Pediatric Dermatology Vol. 31 No. 6 e145–e148, 2014

Microcystic Adnexal Carcinoma in the Axilla of an 18-Year-Old Woman Margaret Green, D.O., Marsha Mitchum, M.D., Jason Marquart, M.D., Lynden P. Bowden, III, M.D., and Jonathan Bingham, M.D. Dermatology Department, Walter Reed National Military Medical Center, Bethesda, Maryland

Abstract: Microcystic adnexal carcinoma (MAC) is an uncommon adnexal neoplasm with a predilection for the head and neck. The tumor rarely metastasizes but is locally aggressive and commonly demonstrates perineural invasion. MAC occurs most often in older adults. This report describes a young woman with a MAC in her left axilla who required two stages of Mohs micrographic surgery followed by a wide local excision because of persistent perineural invasion in close proximity to the brachial plexus. Other cases presenting in the pediatric age group are discussed.

We report an unusual case of microcystic adnexal carcinoma (MAC) arising in the axilla of an 18-yearold Caucasian woman. The patient presented for the removal of a painful, enlarging lesion in the left axilla that she had first noticed 9 months earlier. On examination, an approximately 4-mm, firm nodule was noted without accompanying erythema, induration, or cervical or axillary lymphadenopathy. The patient was otherwise healthy and had no history of significant medical problems. The lesion was excised, and pathology demonstrated MAC with perineural invasion (PNI). The patient subsequently underwent two stages of Mohs micrographic surgery (MMS), which revealed persistent PNI adjacent to the brachial plexus (Figs. 1 and 2). MRI of the left brachial plexus did not reveal tumor invasion. The patient was referred to surgical oncology for wide local excision (WLE) and sentinel lymph node biopsy (SLNB).

There was no evidence of residual tumor after the WLE, and the SLNB was negative for metastasis. MRI was performed 5 months postoperatively and showed no evidence of recurrence. HISTOPATHOLOGY Margins after the second stage of MMS were clear at the perimeter of the lesion. Persistent PNI was seen in one cut within the 9 o’clock to 3 o’clock aspect of the wound (Figs. 3 and 4). DISCUSSION MAC is an uncommon cutaneous neoplasm with a deeply infiltrating, locally invasive growth pattern (1). The age of our patient and the location of her tumor underscore how little is known about the risk factors

Address correspondence to Margaret C. Green, D.O., M.S., LT MC USN, Dermatology Department, Walter Reed National Military Medical Center, 8901 Wisconsin Avenue, Bethesda, MD 20814, or e-mail: [email protected]. DOI: 10.1111/pde.12430

Published 2014. This article is a U.S. Government work and is in the public domain in the USA

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Figure 1. Clinical photograph of the axillary lesion after the initial excision, before Mohs micrographic surgery.

Figure 3. Microcystic adnexal carcinoma based in the deep dermis with characteristic horn cysts, islands of infiltrative basaloid cells, and sclerotic changes in the dermis (hematoxylin and eosin, 29).

Figure 2. Clinical photograph of the surgical defect after two levels of Mohs micrographic surgery.

for developing MAC, as the majority of MAC cases have been reported in middle-aged and older individuals. In several of these cases the patient reported the presence of the lesion for several years and even decades before it was identified as MAC (1). MAC could therefore be arising in children but be unrecognized until adulthood if patients overlook the lesion when it is slow growing and asymptomatic. Ten cases of MAC have previously been reported in children younger than 18 years of age, two of which were congenital (Table 1) (2–9). There has also been MAC reported in two siblings, suggesting a genetic basis for this tumor or a common exposure history (10). The occurrence of MAC in the axilla is uncommon, with five other cases reported (10–14). MAC most often develops on the head and neck, indicating that ultraviolet (UV) exposure is a possible risk factor (10). Other cases of MAC have developed after radiation therapy for acne, breast cancer, basal cell

Figure 4. Nerve in the deep dermis with surrounding microcystic adnexal carcinoma perineural invasion (hematoxylin and eosin, 409).

carcinoma, and squamous cell carcinoma and in cases of immunosuppression (10). One of the children with MAC had a history of cranial radiation therapy for acute T-cell lymphoblastic leukemia with central nervous system involvement at age 9 years and developed MAC on his scalp 6 years later (9). In the remainder of the pediatric cases referenced in this article, no risk factors were reported. Specific to this case, the axilla is not a classic site for UV exposure and our patient had no history of radiation or immunosuppression. Treatment methods used for other cases of MAC in the axilla have included MMS, simple excision followed by MMS, and simple excision followed by WLE (10,11,13). Because this tumor is rare, evidencebased treatment protocols have not been developed.

