Search

Introduction: Syringocystadenocarcinoma papilliferum is an extremely rare malignant adnexal tumor that typically arises from a papilliferous syringocystadenoma (World Health Organization classification of skin tumors, 2018.). This tumor predominantly occurs in the cephalic region., Case Presentation: We present the case of a 68-year-old Moroccan male with no significant medical history who presented with a slowly progressing skin mass in the perianal region. Despite the chronic nature of the swelling, the patient remained in overall good health. Physical examination revealed a firm 2.3-cm mass in the perianal area, with no other remarkable findings. The patient underwent surgical excision of the mass. Macroscopic examination showed a solid-cystic, rounded mass. Microscopic examination revealed a malignant adnexal tumor with apocrine differentiation and papillary architecture. Immunohistochemistry was positive for p63, cytokeratin 7 (CK7), and smooth muscle actin, with a Ki67 labeling index in 60% of tumor cells, and negative for p16. The diagnosis of syringocystadenocarcinoma papilliferum was confirmed. A further surgical resection was performed, and the patient's postoperative course was unremarkable., Conclusion: Syringocystadenocarcinoma papilliferum is an extremely rare tumor, with its occurrence in the perianal region being particularly uncommon. This case contributes to the limited literature on this malignancy, highlighting its clinical and pathological features., Competing Interests: Declarations. Ethics approval and consent to participate: Yes. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Purpose: To describe the clinical features of a conjunctival apocrine hidrocystoma that developed 6 decades after a strabismus surgery and review existing literature on apocrine hidrocystomas of the conjunctiva and caruncle., Methods: Case report and review of literature on conjunctival apocrine hidrocystomas., Results: A 71-year-old man with a history of strabismus surgery as a child presented with a cystic lesion on the nasal conjunctiva and caruncle for 1 year. Excision of the lesion showed a unilocular cavity lined by a double layer of cells with the hallmark finding of apical decapitations, confirming a diagnosis of apocrine hidrocystoma. Seven additional cases of conjunctival and caruncular apocrine hidrocystomas were reviewed. All cases presented after 50 year of age. Most cases presented nasally or within the caruncle and had a pigmented appearance. No other reported cases had a history of trauma or surgery. All cases were treated with surgical excision without recurrence., Conclusions: History of strabismus surgery or conjunctival trauma may lead to ectopic deposition of apocrine glands that may contribute to the formation of an apocrine hidrocystoma., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To evaluate the postoperative complication rate and local recurrence rate of apocrine gland anal sac adenocarcinoma (AGASACA) in dogs surgically treated with a modified closed anal sacculectomy technique between 2015 and 2022., Study Design: Observational clinical retrospective study., Animal Population: Forty-seven client-owned dogs histologically diagnosed with AGASACA., Methods: Medical records were evaluated for patient demographics and history, physical examination findings, diagnostic imaging, incidence of concurrent neoplasia, postoperative complications, and incidence and time to local recurrence. Dogs with at least 150 days of follow-up were included in evaluation of local recurrence., Results: Two dogs were euthanized at 4 and 11 days after surgery. Forty-five dogs were included for long-term local recurrence evaluation, with a median of 364 days of follow-up (range 156-2156 days). Only one dog (2.2%) developed local recurrence with a time to recurrence of 90 days. Postoperative complications were reported in 15 dogs (31.9%) and were considered minor in 14 dogs (93.3%) and major in one dog (6.7%). Mean survival time for the 20 dogs that were deceased as of November 1, 2022 was 521 days (range 156-1409 days) and the median survival time was 388 days., Conclusion: The modified closed anal sacculectomy technique resulted in a lower AGASACA local recurrence rate than previously reported in the veterinary literature with a comparable postoperative complication rate., Clinical Significance: Given the low recurrence rate found in this study, the modified closed anal sacculectomy technique may reduce the need for adjuvant radiation therapy and potentially chemotherapy in AGASACA patients., (© 2024 American College of Veterinary Surgeons.)

Clinical and histopathologic case of an eyelid eccrine poroma, a benign adnexal neoplasm rarely found on the periorbital skin.

