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A Case of Acinic Cell Carcinoma Involving Skin

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Letter to the Editor

Vol. 28, No. 2, 2016 255

Received October 27, 2014, Revised March 23, 2015, Accepted for publication April 29, 2015

Corresponding author: Mi Woo Lee, Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. Tel: 82-2-3010-3460, Fax:

82-2-486-7831, E-mail: miumiu@amc.seoul.kr

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.

org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Copyright © The Korean Dermatological Association and The Korean

Society for Investigative Dermatology Fig. 1. A single 2-cm sized flesh-toned subcutaneous round nodule on the right cheek.

http://dx.doi.org/10.5021/ad.2016.28.2.255

A Case of Acinic Cell Carcinoma Involving Skin

Kwang Hee Won, Mi Hye Lee, Woo Jin Lee, Mi Woo Lee

Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Dear Editor:

A 62-year-old man presented with a 5-month history of soli- tary subcutaneous nodule on the right cheek that. On phys- ical examination, there was a 2-cm flesh-toned subcuta- neous round nodule on the right cheek (Fig. 1). The mass was soft and somewhat fluctuant on palpation without tenderness. Histopathologic examination revealed multiple well-circumscribed tumor nests with solid, lobular, and mi- crocystic structures (Fig. 2A). A large portion of tumor cells displayed serous acinar differentiation with cytoplasmic granules, termed cytoplasmic zymogen-like granules (Fig.

2B). The cytoplasmic granules were periodic acid-Schiff (PAS)-positive and diastase-resistant (Fig. 2C). Moderate nu- clear pleomorphism and hyperchromasia were present.

Immunohistochemical staining revealed that the tumor cells were immunopositive for cytokeratin, α-1 antitrypsin, and α-1 antichymotrypsin (Fig. 2D∼F), while they were immunonegative for S100 protein. We diagnosed the pa- tient with secondary cutaneous acinic cell carcinoma (ACC) that had locally advanced from the right parotid gland.

ACC is a malignant salivary gland neoplasm that accounts for 20% of all salivary gland malignancies, and 90% of cases arise in the parotid gland. The onset incidence peaks in the fifth and sixth decades although age at onset varies from 5 years to over 80 years of age1. ACC presents as a painless subcutaneous nodule. In rare cases, pain and ten- derness can occur intermittently. Facial paralysis, which occurs in 11% of cases, is usually a serious prognostic

sign2. Cutaneous ACC metastasis is rare, with only a few reported cases3.

Microscopically, primary tumors can present with four characteristic growth patterns: solid/lobular, microcystic, papillary cystic, and follicular, with several patterns often seen in the same tumor. The diagnostic feature of ACC is the presence of at least some neoplastic cells demonstrat- ing serous acinar differentiation, which is characterized by cytoplasmic zymogen secretory granules4. These tumor cells are polygonal with lightly basophilic to amphophilic fine granules in the cytoplasm. The granules are character- istically PAS-positive and diastase-resistant. In addition to serous cells, there may also be clear cells, intercalated duct cells, vacuolated cells, and non-specific glandular cells4. Tumor size, degree of differentiation, and in- filtrative growth patterns are useful in predicting clinical courses5. Immunohistochemistry showed that tumor cells can be positive for cytokeratin, α-1 antichymotrypsin, α 1-antitrypsin, and lysozyme, with approximately 10% of cells positive for S100 protein. However, there is no spe- cific immunoprofile, and immunohistochemistry has little impact on diagnosis4.

ACC diagnosis is usually confirmed with fine-needle aspi- ration biopsy, and surgical excision is the treatment of

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Letter to the Editor

256 Ann Dermatol

Fig. 2. (A) Tumor nests with variable growth patterns, including solid, lobular, and microcystic patterns (H&E, ×15). (B) Tumor cells with serous acinar differentiation and cytoplasmic granules, termed cytoplasmic zymogen-like granules (H&E, ×400). (C) The cytoplasmic granules are periodic acid-Schiff (PAS)-positive and diastase-resistant (D-PAS, ×400). (D∼F) Tumor cells were immunopositive for cytokeratin, α-1 antitrypsin, and α-1 antichymotrypsin (immunoperoxidase stain, ×200).

choice. Radiation therapy is generally reserved for more aggressive or recurrent tumors, while neck dissection is performed in patients with clinically positive nodes. In one study, the 5-year survival rates were 83.3% (observed) and 91.4% (disease-specific), and poor survival was asso- ciated with high grade, patient age >30 years, and meta- static disease1.

We have here reported a rare case of secondary cutaneous ACC presenting as solitary subcutaneous nodule on the right cheek in a 62-year-old man. When examining der- mal or subcutaneous tumors of the face, tumors originat- ing from the salivary gland should be considered. 

REFERENCES

1. Hoffman HT, Karnell LH, Robinson RA, Pinkston JA, Menck

HR. National Cancer Data Base report on cancer of the head and neck: acinic cell carcinoma. Head Neck 1999;21:297- 309.

2. Laskawi R, Rödel R, Zirk A, Arglebe C. Retrospective analysis of 35 patients with acinic cell carcinoma of the parotid gland. J Oral Maxillofac Surg 1998;56:440-443.

3. Varsegi MF, Ravis SM, Hattab EM, Henley JD, Billings SD.

Widespread cutaneous metastases from acinic cell car- cinoma 20 years after primary presentation. J Cutan Pathol 2008;35:591-593.

4. Thompson L. World Health Organization classification of tumours: pathology and genetics of head and neck tumours.

Ear Nose Throat J 2006;85:74.

5. Ellis GL, Corio RL. Acinic cell adenocarcinoma. A clini- copathologic analysis of 294 cases. Cancer 1983;52:542- 549.

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