Pedunculated eccrine poromas on non-acral sites.

A woman in her mid-50s, a patient with metastatic right breast carcinoma, postradical mastectomy and chemoradiation on hormonal therapy, presented with asymptomatic reddish lesions over the neck and trunk. Cutaneous examination revealed three discrete pedunculated, non-tender, firm erythematous growths with smooth surfaces over the neck, chest and abdomen. Histopathological examination was done with the differentials of pyogenic granuloma, haemangioma, giant acrochordon and vascular metastasis, revealing the diagnosis of eccrine poroma (EP). The remaining lesions were removed by electrocautery with no relapse till 1 year of follow-up. EP is a rare benign neoplasm arising from the acrosyringium that commonly presents as solitary, sessile or pedunculated asymptomatic papules or nodules over the palms and soles. Here we report the rare occurrence of multiple pedunculated EPs on a non-acral site in a patient who received chemoradiotherapy, which clinically mimicked pyogenic granuloma and vascular metastases. Thus, emphasising the importance of considering EP as a differential in lesions with vascular morphology.

Porocarcinoma: Clinical and Histological Features, Immunohistochemistry and Outcomes: A Systematic Review.

Porocarcinoma (PC) is a rare adnexal tumor, mainly found in the elderly. The tumor arises from the acrosyringium of eccrine sweat glands. The risk of lymph node and distant metastasis is high. Differential diagnosis with squamous cell carcinoma is difficult, although NUT expression and YAP1 fusion products can be very useful for diagnosis. Currently, wide local excision is the main surgical treatment, although Mohs micrographic surgery is promising. To date, there is no consensus regarding the role of sentinel lymph node biopsy and consequential lymph node dissection. No guidelines exist for radiotherapy, which is mostly performed based on tumor characteristics and excision margins. Only a few studies report systemic treatment for advanced PC, although therapy with pembrolizumab and EGFR inhibitors show promise. In this review, we discuss epidemiology, clinical features, histopathological features, immunohistochemistry and fusion products, surgical management and survival outcomes according to stage, surgical management, radiotherapy and systemic therapy.

Syringocystadenocarcinoma papilliferum: a systematic review of clinical characteristics, reappraisal of associations, diagnostic pitfalls and management challenges.

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.

Based on Immune Microenvironment and Genomic Status, Exploring Immunotherapy in Advanced Hidradenocarcinoma: A Retrospective Analysis.

There are no standard treatment guidelines for hidradenocarcinoma, and the immune microenvironment and genomic data are very limited. Thus, in this study the immune microenvironment and genomic indicators in hidradenocarcinoma was investigated, and immunotherapy for hidradenocarcinoma was initially explored. Forty-seven hidradenocarcinoma patients were retrospectively collected. Immunohistochemical staining was performed to identify CD3/CD8+ T cells and programmed death ligand-1 expression. In total, 89.4% and 10.6% of samples had Immunoscores of 0-25% and 25-70%. Tumour proportion score distribution was as follows: tumour proportion score < 1% in 72.4%, 1-5% in 17.0%, and > 5% in 10.6%. Combined positive score distribution was as follows: combined positive score < 1 in 63.8%, 1-5 in 14.9%, and > 5 in 21.3%. Next-generation sequencing revealed that TP53 (33%), PI3KCA (22%), and ERBB3 (22%) were the most frequently mutated genes. The PI3K-Akt signalling pathway, growth, and MAPK signalling pathways were significantly enriched. Five patients had a low TMB (< 10 muts/Mb), and 9 patients had MSS. Three patients treated with immune combined with chemotherapy achieved significant tumour regression, and the progression-free survival was 28.8 months. In conclusion, the hidradenocarcinoma immune microenvironment tends to be noninflammatory. Evidence-based targets for targeted therapy are lacking. Immunotherapy combined with chemotherapy may be better for most advanced hidradenocarcinoma patients with a noninflammatory microenvironment.

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.

Er:YAG laser combined with botulinum toxin A for patients with local syringomas: A preliminary report.