Adenomyoepithelioma(AME)of the breast is a rare condition, and comorbidity with carcinoma is even more unusual. Herein, we report a case of both AME and apocrine carcinoma in different breasts of a single patient. A 48-year-old woman presented to our clinic with a right breast tumor. Fine needle aspiration cytology(FNAC)was indeterminate and suspicious for both papilloma and non-invasive ductal carcinoma, but excisional biopsy indicated an AME. Immuno-histochemical staining showed EMA(+), AE1/3(+), and CK7(+)mammary duct cells and αSMA(+), CK5/6(+), and p63(+) myoepithelial cells. Six months later, the patient noticed a left breast tumor, and although FNAC indicated no malignancy, after 6 additional months, the tumor size had increased and a mammography revealed tumor microcalcification, suggesting malignancy. Vacuum-assisted biopsy revealed an apocrine carcinoma. The patient underwent partial mastectomy and sentinel node biopsy, followed by radiotherapy and chemotherapy. The post-surgical pathology was pT1pN0M0, Stage Ⅰ, triple- negative, and the patient was disease-free for 12 years postoperatively. To our knowledge, this is only the second case of AME and breast cancer in different breasts reported in Japan.

Syringocystadenoma papilliferum (SCAP) is a benign adnexal tumor commonly found on the scalp and face, and often associated with nevus sebaceous, with about half of cases appearing in early childhood. SCAP exhibits cystic invaginations with papillary structures and a double-layered glandular epithelium linked to the epidermal surface and stromal plasma cells. We are reporting a rare instance of intradermal SCAP in a 55-year-old male. He sought evaluation for a long-standing asymptomatic dark-pink papule in his left popliteal fossa, measuring 0.7 x 0.5 x 0.4 cm. A shave biopsy revealed papillary dermal fibrosis, glandular epithelium with apocrine secretion, and papillary projections without an epidermal connection. Infundibulofollicular keratinization was observed, along with stromal plasma cells. The patient chose local excision as the treatment option. This case highlights the rarity of intradermal SCAP, especially in the left popliteal fossa, with only one other reported case in the literature.

Abstract: Apocrine hidrocystomas are benign, cystic neoplastic lesions resulting from the apocrine secretory component of the sweat gland. They most commonly occur on the head and neck, with predilection to the periorbital area. Less frequent sites include the axilla, nipple, external auditory canal, foreskin, conjunctiva, lower lip, and fingers, among others. The authors report a unique case of a nail bed hidrocystoma in a 55-year-old woman, a site not previously described., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Syringocystadenocarcinoma papilliferum (SCACP) is a rare and aggressive malignant adnexal tumor originating from apocrine or pluripotent appendageal glands, often associated with a preceding syringocystadenoma papilliferum (SCAP) or nevus sebaceus (NS). This systematic review rigorously examines SCACP through an analysis of 78 cases documented between 1980 and 2024. The study aims to provide a comprehensive review of the clinical manifestations, diagnosis, treatment modalities, and outcomes associated with SCACP, while also reappraising its associations, particularly with NS. SCACP predominantly affects older adults, with an average age of 66.3 years and a slight male predominance, commonly presenting as ulcerated nodules or plaques on the scalp. This review highlights the aggressive nature of SCACP, evidenced by significant rates of metastasis and recurrence. Treatment is primarily surgical, with Mohs micrographic surgery offering potential benefits in terms of margin control and cosmetic outcomes. The association of SCACP with NS is critically evaluated, suggesting a complex etiopathogenesis and underscoring the importance of recognizing this association for timely diagnosis and management. Our review also briefly discusses potential pitfalls faced by clinicians in the diagnosis of SCACP. Our findings emphasize the need for standardized treatment protocols and further research into targeted therapies to improve patient outcomes in SCACP., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., (Copyright © 2024 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Syringomatous tumor of the nipple is a benign, locally infiltrative tumor. There are reports in the literature of tumor recurrence in cases of incomplete excision. Clinical and mammographic findings in syringomatous tumors are like those of breast carcinoma and the pathologist has a fundamental role in final tumor diagnosis. Therefore, the aim of this study was to report a case of syringoma located in the areolar region. A 33-year-old woman reported that she had noticed a nodule in her left areolar region 4 years previously (February 2019). A breast ultrasound was performed, detecting intraparenchymatous breast cysts. Surgical resection of the nodule was indicated although it was not performed. Two years later, in August 2021, the patient underwent a mastopexy with prosthesis inclusion. Histopathology study of the surgical specimen revealed a syringomatous tumor with positive margins. Thirteen (13) months after diagnosis (September 3, 2021 - October 16, 2022), the patient is doing well and receives clinical follow-up., Competing Interests: The authors declare no competing interests., (Copyright: Ana Vitória de Jesus Félix et al.)

