Clinical and morphological features of eccrine acrospiroma: analysis of literature data and case from practice.

Eccrine acrospiroma is a rare benign tumor of the skin arising from the epithelial cells of eccrine sweat ducts. The clinical picture is characterized by its variability, so a detailed morphological study of the operative material is necessary to establish a diagnosis. Differential diagnosis must be carried out with hemangioma, melanoma, infected sebaceous cyst, metastatic skin lesion, and other tumors from elements of the sweat gland. In the article the authors presented the clinical and morphological analysis of own case from practice of large eccrine acrospiroma on the back surface of the left thigh which was diagnosed in a 56-year-old man.

Nodular hidradenoma: clinical, dermoscopic, and histopathological features.

Nodular hidradenoma is an infrequent benign tumor originating from the proximal portion of the sweat glands, most commonly associated with the apocrine glands. Owing to its variable clinical presentation, correctly diagnosing nodular hidradenoma can be challenging, with several potential conditions in the differential diagnosis to consider. This article presents a healthy 52-year-old woman with an atypical location of nodular hidradenoma, highlighting the critical role of integrating clinical, dermoscopic, and histopathological characteristics for an accurate diagnosis. We discuss the clinical features, dermoscopic findings, histological examination, differential diagnosis, and treatment options for nodular hidradenoma, emphasizing the importance of surgical intervention in preventing potential malignant transformation.

Clear cell hidradenoma of the breast with MAML2 gene rearrangement.

Clear cell hidradenoma is a rare benign tumor of the breast, its origin and pathogenesis are controversial. We have experienced a case of breast clear cell hidradenoma with mastermind like transcriptional coactivator 2 (MAML2) gene rearrangement. The patient found a painless mass with a hard texture in the left breast areola without nipple discharge. Microscopically, the tumor was cystic and solid, locally arranged in a glandular structure, covered by single cuboidal cells; it was composed of clear cells, epidermoid cells, and basaloid cells; there were no necrosis or mitotic figures. Immunohistochemical staining showed that the tumor cells positively expressed low-molecular cytokeratin 7, low-molecular cytokeratins (Cam5.2), high-molecular cytokeratin 5/6, cytokeratin 14, CD117, and p63; and did not express calponin, and smooth muscle myosin heavy chain. The cuboidal cells were positive for SOX10 but negative for p63. Additionally, periodic acid-Schiff reaction showed purple-red granules in the tumor cytoplasm, but Alcian blue staining showed no blue mucus in the cytoplasm. The split signals of MAML2 gene were detected by fluorescence in situ hybridization. Subtle histological and immunophenotypical differences may help to distinguish breast clear cell hidradenoma from common breast tumors. Furthermore, the MAML2 gene rearrangement may be a molecular genetic characteristic of breast clear cell hidradenoma.

Challenging diagnosis of male intraductal papilloma masquerading as eccrine hidradenoma in the breast: Case report.

RATIONALE: This article presents a challenging case involving an elderly male patient with a misdiagnosed intraductal mammary papilloma initially identified as a sweat adenoma through ultrasound imaging. The study aims to explore the histopathology, clinical presentations, and sonographic features of both conditions, emphasizing the contributing factors to the diagnostic misstep. PATIENT CONCERNS: A 61-year-old male reported a persistent left breast mass, along with pain and swelling, spanning a 6-month duration. DIAGNOSES: Ultrasound examination indicated a deep, square, mixed-echo mass in the left nipple, initially suggestive of a sweat adenoma. However, subsequent pathological analysis following resection under general anesthesia confirmed an intraductal papilloma. INTERVENTION: The patient underwent surgical resection of the left breast mass under general anesthesia. OUTCOME: Post-surgery, the patient exhibited satisfactory recovery; however, regrettably, he was lost to follow-up. LESSONS: This study underscores the challenge in differentiating between clear cell sweat adenoma and male intraductal mammary papilloma solely based on ultrasonic characteristics. It emphasizes the susceptibility of ultrasound-based diagnoses to misinterpretation, highlighting the critical need for a comprehensive pathological examination to establish a definitive diagnosis.