Syringoma is a common but refractory benign skin tumor. Conventional treatment, such as ultra-pulsed carbon dioxide (CO2 ) laser or cryotherapy, often requires multiple treatment and can easily cause prolonged erythema, scarring, or depression, which are frustrating, so there is an urgent need to seek a safer and more effective method. In this article, we tried to demonstrate the Er:YAG laser combined with botulinum toxin A (BTXA) as a safer and more efficacious method for treating syringomas. MATERIALS AND METHODS: Twenty-one patients with local syringomas were treated with erbium laser ablation. Immediately after laser treatment, approximately 10 units of BTXA were sprayed on the wound for 10 min. RESULT: In total, 21 patients underwent 1.62 ± 0.74 treatments; their Periorbital Syringoma Severity Index (PSSI) score declined from 4.19 (before treatment) to 1.10 (after treatment), and the number of treatments was significantly lower than those reported in previous literature using the erbium laser alone. CONCLUSION: The Er:YAG laser combined with botulinum toxin A for the treatment of syringoma is a safer and more effective treatment than traditional treatment methods.

Porocarcinoma: an epidemiological, clinical, and dermoscopic 20-year study.

BACKGROUND: Porocarcinoma (PC) is a rare cutaneous adnexal tumor with a variable metastatic potential. Given the paucity of data, guidelines and specific recommendations for PC are not yet well-established. In this study, we evaluate the disease-specific characteristics and outcome of this rare and often underestimated tumor. MATERIALS AND METHOD: A retrospective study of the epidemiological, clinical, and dermoscopic characteristics among cases of histopathologically diagnosed PC, collected from the database of two skin cancer clinics in Italy (Firenze, Pistoia) from 2000 to 2020, was conducted. RESULTS: Among the 52 patients with 53 tumors, 31 were men (59.6%) and 21 were women (40.4%) with an age range of 49-96 years (median age 82 years). The most common locations were the head/neck region in men (34% in men vs. 17% in women) and the lower limb in women (17% in women vs. 9% in men). Forty-eight cases (91%) underwent local excision. Of these patients, two (4%) experienced local recurrence, and one (2%) developed a second PC on a different anatomical site 1 month after the primary tumor's excision. Lymph node metastases were present in three cases (6%). Two of them have been treated surgically with adjuvant radiotherapy (both are disease-free after a 2-year follow-up period), whereas the third case developed visceral metastases followed by PC-related death. CONCLUSIONS: This study, with 52 patients with 53 tumors covering a follow-up period of more than 5 years, shows a less aggressive behavior of PC with 4% local recurrence, 6% nodal metastases, and 2% mortality.

Primary cutaneous apocrine carcinoma of the scalp: Two case reports and literature review.

RATIONALE: Apocrine carcinoma is a rare malignant sweat gland tumor that has been reported in approximately 200 cases. This tumor usually occurs in the axilla, but in rare cases, it can also develop in the scalp. In the present work, we report 2 cases of cutaneous apocrine carcinoma of the scalp. PATIENT CONCERNS: Two men visited our outpatient clinic with recurrence of tumor after undergoing surgery for scalp tumor at another hospital. DIAGNOSES: Brain magnetic resonance imaging of a 56-year old man showed the presence of a 5.0 × 4.5 × 4.4 cm scalp mass in the right parietal region, invading the skull and dura mater and a 2.2 × 2.0 × 0.7 cm bony mass without any skin lesions right next to the scalp mass. Neck magnetic resonance imaging of a 76-year-old man revealed the presence of a well-defined oval mass in the subcutaneous layer of the left occipital scalp and 2 enlarged lymph nodes in the left neck. Definite diagnoses were made postoperatively. The patients were diagnosed with cutaneous apocrine carcinoma. The diagnosis was confirmed through histopathological and immunohistochemical staining tests. INTERVENTIONS: The tumors were removed with a wide safety margin and reconstructive surgery was performed. OUTCOMES: Additional radiotherapy or chemotherapy was performed. Follow-up more than 6 months revealed no recurrence or metastasis. LESSONS: If accurate diagnosis and treatment had taken place at the initial stages of the primary cutaneous apocrine carcinoma, it would have been possible to prevent recurrence and intracranial invasion. As recurrent primary cutaneous apocrine carcinoma can become aggressive and difficult to treat, even a small mass on the scalp must be evaluated carefully and treated properly.