Introduction: We report the clinical features and the management of two cases of orbital hidrocystoma in the setting of an enlarging orbital mass., Cases Description: A 48-year-old man presented with a mass in the right upper medial orbital quadrant, firmly attached to the supraorbital incisure. A 70-year-old man had a well demarcated lesion in the upper lateral orbital quadrant adherent to the lacrimal gland. There was no history of previous orbital trauma. In both cases histopathology confirmed a diagnosis of apocrine hidrocystoma. Following surgery, the first patient complained of mild hypoesthesia in the territory of the supraorbital nerve that resolved spontaneously within 3 weeks. Surgery was uneventful in the other patient. No recurrence was seen during the follow up., Conclusions: Apocrine hidrocystomas have been rarely described in the orbit, but should be considered in the differential diagnosis of orbital cystic masses. Recurrence is rare following complete surgical excision., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Chondroid syringoma (CS) is a benign, slow-growing mixed tumour that arises from the sweat glands and usually presents in the head and neck area. Histopathological examination is important for proper diagnosis, as CS is often confused with epidermal cysts due to its rare presentation. This article presents a man in his 40s with a right upper lip mass that emerged 6 months prior to presentation. An intraoral surgical excision was performed and the histopathological analysis revealed solid epithelial cells that formed multiple, non-branching ducts lined by cuboidal epithelium. Cystic spaces were filled by heterogeneous eosinophilic material embedded in chondromyxoid stroma. Histopathology identified the lesion as an eccrine-variant CS. The patient recovered well., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

BACKGROUND Eyelid tumors belong to a diverse group of neoplasms ranging from benign lesions to malignant tumors. Poromas are common, benign, mostly unpigmented tumors of the epidermal sweat duct unit, that usually grow slowly and occur in elderly people on the palms and soles. In most poroma cases some gene fusions were detected, which were caused by chromosomal aberrations. CASE REPORT We report the atypical case of a 30-year-old female patient suffering for more than 15 years from a solitary, polypoid, pigmented formation with a focal tuberous surface on the left lower eyelid. The lesion was not growing during the first years, but in the last 6 months before diagnosis its size more than doubled, finally reaching 12×14 mm. It was removed and histopathological analysis confirmed the diagnosis of a rare tumor - a poroma. There were no complications during healing and no recurrence was reported. CONCLUSIONS There have so far been only 9 reports of eyelid poromas, and the presented case significantly differed from the previous ones, as it appeared at an early age and showed rapid growth during a short time due to the war-related acute psychological stress. Moreover, it had unusual pigmentation and unpleasant smell. Reporting such untypical cases is clinically important because it is crucial to be aware of the diversity of eccrine poroma manifestation to distinguish it from malignant lesions.

Spiradenomas are benign cutaneous adnexal neoplasms with sweat gland differentiation that can manifest a broad spectrum of histomorphologic appearances. While they show a characteristic histopathologic phenotype and clinical management involves surgical excision with a low risk of recurrence, there have been unusual histopathologic variants of spiradenoma reported, including cases with adenoid cystic carcinoma (ACC)-like changes. Primary cutaneous ACC is a low-grade malignancy presenting as a subcutaneous mass with the potential for local invasion, perineural invasion, and high rates of local recurrence after excision. The diagnosis of spiradenomas with ACC-like features can be challenging, especially when only the ACC-like component is present for evaluation. A retrospective analysis of 21 cases of spiradenoma with ACC-like changes were obtained from large academic institutions, was performed, and summarized below. All cases showed background of conventional spiradenoma, and the ACC-like areas represented a component in all lesions. The percentage of ACC-like component ranged from 5% to 40% in all cases. The ACC-like component was multifocal and without pleomorphism, perineural and/or vascular invasion, necrosis, or increased mitotic activity. MYB translocation and protein expression was studied in 16 cases by fluorescence in situ hybridization, polymerase chain reaction, and immunohistochemistry. All studied cases were negative for MYB / NFIB , MYB L1, and MYB F by fluorescence in situ hybridization and polymerase chain reaction and 3 cases were positive for MYB expression by immunohistochemistry. Our study expands on spiradenomas with ACC-like features that ought to be considered in the differential diagnosis of cutaneous neoplasms such as primary cutaneous ACC. Our results indicate that a thorough histopathologic inspection and strict application of well-defined histologic criteria are necessary to support the diagnosis of this unusual histopathologic variant. These tumors can be difficult to diagnose, and awareness of their histomorphologic spectrum will facilitate definitive diagnosis and avoid misdiagnosis with other conditions., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Apocrine carcinoma is an extremely rare malignant cutaneous neoplasm that usually arises in areas with a high density of apocrine glands. Diagnosis can be challenging as tumours share histological and immunophenotypic characteristics with them. At first evaluation, the disease is often assumed to be benign. There have been approximately 100 reports of apocrine neoplasms in the literature. A 48-year-old male presented with a right axillary mass which increased in size over a period of 2 years. The patient was reported to have had ayurvedic therapy, but his swelling remained unchanged. Axillary lymph nodes were palpable. USG axilla suggested a well-defined fungating solid isoechoic lesion. USG neck did not reveal any abnormality. The mass was surgically excised as a whole by removing the overlying skin with margins and lymph node excision. The patient was diagnosed with primary apocrine carcinoma after surgical excision. The differentials include adenocarcinoma of breast and prostate and apocrine adenoma. There are no established standards for the care of this form of carcinoma due to its rarity and the absence of clinical studies. A literature evaluation and further reporting will aid in developing diagnostic standards and the most efficient treatment options., Competing Interests: No competing interests were disclosed., (Copyright: © 2023 Naseri DS et al.)

