|Year : 2012 | Volume
| Issue : 4 | Page : 633-635
Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity
Sujata Raychaudhuri1, KV Santosh1, HV Satish Babu2
1 Department of Pathology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India
2 Department of Neurosurgery, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India
|Date of Web Publication||29-Jan-2013|
RA-803 Purva Riviera, Varthur Main Road, Marathahalli, Bangalore - 560037, Karnataka
Source of Support: None, Conflict of Interest: None
Primary Cutaneous Adenoid Cystic Carcinoma (PCACC) is a rare form of cutaneous carcinoma. It closely resembles adenoid cystic carcinoma of salivary glands. The lesion tends to infiltrate and recur locally but rarely metastasizes to distant organs and lymph nodes. We present here a case of a31-year-old male with complaints of swellings in the chest wall and scalp, progressively increasing in size over a period of 10 years. Clinical diagnosis of sebaceous cysts of scalp and chest wall was made. Histopathology revealed PCACC of the chest wall and dermoid cyst of the scalp.
Keywords: Adenoid cystic carcinoma, sweat gland, cutaneous, skin, primary
|How to cite this article:|
Raychaudhuri S, Santosh K V, Satish Babu H V. Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity. J Can Res Ther 2012;8:633-5
| > Introduction|| |
Primary Cutaneous Adenoid Cystic Carcinoma (PCACC) is a rare but well documented entity, first described in 1975 by Boggio.  This is a rare form of sweat gland carcinoma, which has a histological resemblance to adenoid cystic carcinoma (ACC) of salivary gland origin. A diagnosis of a primary tumor arising in the skin can be made only after excluding metastatic deposits from other, more common sites. PCACC affects the middle aged and the elderly with a female preponderance. , The scalp is the most common site of occurrence; the tumor presents as a slow growing nodule which rarely metastasizes. [2-4]
A 31-year-old Indian male presented with separate swellings over the scalp and upper part of the chest wall, both gradually progressing in size over a period of 10 years. The swelling on the scalp preceded the one on the chest wall by a few months. Both the swellings were ill defined, nontender, nonpulsatile, and soft in consistency. Cough impulse was absent for the scalp swelling. No history of trauma or weight loss was elicited. There was a crest over the swelling of the chest wall. The scalp swelling measured 4 × 3 cm and the chest swelling measured 3 × 2 cm. General physical and systemic examinations were normal. A clinical diagnosis of dermoid cyst/sebaceous cyst was made. Excision biopsies of both the swellings were done under local anesthesia and the specimens were sent for histopathologic examination.
The scalp specimen consisted of five soft to firm gray-white tissue bits, three of which were skin covered. The largest bit measured 2 × 0.8 cm and the smallest bit measured 0.5 cm in diameter. The chest wall specimen consisted of a flap of skin covered tissue measuring 1.3 × 1 cm. No cyst was identified.
Microscopic examination of the section from the scalp depicted a cystic lesion, the wall of which was composed of loose connective tissue and lined by stratified squamous epithelium with all its layers intact. The lumen was packed with lamellated keratin. No dermal adnexal structures were seen in relation to the cyst wall.
The section from the chest wall swelling displayed a circumscribed tumor in the reticular dermis and subcutis. It was composed of basaloid cells arranged predominantly in cribriform pattern and focally showing tubular and cystic formations [Figure 1]. The tumor cells were round and ovoid with vesicular nuclei and scant cytoplasm. Mitoses were noted occasionally. Luminal secretory material was also noted [Figure 2]. Palisading of tumor cells was not seen. There was a moderately abundant stroma in between the tumor cells. No perineural invasion was evident. The overlying epidermis was thinned out. A diagnosis of epidermal cyst of the scalp swelling and cutaneous ACC of chest wall was made.
|Figure 1: Intradermal tumor composed of islands of basaloid cells predominantly arranged in cribriform pattern with areas showing cystic degeneration. (H and E, × 40)|
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|Figure 2: Cystic spaces filled with intraluminal secretions (H and E, × 100)|
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To rule out metastatic deposit from a primary tumor elsewhere, further investigations were performed. X-ray of the chest, USG of the abdomen, and CT scan of the head and neck did not reveal the presence of a primary tumor elsewhere.
| > Discussion|| |
PCACC is a rare form of salivary gland ACC. ACC is usually not only a tumor of the salivary glands and seromucinous glands of the upper airways but may also rarely appear at sites like the skin. Among the cutaneous sites, the scalp, accounting for 40% of the cases, is the most common.  Occasional cases on the chest wall and abdomen have been recorded. 