Hidradenocarcinoma: A Case Series From the Scripps Clinic With a Systematic Review of the Literature.

BACKGROUND: Hidradenocarcinoma (HAC) is a rare adnexal carcinoma. To the best of the authors' knowledge, there are no published systematic reviews on HAC. OBJECTIVE: To incorporate a case series from the authors' institution and systematically integrate reported information to provide a reference tool for optimization of diagnosis and management. METHODS: A comprehensive MEDLINE search was conducted from database inception to 2021 using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This yielded 225 studies with 165 cases of HAC. References of included articles were also searched. In addition, 9 patients with HAC were identified from the authors' institution over the past 10 years. RESULTS: The mean age of HAC presentation is 60 years with a slight male predilection (60%). The head and neck is the most commonly affected region. Over 36% of cases either presented with metastatic disease or went on to metastasize. The most common treatment type was wide local excision, followed by Mohs micrographic surgery. CONCLUSION: Early detection with accurate histologic interpretation is prudent in all cases of HAC. Wide local excision is the current first-line treatment. However, Mohs micrographic surgery offers complete marginal analysis with evidence of reduced risk of metastasis and better outcomes compared with wide local excision. Currently, there are no National Comprehensive Cancer Network guidelines for the treatment of HAC, and consensus guidelines are limited to tumor and nodal metastasis staging provided by the American Joint Committee on Cancer, eighth edition. Thus, this case series and systematic review integrates important aspects of diagnosis, workup, and management of HAC.

NUT Expression Is of Diagnostic Utility in the Distinction of Digital Papillary Carcinoma From Poroid Hidradenoma.

ABSTRACT: The distinction between digital papillary adenocarcinoma (DPAC) and benign cutaneous adnexal tumors is clinically important and can be challenging. Poroid hidradenoma frequently occurs at acral sites and can show a number of histological features, which overlap with digital papillary adenocarcinoma. Recent work has shown that YAP1-NUTM1 fusions are frequent in poroid hidradenoma and are associated with nuclear protein in testis (NUT) expression by immunohistochemistry. We evaluated the expression of NUT-1 by immunohistochemistry in 4 cases of DPAC and 4 cases of poroid hidradenoma. Three of 4 cases of poroid hidradenoma showed strong NUT-1 expression, with no staining in any of the cases of DPAC. These results suggest that NUT-1 immunohistochemistry may be a useful additional tool in evaluating this differential diagnosis.

Sweat Gland Tumors Arising on Acral Sites: A Molecular Survey.

Recurrent oncogenic drivers have been identified in a variety of sweat gland tumors. Recently, integration of human papillomavirus type 42 (HPV42) has been reported in digital papillary adenocarcinoma (DPA). The main objectives of the present study were (i) to provide an overview of the prevalence of previously identified oncogenic drivers in acral sweat gland tumors and (ii) to genetically characterize tumors in which no recurrent genetic alteration has been identified yet. Cases of acral sweat gland tumors were identified from the database of the French network CARADERM. After histologic review, the presence of previously identified genetic alterations was investigated in the entire cohort (n=79) using a combination of immunohistochemistry and targeted DNA and RNA sequencing. Tumor entities with no recurrent genetic alterations were submitted to whole-transcriptome sequencing. CRTC1::MAML2 fusion was identified in cases of hidradenoma and hidradenocarcinoma (n=9/12 and n=9/12). A p.V600E mutation of BRAF was observed in all cases of tubular adenoma (n=4). YAP1:MAML2 and YAP1::NUTM1 fusions were observed in poroid tumors (n=15/25). ETV6::NTRK3 and TRPS1::PLAG1 fusion transcripts were identified in secretory carcinoma (n=1/1) and cutaneous mixed tumors (n=3/4), respectively. The HPV42 genome was detected in most cases of DPA (n=10/11) and in 1 adnexal adenocarcinoma not otherwise specified. Finally, whole-transcriptome analysis revealed BRD3::NUTM1 or NSD3::NUTM1 fusions in 2 cases of NUT adnexal carcinoma and NCOA4::RET and CCDC6::RET fusion transcripts in 2 cystadenoma/hidrocystoma-like tumors. Our study confirms distinctive cytogenetic abnormalities in a wide number of acral adnexal neoplasms and supports the use of molecular analysis as a valuable aid in the diagnosis of these rare and often difficult to diagnose group of neoplasms.