Apocrine hidrocystoma is a cystic tumor originating from apocrine sweat glands. It is predominantly located in the eyelid margins. Here, we report a case of apocrine hidrocystoma of the parotid gland in a 19-year-old man who was referred to our outpatient clinic with a 5-year history of a gradual swelling in the left parotid region. The patient underwent left superficial parotidectomy. Histological examination confirmed the diagnosis of apocrine hidrocystoma. The case is original by the tumor's location: to the best of our knowledge, this could be the first case in English and French literature reporting an apocrine hidrocystoma affecting the parotid gland. The purpose of this article is to report our case and discuss its clinical and anatomopathological features as well as its differential diagnoses., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The authors present the third example of an eccrine ductal carcinoma of the eyelid. A woman in her early 70s presented with a lesion of the central right lower eyelid margin in the vicinity where an actinic keratosis was diagnosed by biopsy 2.75 years previously. Her dermatologist and ophthalmologist monitored the area of actinic keratosis, and it was stable for 2.5 years until the area became ulcerated and thickened with the loss of eyelashes. A wedge resection disclosed a squamous cell carcinoma in situ and a separate eccrine ductal carcinoma. The eccrine ductal carcinoma had in situ tumor thickening, an eccrine duct component, and an invasive tumor infiltrating the tarsal plate and replacing the normal meibomian glands. The invasive eccrine ductal carcinoma only mildly thickened the tarsal plate and was most likely an incidental finding in a biopsy prompted by the squamous cell carcinoma in situ. The 5-year relative survival rate for malignant apocrine-eccrine tumors is approximately 97%, and our patient is alive and without evidence of local or distant tumor recurrence 5.5 years following the excision of her eyelid tumor., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

A rare case of pericanalicular eccrine hidrocystoma of the upper eyelid is reported in a child who underwent surgical excision and canalicular repair with a successful outcome., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Introduction: The main aim of this article is to discuss and summarize the research advancements and the treatment methods for sweat gland carcinoma (SGC) based on 2 cases of SGC in our hospital and the related literature., Case Report: This article presents 2 patients with SGC who were treated in the China Medical University, Liaoning Provincial Key Laboratory of Oral Diseases from 2007 to 2019. We analyzed the clinical features, therapies, and prognosis of the patients and searched for related literatures., Discussion: Two patients underwent extended resection for local lesions with no adjuvant radiotherapy. Neither local recurrence nor distant metastasis was detected during follow-up. Reviewing previous literature, the treatment of SGC includes surgical resection, radiotherapy, and chemotherapy. We have not found an effective treatment. The prognosis of SGC occurred in head and neck is relatively good compared with another primary-site location, primary surgical excision with safe resection margins and neck dissection is recommended.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) and primary cutaneous mucinous carcinoma (PCMC) are rare low-grade neoplasms thought to arise from apocrine glands that share many histological features and are proposed to be on a single histopathologic continuum, with EMPSGC as the in situ form that may progress to the invasive PCMC. Management involves a metastatic workup and either wide local excision (WLE) with greater than 5 mm margins or Mohs micrographic surgery (MMS) in anatomically sensitive areas. We present 2 cases of EMPSGC and 3 cases of PCMC and review their clinical and histopathologic features, differential diagnoses, and treatment.

