of this tumor. The case is original in the tumor's location, size, and its double visual and lachrymal functional involvement as well as its aesthetic damage., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Microcystic adnexal carcinoma is a rare sweat gland malignancy, characterized by slow, but aggressive infiltrative growth. Histologic characteristics are follicular and glandular differentiation, as well as bland cytologic features. The benign histologic appearance, particularly in the upper level of the tumor, can be confused with syringoma or benign follicular neoplasm. Thus the risk of misdiagnosis is considerable, especially if only superficial biopsies are provided. We report two patients with microcystic adnexal carcinoma, using them as a basis to discuss, pathogenesis, diagnosis, histology and therapy of the tumor.

Syringocystoadenoma papilliferum is a benign adnexal tumor usually located in head and neck that occurs during childhood or adolescence. A case of a syringocystoadema papilliferum associated with apocrine hydrocystoma and verruca is presented. It is unusual to see the occurrence of three histopathologic types of tumors coexisting in one cutaneous lesion.

Syringocystadenoma papilliferum (SCAP) is a benign rare adnexal skin neoplasm, which in a third of cases arises from a nevus sebaceous and is most commonly found on the head and neck and in very rare instances found on the genitalia. We report on a 59-year-old man with SCAP on the scrotum. The clinical scenario and histopathological findings are outlined. Following excision and histological confirmation of a noninfiltrative process, the patient remains asymptomatic. To our knowledge, only three other cases of such a lesion on the scrotum have been reported in the literature.

Objective: To review the cytologic features of hidradenoma to describe criteria that may aid in identification of these tumors at the time of aspiration and allow for a more specific diagnosis than the umbrella term of benign skin adnexal tumor., Study Design: Three patients aged 10-23 years presented with mass lesions in the region of the head. Fine needle aspiration (FNA) of these lesions showed the presence of benign skin adnexal tumors. Subsequent histology showed the presence of hidradenomas. A retrospective analysis of the cytology was performed., Results: The following cytomorphologic findings are consistent with the diagnosis of hidradenoma: a cystic component to the aspirate as represented by amorphous background material with or without foam cells and epithelial duct-like cells and tubular structures. In addition, a biphasic cytoplasmic staining pattern with both eosinophilic and clear to basophilic cells may be seen with the Papanicolaou stain in hidradenomas., Conclusion: Knowledge of the cytologic features of hidradenoma will allow for correct management of the patient and prevent misdiagnosis as a malignant tumor.

Clear cell hidradenoma is a rare skin appendage tumor. A 41-year-old female presented with right gluteal mass. Excisional biopsy of the mass was performed. Under the epidermis, an eosinophilic-cytoplasm, uniform-appearance, oval-round-nucleus, benign tumor with cystic and solid components was detected. These results were consistent with clear cell hidradenoma. The patient had not been given postoperative adjuvant treatment and has been under follow up free from disease for 2 years.

Adenocarcinoma in situ rarely occurs in vulvar papillary hidradenoma. We encountered 2 cases of adenocarcinoma in situ arising in a papillary hidradenoma of the vulva. Both patients were asymptomatic women, aged 83 and 92 years, who presented with nodules (1.0 and 2.0 cm) on the vulva. Macroscopically, the lesions seemed tan-pink, fleshy, and well circumscribed. One tumor ulcerated the overlying epidermis. On microscopic examination, the tumors showed focal features of benign hidradenoma at the periphery with transitions into areas of increasing cytologic atypia that fulfilled criteria for adenocarcinoma in situ similar to that seen in the breast. One tumor showed a predominant cribriform pattern with moderate atypia and many mitoses; the other showed a mixture of cribriform and micropapillary patterns, mild atypia, and fewer mitotic figures. There was no evidence of destructive invasion or desmoplasia in either tumor. Both lesions show areas strongly immunoreactive for mammaglobin and gross cystic disease fluid protein 15 as well as estrogen and progesterone receptor protein in 1 case. There was no evidence of benign ectopic breast tissue within or adjacent to the neoplasms. Both patients underwent local excision with no evidence of tumor recurrence at 15 and 32 months of follow-up. The fact that these tumors displayed morphologic and immunohistochemical features that resembled ductal carcinoma in situ of the breast demonstrates the close homology between papillary hidradenoma and breast epithelium. In the absence of invasion, our experience suggests that these tumors can be cured by local excision.