Apocrine Papillary Hidrocystoma With Mucinous Metaplasia (Goblet Cell Type): A Case Report and Review of the Literature.

ABSTRACT: Mucinous metaplasia (goblet cell type) is exceptionally rare in the skin. This is the second case of apocrine papillary hidrocystoma with mucinous metaplasia (goblet cell type) and a review of the literature exploring the significance and frequency of mucinous metaplasia with goblet cells in nongenital skin. The patient is an elderly man who presented with a blue-pigmented nodule on the scalp that was clinically suggestive of an atypical nevus. Histologically, the lesion was composed of a simple cyst of cuboidal cells with decapitation secretion and mucinous metaplasia with goblet cells. Papillary formation was identified in the cysts. Most cases of cutaneous mucinous metaplasia have been reported on genital skin, usually after chronic inflammation of the area. This type of mucinous metaplasia is categorized as benign mucinous metaplasia of the genitalia (BMM) and is believed to be unrelated to apocrine glands owing to the different histologic features and absence of apocrine differentiation by immunohistochemistry. Mucinous metaplasia (goblet cell type) has been previously reported in benign adnexal tumors (eccrine acrospiroma/hidroadenoma, mixed tumor, and syringocystadenoma papilliferum) and in malignant tumors (apocrine hidradenocarcinoma and squamous cell carcinoma). To date, mucinous metaplasia has not been identified in the histologically normal apocrine glands.

Eccrine Hidradenoma of the Nasal Vestibule: A Rare Clinical Entity.

Eccrine hidradenoma is a relatively rare benign tumor of sweat gland origin but with possible malignant transformation. It usually consists of solitary, well-demarcated papules or nodules covered with normal skin. Common sites of involvement are the scalp, face, limbs, and anterior trunk. Although the lining of the nasal vestibule includes hair follicles, sebaceous glands, and sweat glands, an eccrine hidradenoma originating in the nasal vestibule has yet to be reported. Herein, we describe a rare clinical presentation of nasal eccrine hidradenoma, treated successfully using a transnasal endoscopic approach.

Reappraisal of tubulopapillary hidradenoma-like tumor of the mandible: Suggested change in nomenclature to reflect tumor origin.

Several cases of intraosseous mandibular tumors have been reported under the name "tubulopapillary hidradenoma-like tumor of the mandible (TPHLTM)." However, the intraosseous occurrence of sweat gland tumors needs to be reappraised. The aim of this review was to propose a new name for these tumors to reflect the possible tumor origin. In view of the incidence and the tissue of origin, TPHLTM is more likely to be a salivary gland tumor than a sweat gland tumor. Among salivary gland tumors, a recently described salivary neoplasm called "sialadenoma papilliferum-like intraductal papillary tumor (SP-IPT)" seems to be histologically and genetically identical to tubulopapillary hidradenoma. Therefore, we proposed that the term TPHLTM be replaced by "SP-IPT of the mandible," which better explains its origin and could help in clarifying the nature of SP-IPT.

Direct intracranial invasion of eccrine spiradenocarcinoma of the scalp: a case report and literature review.

BACKGROUND: Eccrine spiradenocarcinoma (SC), also known as malignant eccrine spiradenoma, is a rare malignant cutaneous adnexal neoplasm arising from long-standing benign eccrine spiradenoma. Malignant skin tumors rarely show direct intracranial invasion. However, once the intracranial structure is infiltrated, curative excision with sufficient margins can become extremely difficult, particularly when the venous sinuses are involved. No effective adjuvant therapies have yet been established. Here, we report an extremely rare case of scalp eccrine SC with direct intracranial invasion, which does not appear to have been reported previously. CASE PRESENTATION: An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC. CONCLUSIONS: Scalp SC with direct intracranial invasion is extremely rare. Radical resection with tumor-free margins is the mainstay of treatment, but the involvement of venous sinuses makes this unfeasible. High-precision EBRT in combination with maximal resection preserving the venous sinuses could be a treatment option for local tumor control.