Eccrine porocarcinoma is a rare and aggressive cutaneous malignancy that develops in the seventh and eight decades of life. We present a 76-year-old male with eccrine porocarcinoma developing from a long standing previously benign lesion who underwent successful treatment with wide local excision. It can also develop de novo, presenting most commonly as a mass or nodule. Tissue biopsy with histopathology is required to confirm the diagnosis. Wide local excision is recommended for local disease. Radiation and chemotherapy can be used as adjuncts in advanced and metastatic disease. Given its rarity, there are no guidelines to direct therapy for locally advance or metastatic disease and for follow-up. Further studies are needed to better understand and guide management of this entity.

Hidradenocarcinomas are rare cutaneous adnexal malignancies with sweat gland differentiation that can show a broad spectrum of histomorphologic appearances, ranging from low to high grade. The diagnosis of low-grade hidradenocarcinoma can be challenging and may be mistaken for benign hidradenomas, especially on superficial and partial samples. We performed a retrospective analysis of 16 low-grade hidradenocarcinomas, obtained from 4 large academic institutions. All neoplasms presented clinically as nodular lesions that ranged in size from 1.5 to 6.0 cm. All patients were adults and their age ranged from 33 to 74 years of age. All cases shared features similar to hidradenomas in the surface and mid portion of the tumors and all tumors had 1 or more histomorphologic clues to malignancy, including the presence of an asymmetric and infiltrative growth pattern (especially at the base of the tumors), perineurial invasion, and a desmoplastic stromal reaction. In the tumors evaluated for immunohistochemistry, the tumor cells were positive for p63, EMA, AE1/AE3, MNF116, and CK7. Three patients underwent sentinel lymph node biopsy, and 2 cases showed metastatic disease to regional lymph nodes. All cases (including the 2 cases that had regional lymph node metastasis), showed no local recurrence or distant metastasis observed after a complete re-excision of the tumors (follow-up range from 6 to 72 mo). Our study highlights the salient clinical and histopathologic features of low-grade hidradenocarcinomas and emphasizes the potential diagnostic pitfalls in distinguishing this entity from other neoplasms. Our results indicate that a combination of thorough histopathologic inspection is necessary to support the diagnosis of this rare neoplasm. These tumors can be exceedingly difficult to diagnose and awareness of the subtle features of low-grade hidradenocarcinoma is of importance are as it remains a diagnostic challenge for practicing pathologists., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Eccrine porocarcinoma is a rare skin adnexal tumour that affects elderly people. Most eccrine porocarcinomas are stage I or II according to the American Joint Committee on Cancer. The prognosis is good in early stages, but worsens when advanced. Since information on the use of sentinel lymph node biopsy in these patients is scarce, this study examined the records of all patients with eccrine porocarcinoma treated at Helsinki University Hospital during a 17-year period and focused on sentinel lymph node biopsy patients. The study identified 14 patients (9 male, 5 female). There were 2 metastases to the lymph nodes and 2 recurrences at initial referral to our institution. All primary tumours had wide local excision and 6 patients also had sentinel lymph node biopsy, of whom none had positive lymph nodes. There were no new metastases or recurrences during follow-up. Three patients died of causes other than eccrine porocarcinoma. When comparing the wide local excision only and wide local excision with sentinel lymph node biopsy groups, no parameters reached statistical significance. The decision process of the multidisciplinary tumour board meeting on whether to perform sentinel lymph node biopsy was not clear, perhaps due to the limited knowledge of eccrine porocarcinoma. Further studies and international collaboration are warranted.

Eccrine porocarcinoma is a rare malignant tumor of the eccrine sweat gland. This malignancy occurs most commonly in the lower extremities. It tends to occur in patients aged 60 to 80 years, affecting men and women equally. We present the case of a 62-year-old man with a lesion on the left foot. The diagnosis of the initial biopsy was squamous cell carcinoma. Six months later, the lesion reoccurred, and a second biopsy confirmed it to be eccrine porocarcinoma.

