Temnithikul Bhakinai, Rungrunanghiranya Suthat, Limtanyakul Piyakan, Jerasuthat Suthep, Paige David G
Department of Internal Medicine, Faculty of Medicine Srinakharinwirot University Bangkok Thailand.
Department of Dermatology Chulabhorn Royal Academy Bangkok Thailand.
Skin Health Dis. 2022 Apr 28;2(2):e118. doi: 10.1002/ski2.118. eCollection 2022 Jun.
Primary cutaneous adenoid cystic carcinoma (PCACC) is an uncommon adnexal skin tumour with fewer than 200 cases studied in detail in the English literature. We describe the diagnosis and treatment of a few Southeast Asian cases of PCACC on the scalp of a 70-year-old Thai female. She presented with a slow-growing, painless, solid to cystic, skin-coloured tumour on her scalp. When excisional biopsy was done, histopathological findings showed dermal tumour that had a classic histologic appearance composed of basaloid cells arranged in a cribriform pattern with 'punched-out' pseudocysts filled with mucin (swiss cheese pattern) and had perineural invasion. The clinical and histopathological findings, and complete investigations confirmed the diagnosis of PCACC. Our case illustrates that PCACC, is an important histopathological differential diagnosis to bear in mind due to its locally aggressive nature and tendency to recur due to perineural invasion. A wide local excision with at least 2 cm of tumour-free margins was performed, which revealed no residual carcinoma. The patient remained disease-free for 16 months after diagnosis. PCACC is usually located on the head or neck of people in their sixth decade of life, with a female predominance. The aetiology of PCACC is unclear. The majority of PCACCs have the fusion gene or show overexpression of by immunohistochemistry. Diagnosis of PCACC is primarily based on the characteristic histological appearance, as there are no distinguishing clinical features. The diagnosis of PCACC requires careful exclusion of infiltration or metastasis from other primary lesions. Treatment of this rare tumour is wide surgical excision with at least 2 cm of tumour-free margins to reduce the risk of local recurrence, and long-term follow-up for possible recurrence of PCACC is recommended. This case emphasizes the importance of careful inspection for the diagnosis of PCACC after initial surgery and pathological evaluation of the mass lesion for appropriate diagnosis and therapy.
原发性皮肤腺样囊性癌（PCACC）是一种罕见的皮肤附属器肿瘤，英文文献中详细研究的病例不足200例。我们描述了一名70岁泰国女性头皮上几例东南亚PCACC病例的诊断和治疗情况。她头皮上出现了一个生长缓慢、无痛、实性至囊性、肤色的肿瘤。进行切除活检时，组织病理学检查结果显示真皮肿瘤具有典型的组织学表现，由呈筛状排列的基底样细胞组成，有充满黏液的“筛孔状”假囊肿（瑞士奶酪样），并伴有神经周围浸润。临床和组织病理学检查结果以及全面的检查证实了PCACC的诊断。我们的病例表明，由于PCACC具有局部侵袭性且因神经周围浸润有复发倾向，因此是一个需要牢记的重要组织病理学鉴别诊断。进行了至少2厘米切缘无瘤的广泛局部切除，未发现残留癌。患者诊断后16个月无疾病复发。PCACC通常位于60岁左右人群的头颈部，女性居多。PCACC的病因尚不清楚。大多数PCACC有融合基因或通过免疫组织化学显示其过表达。PCACC的诊断主要基于特征性的组织学表现，因为没有明显的临床特征。PCACC的诊断需要仔细排除其他原发性病变的浸润或转移。治疗这种罕见肿瘤需进行至少2厘米切缘无瘤的广泛手术切除以降低局部复发风险，建议对PCACC可能的复发进行长期随访。该病例强调了初次手术后仔细检查对PCACC诊断的重要性，以及对肿块病变进行病理评估以进行恰当诊断和治疗的重要性。