Initially it was considered to be of eccrine origin but recent evidence suggests it to have an apocrine origin.  Ceruminous glands which occur in the external ear canal are a type of apocrine glands that often gives rise to ACC.  The cutaneous tumor is usually located in the reticular dermis and subcutis, with tumor cells arranged in lobules characterized by cribriform, tubular, and cystic areas.
Most studies show that PCACC is more common in elderly females with an average age of 58.1 years.  However, a US-based study comprising a larger population group shows the incidence to be equal in both the sexes. 
Perineural invasion, although an important feature is noted only in 76% of cases.  In comparison to the analogous tumor of the salivary glands, PCACC shows less aggressive behavior.  Local recurrence rate in PCACC ranges from 44% to 51%. , The occurrence of lung and lymph nodes metastasis is exceptionally rare in PCACC but, although infrequent, can be found in ACCs arising from salivary gland and breast  Moreover, perineural invasion, may be absent in PCACC, but is a characteristic of ACC arising at other noncutaneous site.  Since PCACC has a high tendency to recur locally, the recommended treatment for PCACC is wide surgical excision with at least 2 cm tumor free margin.  A simple excision result in more frequent recurrences-the local recurrence rate of PCACC is more than 50%. 
The most important differential diagnosis in this case would be adenoid basal cell carcinoma, which also shows gland formations, adenoid cystic like characteristics, peripheral palisading, artifactual cleft, and continuity with the epidermis. It is important to distinguish it from adenoid basal cell carcinoma, which never metastasizes. In difficult cases, immunohistochemistry may be resorted to-EMA, CEA, and S100 are positive in PCACC but not in adenoid basal cell carcinoma.  Other primary skin tumors which needs to be differentiated includes mucinous carcinoma of skin, primary cutaneous cribriform apocrine carcinoma (PCCAC), dermal cylindroma, and spiradenoma.  Mucinous carcinoma shows positivity for sialomucins. PCCAC lacks perineural invasion and has cribriform architecture in the entire tumor with nuclear pleoorphism while PCACC has uniform cells. Spiradenoma has small adenoid cystic areas only focally. Dermal cylindroma has solid nests of tightly fitting tumor cells with hyaline material around them. 
The concurrent occurrence of a sebaceous cyst in a case of PCACC, has not, to the best of our knowledge been reported in literature. We assume that this is coincidental and not indicative of any association with each other.
| > References|| |
|1.||Boggio R. Adenoid cystic carcinoma of scalp. Arch Dermatol 1975;111:793-4. |
|2.||Van der Kwast TH, Vuzevski VD, Ramaekers F, Bousema MT, Van Joost T. Primary cutaneous adenoid cystic carcinoma: Case report, immunohistochemistry and review of the literature. Br J Dermatol 1988;118:567-78. |
|3.||Kato N, Yasukawa K, Onozuka T. Primary cutaneous adenoid cystic carcinoma with lymph node metastasis. Am J Dermatopathol 1998;20:571-7. |
|4.||Chu SS, Chang YL, Lou PJ. Primary cutaneous adenoid cystic carcinoma with regional lymph node metastasis. J Laryngol Otol 2001;115:673-5. |
|5.||Xu YG, Hinshaw M, Longley BJ, Ilyas H, Snow SN. Cutaneous adenoid cystic carcinoma with perineural invasion treated by mohs micrographic surgery-a case report with literature review. J Oncol 2010;2010:469049. |
|6.||Dores GM, Huycke MM, Devesa SS, Garcia CA. Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival and associated cancers 1976 to 2005. J Am Acad Dermatol 2010;63:71-8. |
|7.||Naylor E, Sarkar P, Perlis CS, Giri D, Gnepp DR, Robinson-Bostom L. Primary cutaneous adenoid cystic carcinoma. J Am Acad Dermatol 2008;58;636-41. |
|8.||Wick MR, Swanson PE. Primary adenoid cystic carcinoma of the skin. A clinical, histological, and immunocytochemical comparison with adenoid cystic carcinoma of salivary glands and adenoid basal cell carcinoma. Am J Dermatopathol 1986;8:2-13. |
|9.||McKee PH, Calonje E, Granter SR. Pathology of the skin. 3rd ed. Philadelphia: Elsevier-Mosby; 2005. p. 1653-5. |
|10.||Cacchi C, Persechino S, Fidanza L, Bartolazzi A. A primary cutaneous adenoid-cystic carcinoma in a young woman. Differential diagnosis and clinical implications. Rare Tumors 2011;3:e3. |
[Figure 1], [Figure 2]
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