Background: Hidradenocarcinoma is a rare malignant sweat gland tumour, characterized by a slow but aggressive course, with high rates of local recurrence and metastasis. Due to its rarity, histological criteria and therapeutic guidelines are poorly defined, posing a major challenge for clinicians and pathologists., Objectives: To present two new cases of metastatic hidradenocarcinoma as well as a review of the literature., Materials & Methods: We describe two case studies and a review of the literature based on a search using the MEDLINE (PubMed) electronic database., Results: The first patient was a 61-year-old woman with a perimamillary hidradenocarcinoma that arose from the malignant transformation of a benign childhood lesion and developed regional lymph node metastases after wide excision and adjuvant radiotherapy. The second patient was a 63-year-old man who developed cutaneous and renal metastases several years after the complete excision of a lumbar hidradenocarcinoma. As far as we can ascertain, kidney metastasis from hidradenocarcinoma has not previously been described., Conclusion: Most authors recommend wide excision as the treatment of choice for hidradenocarcinoma, however, optimal adjuvant therapy remains to be determined. Our cases add to the limited knowledge available, but high-quality studies to find new effective treatments are needed.

Although chondroid syringoma rarely occurs outside the head and neck, the majority of malignant chondroid syringomas are identified in the extremities. Here, we present a case of atypical chondroid syringoma in the fifth toe. Diagnosis of chondroid syringoma with atypical cells was made following initial excisional biopsy and histology, necessitating repeated surgery for positive margins. In this case report, we examine the radiopathologic correlation of this diagnosis, detail the imaging findings of benign and malignant chondroid syringomas, and highlight how magnetic resonance imaging can be used to guide surgical planning and treatment course of this potentially malignant tumor.

Abstract: Hidradenocarcinoma (HAC) is a rare adnexal tumor associated with the potential for locoregional recurrence and systemic metastasis. The clinical appearance of HAC is nonspecific, frequently presenting as a solitary firm subcutaneous nodule or plaque on the head and neck region or distal extremities. These tumors show histomorphologic heterogeneity, as they can be low and high grade. Distinguishing HAC from hidradenoma, especially the low-grade variant of HAC, can be challenging as both tumors can show histologic overlapping features. In this article, we describe a case of a 33-year-old patient presenting with a low-grade HAC of the plantar foot who was subsequently found to have lymph node metastasis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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.

Abstract: A 43-year-old woman presented with a palpable, pruritic, minimally painful right vulvar lesion. Physical examination revealed approximately 2.0-cm tender nodule at 70' clock in the right labia majora. Histological sections of the excision specimen showed an unremarkable epidermis with large, well-circumscribed dermal proliferation with extension to the reticular dermis. Within this proliferation are small solid and ductal structures relatively evenly distributed in the sclerotic stroma. The epithelial elements consisted of monomorphous cuboidal cells and assumed round, oval, curvilinear, or have other peculiar geometric shapes, including "comma-like" or "tadpole"-like configurations. The tumor cells were positive for CEA, EMA, and estrogen receptor and negative for progesterone receptor. The clinical presentation and the deep extension of the tumor were similar to the microcystic adnexal carcinoma. Although a syringoma generally presents with multiple lesions and usually involves the superficial dermis, a syringoma with deep extension was favored based on the lack of follicular differentiation, atypia, mitoses, and perineural invasion. Microcystic adnexal carcinoma and syringoma have a morphologic overlap and are misdiagnosed in 30% of the cases. Thus, it is exceptionally important for pathologists to be aware of and be able to distinguish these entities. To the best of our knowledge, this is the first case of a solitary, painful vulvar syringoma with deep extension., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Eccrine syringofibroadenoma (ESFA) is a rare benign skin adnexal lesion of the acrosyringium of eccrine sweat ducts. Reactive ESFA, a subtype of ESFA, is usually associated with non-neoplastic cutaneous dermatoses or neoplastic skin tumors. Clinically, the lesions can be solitary or multiple, pink, or skin-colored coalescing papules or nodules of variable sizes. Histopathologically, this tumor is composed of numerous anastomosing cords of monomorphic cuboidal epithelial cells with eccrine duct formation. The association of reactive ESFA with benign conditions, such as psoriasis, diabetic polyneuropathy, scars, and leprosy, has been reported. However, the association of reactive ESFA with malignant tumors is extremely rare, with very few cases reported in the literature. We present a case of a 72-year-old woman who developed reactive ESFA associated with Merkel cell carcinoma excision scar. The ESFA tumors developed in the area of the surgical graft 10 months after the Merkel cell carcinoma had been excised. New ESFA tumors have continued to appear in the scar on a yearly basis while, so far, has been no recurrence of the original tumor. However, the presence of new tumor growths in the area suggested the possibility of recurrence of the Merkel cell carcinoma. That possibility was enhanced by the fact that PET scans revealed hypermetabolic activity in the ESFA papules., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Hidradenoma papilliferum (HP) is a rare benign tumor of the apocrine gland and is commonly seen at the anogenital region. A 32-year-old post-hysterectomy woman presented with excessive vaginal discharge and itching. Small polypoidal mass was noted at vault. Excision of the lesion was done, and histopathology confirmed HP. The patient is symptom free following excision. This case report highlights the presence of pluripotent cells in the vagina and their differentiation into any cell lineage., (Copyright © 2023 Copyright: © 2023 Journal of Cancer Research and Therapeutics.)

