Answer of Dermatopathology Case 7

Primary Cutaneous Adenoid Cystic Carcinoma

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Abstract:

Primary cutaneous adenoid cystic carcinoma. J Am Acad Dermatol. 2008 Apr;58(4):636-41.
Primary cutaneous adenoid cystic carcinoma is a rare, slow-growing malignancy first described by Boggio in 1975. This tumor characteristically consists of basophilic cells with a distinct adenoid or cribriform pattern in the mid to deep reticular dermis. Modified myoepithelial cells with prominent basement membrane material often surround true lumina. Definitive diagnosis relies on the characteristic histologic features and the exclusion of metastatic disease. We describe two patients who presented with painful papules of the scalp and weresuccessfully treated with wide local excision.

Adenoid cystic carcinoma. Actas Dermosifiliogr. 2006 Nov;97(9):578-80.
Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although itis mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists inextensive local excision with free margins. Radiotherapy is not curative andshould be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma.

Adenoid cystic carcinoma arising from the lower eyelid. Jpn J Ophthalmol. 2006 Jul-Aug;50(4):374-6.
BACKGROUND: Primary cutaneous adenoid cystic carcinoma is one of the rarest eyelid tumors. CASE: A 39-year-old man presented with a small subcutaneous induration in his left lower eyelid close to the lower lacrimal punctum.OBSERVATIONS: Under a clinical diagnosis of chalazion, the eyelid induration was extracted surgically via the palpebral conjunctiva. Histopathological examinationof the extracted tumor revealed proliferation of basaloid cells with a prominent cribriform pattern containing Alcian blue-positive and periodic acid Schiff-positive material, leading to a diagnosis of adenoid cystic carcinoma. The patient subsequently underwent extended radical excision including the lacrimal sac. No recurrence was observed during 20 months after surgery. CONCLUSIONS: Adenoid cystic carcinoma should be considered as a differential diagnosis of eyelid tumors simulating chalazion. Adenoid cystic carcinoma in the eyelid may have a more favorable prognosis than that in the lacrimal gland.