Association of HPV42 with digital papillary adenocarcinoma and the use of in situ hybridization for its distinction from acral hidradenoma and diagnosis at non-acral sites.

Digital papillary adenocarcinoma (DPAC) is a rare tumor of sweat gland origin that preferentially affects the digits and has the potential to metastasize. Its tumor diagnosis can be difficult. Well-differentiated variants of DPAC can be confused with a benign sweat gland tumor, in particular nodular hidradenoma. With the recent detection of HPV42 DNA in DPAC by next-generation sequence analysis, we reasoned that this association could be used for diagnostic purposes. To this end, we performed in situ hybridization for HPV42 on 10 tumors diagnosed as DPAC as well as 30 sweat gland tumors of various histology types, including 8 acral hidradenomas. All DPAC were positive for HPV42. Positive hybridization signals for HPV42 were seen in both primary and metastatic DPACs. All other tumors and normal tissues were negative. This study confirms the association of HPV42 with the tumor cells of DPAC through in situ hybridization. The positive test result in all lesions of DPAC and lack of detection of HPV42 in any of the acral hidradenomas or other sweat gland tumors examined in this series is encouraging for the potential diagnostic utility of the assay. As documented by two scrotal tumors of DPAC, the in situ hybridization test for HPV42 can also help support the rare occurrence of this tumor at a non-acral site.

Cutaneous ultrasound: key diagnostic tool for the relapse of a single eccrine spiradenoma.

Eccrine spiradenoma is a rare, benign, adnexal skin tumor of the sweat gland. It is frequently solitary and presents as a small lesion in the dermal or the subcutaneous fat layer. Eccrine spiradenomas rarely progress to malignant transformation but they can relapse. Due to its rarity, there have been few reports about the sonographic appearances of eccrine spiradenoma. Sonographic findings were reported in a relapsing case of an eccrine spiradenoma, located in the deep dermal layers and hypodermis of the preauricular region in a middle-aged man. Ultrasound was very useful to suspect the relapse. Histology was correlated with the sonography and discussed the previously reported imaging findings of eccrine spiradenoma and other sweat gland tumors.

Giant vascular spiradenocylindroma of the scalp clinically mimicking a vascular tumor.

We present a giant vascular spiradenocylindroma (GVSC) with a unique presentation. The diagnosis of GVSC can be clinically and radiologically elusive. The differential diagnosis includes angiolipoma, angiosarcoma, and glomus-tumor-like vascular lesions. Dermatologists should consider this variant of spiradenocylindroma in any vascular-like lesion.

A rare case of Hidradenoma of the lower lip.

Hidradenoma are rare benign tumors with eccrine or apocrine differentiation that most often presents as a small, firm, solitary dermal nodule. At times, hidradenoma shows striking cytologic and histologic similarity to other neoplasms, including its malignant counterpart, hidradenocarcinoma. We present the first reported case of benign hidradenoma of the lower lip. The case illustrates important diagnostic features of hidradenoma and discusses the current understanding of its malignant potential and the appropriate management of such lesions.

Giant vascular eccrine spiradenoma: the first case in the scrotum and review of the literature author.

BACKGROUND: Giant vascular eccrine spiradenoma is a rare variant of eccrine spiradenoma. It is different from the eccrine spiradenoma in its larger size and greater degree of vascularity. It is often clinically confusedwith a vascular or malignant tumor. CASE PRESENTATION: Here, we report a case of a 67-year-old man who presented with a tumor in the scrotum scrotal region for 6 years. The tumor had begun as a small nodule and had grown slowly with over time. Surgical excision and pathological examination revealed that the lesion was the a giant vascular eccrine spiradenoma. CONCLUSION: This study reports the first case of giant vascular eccrine spiradenoma in the scrotum.