Background: Mohs micrographic surgery is an established technique in the treatment of cutaneous neoplasms. It offers higher cure rates and the main indications are non-melanoma malignant skin tumors. Few studies have been performed on the treatment of rare tumors through this technique., Objective: To study rare skin tumors and rare variants of basal cell carcinoma and squamous cell carcinoma submitted to Mohs micrographic surgery in a tertiary service in relation to frequency, disease-free evolution, and applicability of this surgical procedure for this group of tumors., Methods: This was a retrospective observational study including rare skin tumors and less common variants of basal cell carcinoma and squamous cell carcinoma treated using Mohs micrographic surgery, between October 2008 and April 2021., Results: During the study period, 437 tumors were treated using Mohs micrographic surgery, and 22 (5%) rare skin tumors were selected. The tumors comprised three dermatofibrosarcomas protuberans, two atypical fibroxanthomas, two spiradenomas, two hypercellular fibrohistiocytomas, one primary cutaneous adenocarcinoma, one trichoblastoma, one porocarcinoma, one chondroid syringoma, one cutaneous angiosarcoma, one Merkel cell carcinoma, and one sebaceous carcinoma. Six other cases of rare basal cell carcinoma variants with trichoepitheliomatous differentiation, metatypical basal cell carcinoma, and clear cell squamous cell carcinoma were included. There were no cases of recurrence after an average of six years of follow-up., Study Limitations: This is a retrospective study on rare neoplasms carried out in a single referral center, and this surgical technique isn't widely available in the public service., Conclusion: This retrospective case series showed that Mohs micrographic surgery is an appropriate treatment for rare skin tumors. They corresponded to 5% of the tumors treated by the technique during a 12-year-period, with no recurrences identified., (Copyright © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)

Syringomas are benign adnexal neoplasms that may induce psychological stress when they are large or disfiguring or present in delicate regions such as the periorbital area. Despite the availability of various lasers for syringomas, no consensus has been established on the optimal laser setting and side effects of these therapies. The current review aims at understanding the efficacy and safety of various laser therapies available for the treatment of syringomas. A literature search was carried out using PubMed and Ovid databases for articles published from Jan 2000 through Mar 2022. Screening the eligible articles yielded 27 studies, comprising clinical studies, case series, and case reports, which were included in this review. The CO 2 laser is the most widely used ablative laser therapy but is usually associated with adverse events. Pinhole and multiple drilling methods using CO 2 laser yielded excellent cosmetic results with minimal adverse effects. Fractional lasers reduced the downtime and complications compared to non-fractionated ones. Non-ablative fractional lasers could be advantageous in terms of easy operation, minimal side effects and moderate recovery period compared with ablative lasers. Large clinical trials are needed to generate strong evidence to guide clinicians in choosing the most appropriate laser therapy for syringoma treatment.

Digital papillary adenocarcinoma (DPA) is a rare but locally aggressive eccrine tumour that has metastatic potential and most frequently found in the upper extremity. It usually presents as an unremarkable swelling of the skin, commonly misdiagnosed as a benign lesion. We present a 67-year-old male who presented with a slowly growing mass of the right middle finger that was biopsied and diagnosed with DPA. The patient was treated with wide local excision (WLE) and sentinel lymph node biopsy by us during the same operative encounter. This report highlights the need for increased clinical vigilance to diagnose DPAs, and that WLE and sentinel lymph node biopsies can be safe and effective treatments offered. Level of Evidence: Level V (Therapeutic).