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TABLE 1. Pediatric Cases of Microcystic Adnexal Carcinoma (Age ≤ 18 Years) Reference

Age

Sex

Location

Time until diagnosis

Smart et al (2)

3 days

Female

Left malar cheek

Congenital

Fu et al (3)

2 wks

Female

Left temple

Congenital

Treatment

Nelson et al (4) Martin et al (5) Sharma et al (6)

6 yrs 8 yrs 10 yrs

Female Female Male

Left preauricular Left thigh Right chest

2 mos 2–3 yrs Unknown

Leibovitch et al (7)

10 yrs

Female

Arm

6 yrs

Excision at 3 mos old; en bloc resection of residual scar and subcutaneous mass at 5 mos old Excision at 10 wks old, re-excision with 1.5-mm margins Excision and re-excision WLE Excisional biopsy of presenting lesion, WLE and axillary dissection at 1 yr, excisional biopsy at 5 yrs MMS

McAlvany et al (8) Leibovitch et al (7)

11 yrs 14 yrs

Forehead Not reported

2 yrs Not reported

MMS, 1 stage MMS

Friedman et al (9) Leibovitch et al (7)

15 yrs 16 yrs

Left parietal scalp Not reported

1 yr Not reported

MMS, 2 stages MMS

Present case

18 yrs

Male Not reported Male Not reported Female

Left axilla

9 mos

MMS 2 stages, followed by WLE and sentinel lymph node biopsy

Recurrence/length of follow-up None/17 mos None/3 yrs None/1 yr None/24 mos Local recurrence and axillary metastasis/ 1 yr local recurrence/5 yrs Recurrence not reported/>5 yrs* None/32 mos Recurrence not reported/>5 yrs* None/2 yrs† Recurrence not reported/>5 yrs* None/1 yr

*Overall 5% recurrence rate reported for all of the cases in this article with at least 5 years of follow-up; recurrence rates and follow-up times were not reported for individual cases. †Patient had a history of radiation exposure for treatment of acute T-cell lymphoblastic leukemia with central nervous system involvement. MMS, Mohs micrographic surgery; WLE, wide local excision.

In several studies, MMS is reported to be the treatment of choice for MAC because of lower rates of recurrence and because adequate margins using WLE have not been standardized (1). Management strategies in addition to MMS include WLE, radiation, chemotherapy, and clinical observation (1). Several studies have compared the rate of recurrence of MAC after MMS with that after WLE. Reported recurrence rates are 0 to 22% for MMS and 5% to 60% for WLE (1,2). Chiller et al (15) reported a similar overall 10-year recurrence rate for MMS and WLE. Studies with longer follow-up are necessary to establish treatment protocols for MAC, especially in young patients. Close clinical monitoring for recurrence will be necessary in our patient, because MAC has been reported to recur up to 30 years after the initial excision (1,10). The incidence of PNI is high for MAC and is associated with persistent disease, recurrence, and greater morbidity (1). MMS is beneficial for assessing PNI, because all surgical margins are evaluated. This patient underwent two stages of MMS after initial local excision. Most studies report that an average of 2.5 MMS stages (range 1–4) are necessary to clear the tumor (1). In this case, the proximity to the brachial plexus prevented further stages from being performed, prompting referral to surgical oncology. To our knowledge, there are no reports of the outcome of

MAC treated first with MMS followed by WLE that might help guide management. There is limited evidence behind the need to sample sentinel or regional lymph nodes. Only 1% of the 223 cases that Yu et al (16) reviewed in 2010 had pathologic involvement of lymph nodes. One case of MAC in the right axilla was found to have metastases to the right axillary lymph nodes (11), and Sharma et al (6) reported the case of a 10-year-old with a MAC on the chest wall with axillary lymph node metastasis. The decision to perform SLNB was made in this case because of the proximity of the tumor to the axillary lymph nodes and in an attempt to ensure removal. Adjuvant radiation therapy was being considered for this patient, but no randomized clinical trials have been performed to assess the benefits of radiation as monotherapy or adjuvant therapy for MAC, making its use controversial (1,10). MAC has been reported to be radioresistant, and radiation therapy has been linked to MAC recurrence with a more aggressive histologic pattern (1). A recent three-patient case study by Pugh et al (17) describes the effective use of radiation therapy for MAC, but the longest follow-up to evaluate for recurrence was 30 months. In a 2010 retrospective review, Baxi et al (18) reported 14 cases of MAC treated using local excision and adjuvant radiotherapy with a crude local control rate of 93%,

