Abstract:Primary cutaneous adenoid cystic carcinoma (PCACC) is a very rare malignant tumor. Here a case of PCACC on the left lower leg with metastasis to the inguinal lymph node. The tumor resection and the inguinal lymph node dissection were performed under general anesthesia, and the defect was covered with free meshed skin graft. No complication or recurrence has occurred after the surgery.
Introduction: Primary cutaneous adenoid-cystic carcinoma (PCACC) is a rare malignant tumour reported in only about 450 cases in the literature, with only two adolescent cases reported. PCACC seems to occur between the fifth and seventh decade of life, and the most frequent regions involved are head and neck (46%). Aplasia cutis congenita (ACC) has an incidence of 1:10,000, and it seems to be rarely associated with neoplastic lesions. Interestingly, the association between PCACC and ACC has, so far, never been described. Methods: We report a case of PCACC in the scalp associated with ACC in a four-year-old patient. Discussion: The patient was under follow-up at the dermatology unit, but suddenly a red lesion appeared within the ACC. This red, ulcerated area increased rapidly over six months, so it was surgically removed, and the pathological examination results were suggestive for cribriform PCACC. According to the guidelines for skin tumours, the patient underwent widening resection, and an advancement-sliding skin flap was performed to recreate the scalp. After one year of follow-up, the patient has no local or widespread recurrence of the PCACC, and the surgical scar appears to have healed well. Conclusions: This clinical case is the first known patient with PCACC associated with ACC. A skin excision biopsy should be performed with wide margins to avoid a second widening resection of skin in a similar scenario. Genetic studies may help to identify the origin of this rare association.
Introduction: Primary cutaneous adenoid-cystic carcinoma (PCACC) is a rare malignant tumour reported in only about 450 cases in the literature, with only two adolescent cases reported. PCACC seems to occur between the fifth and seventh decade of life, and the most frequent regions involved are head and neck (46%). Aplasia cutis congenita (ACC) has an incidence of 1:10,000, and it seems to be rarely associated with neoplastic lesions. Interestingly, the association between PCACC and ACC has, so far, never been described. Methods: We report a case of PCACC in the scalp associated with ACC in a four-year-old patient. Discussion: The patient was under follow-up at the dermatology unit, but suddenly a red lesion appeared within the ACC. This red, ulcerated area increased rapidly over six months, so it was surgically removed, and the pathological examination results were suggestive for cribriform PCACC. According to the guidelines for skin tumours, the patient underwent widening resection, and an advancement-sliding skin flap was performed to recreate the scalp. After one year of follow-up, the patient has no local or widespread recurrence of the PCACC, and the surgical scar appears to have healed well. Conclusions: This clinical case is the first known patient with PCACC associated with ACC. A skin excision biopsy should be performed with wide margins to avoid a second widening resection of skin in a similar scenario. Genetic studies may help to identify the origin of this rare association.
“…2,4 Less often, it can be found on the trunk 6–8 or extremities. 9 Unlike ACC that can metastasize to lungs, bones and soft tissues, PCACC rarely metastasizes 10 but can display local perineural involvement with a tendency to reoccur locally. 11 As PCACC might resemble micronodular basal cell carcinoma (BCC) on histopathology, distinctive features help to rule out other differentials.…”
Adenoid cystic carcinoma is predominantly a tumor of the parotid glands and can sometimes be found in other glands. In most cases, skin location is usually a metastatic presentation and rarely a primary tumor. We describe the case of a 59-year-old female patient presenting with a 5-mm skin-colored nodule on the abdomen histologically compatible with a primary cutaneous adenoid cystic carcinoma. Extensive workup revealed no other primary source, nor evidence of metastatic disease; therefore, wide local excision was the preferred treatment given the low potential of recurrence. As this adnexal carcinoma is rare and its morphology non-specific clinically, we wanted to raise awareness of this entity and its management.
“…Primary cutaneous adenoid cystic carcinoma (PCAdCC) is a rare entity, with only 131 cases previously reported in the English literature, after exclusion of cases occurring in the periocular region and ear canal 1‐8 . As compared to its counterpart in salivary gland, it tends to follow a more indolent course, often with multiple recurrences 9,10 .…”
Section: Figurementioning
confidence: 99%
“…Histopathology of primary cutaneous adenoid cystic carcinoma of the scrotum presenting with predominantly solid growth Sarah E. Gradecki MD 1 | Alejandro A. Gru MD 1 | Kerri E. Rieger MD PhD 2 | Shyam S. Raghavan MD 1 Primary cutaneous adenoid cystic carcinoma (PCAdCC) is a rare entity, with only 131 cases previously reported in the English literature, after exclusion of cases occurring in the periocular region and ear canal. [1][2][3][4][5][6][7][8] As compared to its counterpart in salivary gland, it tends to follow a more indolent course, often with multiple recurrences. 9,10 The majority of PCAdCCs are found in the head and neck region or trunk.…”
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