Abstract: Sweat gland carcinoma with neuroendocrine differentiation (SCAND) is a newly proposed tumor entity of primary cutaneous apocrine/eccrine adnexal tumor with neuroendocrine differentiation. The histopathologic variations are not yet well known. In this article, we present a case of SCAND mimicking male breast cancer and syringocystadenocarcinoma papilliferum. A 68-year-old man presented with a reddish 12-mm nodule on his left areola. No lymph node or distant metastases were observed. The patient was disease free 1 year and 9 months after the tumor was surgically resected but died of cerebral hemorrhage. Histopathological examination revealed a predominantly intradermal tumor with marked syringotropism, mimicking a component of mammary ductal carcinoma in situ. In addition, another tissue section displayed a cup-shaped papillated tumor with syringocystadenocarcinoma papilliferum-like features, which were also seen because of marked syringotropism. Diffuse immunoexpression of cytokeratin 7, cytokeratin 19, chromogranin A, synaptophysin, INSM1, estrogen receptor, carcinoembryonic antigen, epithelial membrane antigen, and GATA3 was observed in the tumor, but no BRAF immunoexpression was seen. The present case would help us to understand the histopathological variation and differential diagnosis of SCAND. The histopathological diagnosis of male breast cancer or syringocystadenocarcinoma papilliferum should be made by ruling out SCAND., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Syringocystadenoma papilliferum is a rare hamartomatous adnexal tumor and appears mainly in the head and neck region. Rarely, such tumors may appear in the external auditory canal. There are only 14 described cases of the syringocystadenoma papilliferum in the external auditory canal in the Medline database. In this study, we present the first reported case of syringocystadenoma papilliferum arising in the modified radical mastoidectomy and occurring 12 years after ear surgery. A 26-year-old man was admitted to our clinic with complaints of discharge from the left ear, ear fullness, and hearing loss. These symptoms had been worsening for 3 years. Twelve years previously, he had undergone modified radical mastoidectomy by Bondy due to a cholesteatoma. Otoscopic examinations revealed otorrhea and a reddish mass measuring 1 × 0.8 cm arising from the tegmen tympanum and fundus of the mastoid cavity. A surgical biopsy was conducted with local anesthesia in the medical outpatient clinic, and it revealed syringocystadenoma papilliferum. A tumor resection was performed with a retroauricular approach. The bone attachment of the tumor was drilled out, and the ear cavity was covered with a mashed split skin graft. The ear cavity healed, and no tumor recurrence was observed during a 9-month follow-up period. Our case and literature analysis of previously described cases led to the conclusion that cases of syringocystadenoma papilliferum of the external auditory canal may be associated with different medical histories, age distributions, and origins than other syringocystadenoma papilliferums of the head and neck region. Tubular adenoma may occur together with syringocystadenoma papilliferum in 20% of cases. Malignant transformation of syringocystadenoma papilliferum may occur but is extremely rare. Computed tomography imaging may predict malignancy of the tumor. Tumors specific to the external auditory canal may occur in the modified radical mastoidectomy after middle ear surgery.

Nodular hidradenoma is a cutaneous adnexal tumor of sweat gland origin, characterized by its diverse but overlapping histomorphologic features with other skin tumors. In addition, distinction of benign hidradenoma and its malignant counterpart hidradenocarcinoma can be challenging, especially in prognostic prediction. We retrospectively reviewed pathological features of 29 cases, including benign nodular hidradenoma (n = 17) and hidradenocarcinoma (n = 12), with clinical follow-up ranging from 18 to 216 months. Genomic copy number variation (CNV) was studied in selected cases (n = 18) by single nucleotide polymorphism microarray. None of the benign hidradenomas (0/17) or low-grade hidradenocarcinomas (0/6) had recurrence or metastasis after complete excision, whereas all 6 high-grade hidradenocarcinomas (6/6) showed locally destructive disease, recurrence, or local metastases. In benign hidradenomas, CNV abnormality was absent in all clear cell hidradenomas (0/5) but was detected in a considerable portion of poroid hidradenoma (3/5), with number of abnormalities ranging 2, 4, and 9. In malignant cases, regardless of morphological classification, both low-grade hidradenocarcinomas demonstrated limited CNV abnormalities in 2 areas (2/2), whereas all high-grade hidradenocarcinomas contained 8 or more CNV abnormalities (6/6). No disease-associated death was recorded in the cohort except one case was lost to follow-up after the development of metastatic disease. Overall, the findings support that genomic CNV abnormalities may serve as a sensitive but less specific tool in detecting malignancy in these tumors, and potentially have a role in predicting clinical behavior particularly in the tumors of nonporoid morphology., (Copyright © 2022 Elsevier Inc. All rights reserved.)