[Management of malignant adnexal neoplasms of the skin]. / Management von malignen Adnextumoren der Haut.

Malignant adnexal neoplasms of the skin are a heterogeneous group of rare malignancies with eccrine, apocrine, sebaceous and follicular differentiation. Essential clinical practice recommendations for the overall management of these cancers are presented. Moreover, specific evidence-based findings according to diagnosis, therapy and follow-up of porocarcinoma, sebaceous carcinoma and microcystic adnexcarcinoma will be explained.

Apocrine hidrocystoma: a slowly growing postauricular translucent nodule.

Apocrine hidrocystoma is a benign, cystic proliferation of the apocrine sweat gland that may present commonly on sun-exposed areas of the head and neck. However, given its location and features, apocrine hidrocystomas may often be confused with malignant tumors such as basal cell carcinomas or primary cutaneous mucinous carcinomas. Herein, we present an unusual case of an apocrine hidrocystoma presenting in the postauricular region and highlight the importance of histopathological examination of cystic tumors on the periauricular area.

Eccrine porocarcinoma: case report and review of the literature.

Eccrine porocarcinoma is a rare skin cancer that originates from the acrosyringium of eccrine sweat glands. From the clinical point of view the differential diagnosis with other skin cancers such as basal cell carcinoma and squamous cell carcinoma it is often impossible, only the histopathologic features can lead to the definitive diagnosis. Eccrine porocarcinoma can arise from a previous poroma or de novo, it may recur after surgical excision and cause lymph node and visceral metastasis. There are no international guidelines for treatment or follow-up of patients. The aim of this work was to present a rare case of eccrine porocarcinoma of the scalp successfully treated in our clinic and to extrapolate from the international literature the main clinical and histopathological features of eccrine porocarcinoma and the various experiences regarding the types of treatment.

Invasive apocrine carcinoma of the breast: clinicopathologic features and comprehensive genomic profiling of 18 pure triple-negative apocrine carcinomas.

Pure invasive apocrine carcinoma is a rare type of primary breast cancer, constituting ~1% of all breast cancers. Since most pure invasive apocrine carcinomas are triple negative, the lack of targeted therapies for triple-negative breast cancer has fostered efforts to discover actionable molecular targets in these tumors. In this study, we analyzed the clinicopathologic characteristics and comprehensive genomic profiling of 18 patients with pure triple-negative apocrine carcinomas (TNACs) using a 324-gene panel assay (FoundationOne CDx). The median age of these patients was 55.5 years, and the postmenopausal status rate was 77.8%. In total, 83.3% of patients were diagnosed with histological grade II, and 16.7% were diagnosed with grade III. The majority of patients presented at an early tumor-node-metastasis (TNM) stage (I: 38.9%; II: 50.0%; and III: 11.1%). The mean Ki-67 index was 9.7%, and the percent of PD-L1 positivity was 11.7%. With a median follow-up period of 76.5 months, one patient died, and two experienced distant metastases. There were 61 clinically relevant genomic alterations among all 18 pure TNACs, and the mean tumor mutation burden (TMB) was 3 Mut/Mb. The top ranked altered genes were PIK3CA (72.2%), PTEN (33.3%) and TP53 (27.8%). There were four novel mutations found in PTEN and an actionable rearrangement involving FGFR2-TACC2 that has not been reported in breast cancer before. In total, 88.9%, 50%, 44.4%, and 16.7% of TNACs had at least one clinically relevant genomic alteration in genes involved in the PI3K/mTOR, cell cycle, RAS/RAF/MEK and growth factor receptor-related pathways, respectively. All patients had at least one clinically relevant genomic alteration, and 94.4% had at least one actionable alteration. To the best of our knowledge, this study is the largest genomic sequencing cohort of pure TNACs. Incorporation of comprehensive genomic profiling into TNACs might shed light on potential therapeutic opportunities for both targeted drugs and immune checkpoint inhibitors.