Clear cell hidradenoma (CCH) is a benign skin appendageal tumor. Most cases occur in the skin of the head and face. The tumor usually presents as a solitary firm dermal nodule. Histologically, it is a well-circumscribed, nonencapsulated tumor formed of 2 cell types, one with clear cytoplasm and the other with dark eosinophilic cytoplasm. Although most cases are benign, occasional cases with malignant transformation have been reported. We report a case of CCH in a very unusual location. To the best of our knowledge, CCH has not been previously described in this site, in the English literature. Although rare, inclusion of CCH should be included in the differential diagnosis of benign solid and clear cell tumors of the vulvar skin.

We report the third case of eccrine syringofibroadenoma (ESFA) arising in peristomal skin. A 55-year-old man presented with a 15- x 10-cm pale pink verrucous, exophytic, intermittently tender plaque involving his ileostomy site. He had undergone proctocolectomy with ileostomy creation 33 years prior for ulcerative colitis. The clinical differential diagnosis included granulomatous dermatitis, infection (fungus or atypical mycobacterium), or neoplasm. A punch biopsy specimen was performed and showed ESFA. Although ESFA is considered to be benign, recent reports have demonstrated an association of ESFA with malignancy or malignant transformation of ESFA. Furthermore, ESFA and reported cases of ileostomy carcinoma share similar clinical symptoms at presentation including pain, irritation, ulceration, bleeding, and the presence of a fungating mass. The lesion was, therefore, excised in toto and the excisional specimen showed no evidence of malignancy. We speculate that ESFA is a reaction to chronic irritation and, analogous to other long-standing reactive processes such as lichen sclerosis or burn scar ulcers, may be associated with malignant transformation. Because of this possibility and the clinical overlap with ileostomy carcinoma, peristomal ESFA should be treated with complete excision. If it is not amenable to complete excision because of lesion size or anatomic complexity, generous sampling and close clinical follow-up are recommended.

Secondary malignancies are an important cause of morbidity and mortality in childhood cancer survivors. Salivary gland tumors account for about 6% of the second cancers. The majority of these are mucoepidermoid carcinomas (MEC) of the parotid gland. We report the clinical and pathological features of a rarer histological type, acinic cell carcinoma (ACC), in a childhood acute lymphoblastic leukemia (ALL) survivor. The behavior of secondary ACC appears similar to primary tumor and similar treatment may be adopted. Early recognition and complete resection is important for achieving a good outcome. Careful monitoring for recurrence or a third malignancy is needed., ((c) 2007 Wiley-Liss, Inc.)

A case of apocrine adenoma, a rare benign tumor of the breast is reported. A left breast nodule from a 47-year-old female was found by mammography and excised. The tumor was well circumscribed and composed of tightly packed tubules lined by cells with apocrine features. There has been no recurrence in two years. The few previously reported cases are summarized. Apocrine adenomas have been reported arising in a variety of sites including the breast, (1-5) perianal region, (6,7) eyelid, (8) and axilla. (9) Their appearance has similarities to the apocrine metaplasia commonly seen in fibrocystic change of the breast but they are well-circumscribed tumors with closely packed tubular and papillary structures. They are not distinctive by mammography or physical examination and should be managed as any other breast mass.

We present a boy with a congenital, ulcerated nodule on the scalp. At birth, the lesion was considered to be the result of a traumatic injury, but a biopsy at the age of 6 months pointed to a diagnosis of syringocystadenoma papilliferum. We draw attention to the difficulty of identifying head lesions in young children from clinical signs alone.

Eccrine spiradenoma (ES) is a fairly common, benign, cutaneous tumor originating from the sweat glands. In contrast, the malignant counterpart of ES, malignant eccrine spiradenoma (MES), is extremely rare. A long-standing lesion rarely begins to enlarge rapidly. A growth that results in ulceration or discoloration may be associated with malignant transformation. We present the first reported case of this tumor metastasizing to an intramammary lymph node (IMLN). The uncommon metastasizing focus of the periumbilical MES and its histopathological similarity with a primary breast carcinoma made the diagnosis difficult.

The occurrence of an atypical fibroxanthoma in association with a syringocystadenoma papilliferum is reported. Both neoplasms showed characteristic histologic features and displayed typical immunohistochemical profiles. The neoplasms developed in intimate admixture in an area of nevus sebaceus.

Tumors of sweat gland origin are rare in the hand but should be considered in the differential diagnosis when certain imaging features are present. We present a case of nodular hidradenoma of the hand, with previously unreported magnetic resonance imaging features, and a further case in the thigh, both with ultrasound and histopathological correlation. The imaging literature of this tumor is reviewed, and its significance is discussed with respect to the current understanding of its malignant potential.