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but all 14 patients received nonstandardized schedules of radiation. With so few patients being treated with radiation for a rare neoplasm and conflicting reports on efficacy, the role of radiation therapy in the treatment of MAC for all age groups is difficult to determine. Chemotherapy for the treatment of MAC has been reported, but there is little evidence to support its efficacy (10). This case highlights the dilemma concerning management of the tumor and counseling this 18-year-old patient and her family with respect to prognosis. Our patient is 1 year out from the WLE surgery and continues to do well, with no recurrence. She will continue to be followed up annually. MAC is not common in the pediatric or young-adult populations, and the lack of published guidelines for management and long-term clinical course in this demographic poses a challenge to practitioners. Despite this, the Surveillance, Epidemiology, and End Results database study gives excellent overall survival rates for MAC, making any decrease in life span in our patient unlikely (16). ACKNOWLEDGMENTS The authors would like to thank Thomas Lam, M.D., for his technical assistance in the preparation of this manuscript. REFERENCES 1. Diamantis SA, Marks VJ. Mohs micrographic surgery in the treatment of microcystic adnexal carcinoma. Dermatol Clin 2011;29:185–190. 2. Smart DR, Taintor AR, Kelly ME et al. Microcystic adnexal carcinoma: the first reported congenital case. Pediatr Dermatol 2011;28:35–38. 3. Fu T, Clark FL, Lorenz HP et al. Congenital microcystic adnexal carcinoma. Arch Dermatol 2011;147: 256–257. 4. Nelson PS, Bourgeois KM, Nicotri T Jr et al. Sclerosing sweat duct carcinoma in a 6-year-old African American child. Pediatr Dermatol 2008;25:38–42.

5. Martin PC, Smith JL, Pulitzer DR et al. Compound (primordial) adnexal carcinoma arising in a systematized compound epithelial nevus. Am J Surg Pathol 1992;16:417–425. 6. Sharma L, Biwas G, Lokeshwar N et al. Microcystic adnexal carcinoma in a ten year old child: a case report and review of the literature. Indian J Med Paediatr Oncol 2004;25:40–42. 7. Leibovitch I, Huilgol SC, Selva D et al. Microcystic adnexal carcinoma: treatment with Mohs micrographic surgery. J Am Acad Dermatol 2005;52:295–300. 8. McAlvany JP, Stonecipher MR, Leshin B et al. Sclerosing sweat duct carcinoma in an 11-year-old boy. J Dermatol Surg Oncol 1994;20:767–768. 9. Friedman PM, Friedman RH, Jiang SB et al. Microcystic adnexal carcinoma: collaborative series review and update. J Am Acad Dermatol 1999;41:225–231. 10. Abbate M, Zeitouni NC, Seyler M et al. Clinical course, risk factors, and treatment of microcystic adnexal carcinoma: a short series report. Dermatol Surg 2003; 29:1035–1038. 11. Ban M, Sugie S, Kamiya H et al. Microcystic adnexal carcinoma with lymph node metastasis. Dermatology 2003;207:395–397. 12. Ceballos PI, Penneys NS, Cohen BH. Microcystic adnexal carcinoma: a case showing eccrine duct differentiation. J Dermatol Surg Oncol 1988;14:1236–1239. 13. Nadiminti H, Nadiminti U, Washington C. Microcystic adnexal carcinoma in African-Americans. Dermatol Surg 2007;33:1384–1387. 14. Ohtsuka H, Nagamatsu S. Microcystic adnexal carcinoma: review of 51 Japanese patients. Dermatology 2002;204:190–193. 15. Chiller K, Passaro D, Scheuller M, et al. Microcystic adnexal carcinoma: forty-eight cases, their treatment, and their outcome. Arch Dermatol 2000;136:1355– 1359. 16. Yu JB, Blitzblau RC, Patel SC, et al. Surveillance, Epidemiology, and End Results (SEER) database analysis of microcystic adnexal carcinoma (sclerosing sweat duct carcinoma) of the skin. Am J Clin Oncol 2010;33:125–127. 17. Pugh TJ, Lee NY, Pacheco T et al. Microcystic adnexal carcinoma of the face treated with radiation therapy: a case report and review of the literature. Head Neck 2012;34:1045–1050. 18. Baxi S, Deb S, Weedon D et al. Microcystic adnexal carcinoma of the skin: the role of adjuvant radiotherapy. J Med Imaging Radiat Oncol 2010;54:477–482.

Microcystic adnexal carcinoma in the axilla of an 18-year-old woman.

Microcystic adnexal carcinoma (MAC) is an uncommon adnexal neoplasm with a predilection for the head and neck. The tumor rarely metastasizes but is lo...
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