An Update on Endocrine Mucin-producing Sweat Gland Carcinoma: Clinicopathologic Study of 63 Cases and Comparative Analysis.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated (neuroendocrine-type) MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as "EMPSGC" in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was ~21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

Comparative Efficacy of Hidrocystoma Treatments: A Systematic Review.

INTRODUCTION: Although various treatment options for hidrocystomas have been described, the comparative efficacy of these treatments is poorly understood. METHODS: We conducted a systematic review of all articles describing the treatment of hidrocystomas. Treatment modalities were categorized as destructive surgical procedures, skin-directed therapies, systemic medical therapies, general measures, or combined. Patient and tumor characteristics, as well as response rate, recurrence rate, and adverse effects, were extracted from each article. RESULTS: A total of 94 articles involving 192 patients and 255 unique treatment events were included in the final analysis. Destructive surgical procedures had an overall response rate and recurrence rate of 92.9% and 10.8%, respectively. Skin-directed therapies had an overall response rate of 72.6%. The overall response rate to systemic medical therapies was 71.4%. Solitary hidrocystomas were primarily treated with destructive surgical procedures, including excision, which was associated with a 4.7% recurrence rate. Multiple hidrocystomas were successfully treated with a variety of therapies, including destructive surgical procedures and skin-directed therapies requiring ongoing or repeated therapy. CONCLUSIONS: Excision has the highest efficacy for solitary hidrocystomas. A number of therapies have shown efficacy for multiple hidrocystomas, including lasers, intracystic trichloroacetic acid, intracystic hypertonic glucose, topical and oral anticholinergics, and botulinum toxin. Aluminum chloride is associated with a low response rate. Larger comparative studies are needed to further evaluate the optimal treatments for solitary and multiple hidrocystomas.

Treatment of a case with giant eyelid syringomas.

He had a history of multiple asymptomatic flesh-colored papules, which had slowly increased in number and coalesced into plaques, on both upper and lower eyelids. Some of the plaques were giant and mostly coalescent in nature. We excised surgically the giant plaques which were approximately 1.5-2 cm in diameter symmetrically from upper and lower eylids and the remaining plaques were left to be treated with cauterization. Histopathological examination revealed syringoma. Both of the eyelids were observed to have a cosmetically adequate appearence after the surgical intervention.

Prognostic factors and survival outcomes for head and neck cutaneous adnexal cancers.

OBJECTIVES: Malignant cutaneous adnexal tumors (MCAT) are rare and comprise a heterogeneous group of cancers. There have been several studies reviewing prognostic factors of these tumors, but no studies focusing on the head and neck. This study aimed to review a large population based database to evaluate prognostic factors that could impact survival. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify patients with MCAT of the head and neck. Both overall and disease specific survival were the main outcome measures for the study. Univariate and multivariate analyses were performed to evaluate the association of suspected prognostic factors with survival. RESULTS: The five-year OS and DSS were 72.6 and 95.5%, respectively. A favorable factor for OS was surgical resection ([HR] 0.324; P = 0.001), while unfavorable factors for OS include older age (1.051; P < 0.001), higher tumor grade (1.254; P = 0.049), larger tumor size (1.293; P = 0.003), and positive nodal involvement (3.323; P = 0.002). A favorable factor for DSS was surgical resection (0.026; P < 0.001). Unfavorable factors for DSS include older age (1.058; P = 0.046), larger tumor size (2.528; 1.565-4.085; P < 0.001), and positive nodal involvement (4.761; P = 0.022). CONCLUSION: Review of the SEER database shows good 5-year OS and DSS rates, similar to those cited in other studies. We identified several prognostic factors associated with survival, while histologic sub-type does not seem to be associated with survival. Surgical resection is the mainstay of treatment.