Eccrine syringofibroadenoma (ESFA) is a rare, benign adnexal tumor arising most often on the extremities of elderly individuals. It is typically a slow-growing, flesh- to reddish-colored nodule or plaque. Histologically, the tumor consists of anastomosing cords of cuboidal epithelial cells surrounded by a fibrovascular stroma containing plasma cells. The cords contain scattered ductal structures lined with cuboidal cells resembling eccrine ducts. The co-existence of ESFA with squamous cell carcinoma has been described, eliciting the term eccrine syringofibroadenoma. The differential diagnosis includes poroma, porocarcinoma, fibroepithelioma of Pinkus and clear cell acanthoma. ESFA stain positively with epithelial membrane antigen and carcinoembryonic antigen. Cytokeratin studies have been inconsistent.

Apocrine hidradenoma is a benign adnexal neoplasm with apocrine differentiation. The neoplasm is composed of four different types of epithelial cells, including pale or clear cells, polygonal cells, mucinous cells and squamous cells, with variable proportions of them from case to case. In most examples of this neoplasm, clear or the polygonal cells are predominant, whereas the other types of neoplastic cells are less abundant. We report two cases of apocrine hidradenoma mostly composed of squamous cells. Histopathologic examination showed that the neoplasms were composed of both solid and cystic areas. The solid aggregations of neoplastic cells were composed of a peripheral layer of basaloid polygonal cells, whereas squamous cells forming the bulk of the aggregations. These squamous cells showed large eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. In one case, small foci of mucinous cells could also be seen in some aggregations of neoplastic cells, mostly around ductal structures. In both the cases, some of the tubular structures lined by epithelial cells showed evidence of decapitation secretion in their luminal border. The neoplastic stroma consisted of sclerotic collagen bundles when compared with adjacent normal dermis, and artefactual clefts separated the neoplasms from the surrounding tissue. The rare cases described in this report are exceptional because most of the neoplastic cells showed squamous appearance and for that reason we think that squamous cell apocrine hidradenoma is the most appropriate name for these neoplasms.

Poroid hidradenoma is a variant of the eccrine poroma that belongs to the group of poroid neoplasms. It presents architectural features of hidradenoma, with solid and cystic areas and tumour cells restricted to the dermis, and cytological features of poroid neoplasm such as poroid and cuticular cells. Poroid hidradenoma becomes malignant in less than 1% of cases, but its histologic characteristics may resemble those of malignant neoplasms; thus it is easily misdiagnosed. Twenty percent of poroid hidradenomas occur in the extremities. To date, very few cases of this entity have been reported in the literature. We present a case of poroid hidradenoma in a 35-year-old man with a soft-tissue lesion on his right thigh.

Mucinous cutaneous adnexal tumors are an uncommon occurrence. We describe three cases of mucinous hidradenoma characterized by diffuse and prominent mucinous cell proliferation and discuss the relevant literature. The patients (two men and one woman) ranged in age from 32 to 72 years. The lesions were completely excised, and all patients were alive without evidence of disease (follow-up interval 3-4 years). Case 1 presented as a 1.3-cm eyelid nodule and histology showed a mucinous apocrine hidradenoma with a proliferation of mucinous cells and apocrine glands. Case 2, from the shoulder of a 40-year-old man, was a 2.5-cm well-circumscribed nodule composed of hyperplastic villoglandular proliferation of mucinous cells with focal squamous whorls and focal columnar glandular cells showing decapitation secretion. The tumor was also interpretated as a mucinous hidradenoma, most likely of follicular-apocrine origin. Case 3 was a 3.0-cm scalp nodule. The histology showed a mucinous clear-cell hidradenoma composed of numerous mucinous glands amidst small squamous cells with clear cytoplasm and a hyalinized fibrovascular stroma.

Background: Brooke-Spiegler syndrome is a well known but rare cutaneous disorder characterized by the presence of multiple trichoepitheliomas and cylindromas., Methods: Four families with Brooke-Spiegler syndrome are described., Results: Spiradenomas developed on the back in two patients. A CO(2) laser was used to treat facial trichoepitheliomas in two patients with good results., Conclusions: The large number of facial lesions in this syndrome often produce a significant emotional impact. We believe that it is important to provide patients with a good therapeutic option. CO(2) laser therapy may fill this role.

Hidradenoma papilliferum is a rare, benign, cystic, papillary apocrine gland tumor that occurs almost exclusively in women in the skin of the anogenital region. We present the case of a 66 year old woman who presented with a progressively enlarging perianal nodule. We provide a review of the literature emphasizing the clinico-pathological characteristics and differential diagnosis. Local excision is necessary for identification and cure. No recurrence was observed after one year follow-up.