Photodynamic Therapy as Palliative Therapy for Invasive Syringoid Eccrine Carcinoma of the Auricle: A Case Report

doi:10.4236/jct.2013.46125

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Journal of Cancer Therapy, 2013, 4, 1091-1094

http://dx.doi.org/10.4236/jct.2013.46125 Published Online August 2013 (http://www.scirp.org/journal/jct) 1091

Photodynamic Therapy as Palliative Therapy for Invasive

Syringoid Eccrine Carcinoma of the Auricle: A Case Report

Claudia Gutiérrez Gómez1*, AnailAlvarez2, Ana Lilia Ruelas2, Ixchel Landgrave2,

Alfonso Vallarta3, Laura Andrade3

1Department of Plastic and Reconstructive Surgery, Medica Sur Hospital, México City, México; 2Dermatology Department, Medica

Sur Hospital, México City, México; 3Plastic Surgery Department, Medica Sur Hospital, México City, México.

Email: *[email protected]

Received May 9th, 2013; revised June 12th, 2013; accepted June 20th, 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Recurrent eccrine syringomatous carcinoma is an extremely rare adnexal tumor predominantly found in the head and

neck region. We present a case of a 75-year-old woman with a 7-year history of recurrent syringoid eccrine carcinoma

in the scalp and auricle. She had undergone five previous oncologic resections and declined further oncologic ablation

of the auricle, including temporal bone and external ear canal resection. We performed five sessions of palliative photo-

dynamic therapy using topical aminolevulinic acid HCL 20% (ALA) and between 25 and 33 pulses of Intense Pulsed

Light (Quantum) with an intensity of 30 Joules. The ALA incubation time was 3 hours, while the time between each

session was 4 weeks. This treatment helped to control ulceration, pain, and swellin g. We saw the patient 1 month after

the last treatment, by that time she moved and we lost contact.

Keywords: Syringoid Eccrine Carcinoma; Photodynamic Therapy

1. Introduction

Syringoid Eccrine carcinoma (SEC) is a very rare tumor.

It is very locally invasive, destructive, and is often recur-

rent. It was originally described as a basal cell tumor

with eccrine differentiation in 1969 [1].

SEC differs from basal cell carcinoma in its cytology

and enzymatic patterns. Histologically, SEC resembles

syringoma in its ductal, cystic, and comma-like ep ithelial

components. It differs from syringoma in its cellularity,

anaplasia, and deep invasiveness [2].

According to the Cru z Modified Classification System,

SEC is also known as a basal cell tumor with eccrine dif-

ferentiation, adenocarcinoma of the eccrine sweat gland,

syringealhidradenoma, atypical syringoma, sweat gland

carcinoma with syringomatous features, eccrinebasalio-

ma and eccrine syringomatous carcinoma. With a predi-

lection for the scalp, it clinically resembles basal cell

carcinoma and tends to histologically invade beyond its

gross margins [3].

The tumor presents itself in patients ranging from 1 to

86 years of age, with most cases occurring in the fifth to

seventh decades of life. Overall, these tumors occur

equally in both sexes, althou gh certain subtypes appear to

have a predilection for males or females. Most sweat

gland carcinomas grow slowly, with patients frequently

having a delayed presentation of 5 years or more. Lym-

phatic metastasis typically occurs at a rate of 20% to

24%. Regional cutaneous metastasis and hematogenous

spread have also been noted. Local recurrence occurs in

14% to 20% of cases [4]. Most patients survive beyond

10 years and usually die of another cause, although nota-

ble exceptions of death within 6 months of diagnosis due

to metastasis to the lymph nodes and lungs have been

reported.

Wide local excision and evaluation of lymph node bi-

opsy or regional lymphadenectomy samples are recom-

mended. Radiotherapy is reserved for recurrence or me-

tastatic lymph node involvement [5]. Chemotherapy is

not commonly used, although administration of trastu-

zumab has been reported in a metastatic tumor with Her-

2/neu gene amplifi ca t ion [6].

2. Case Report

A 75-year-old female patient developed a small (<1 cm)

tumor in the right temporoparietal scalp that had been

*Corresponding a uthor.

Photodynamic Therapy as Palliative Therapy for Invasive Syringoideccrine Carcinoma of the Auricle: A Case Report

1092

excised 7 years earlier and treated as a benign tumor

without histologic evaluation. Several months after the

first treatment, another tumor grew within the scar; it was

reported to be a basal cell carcinoma and was resected

with tumor-free edges. Two years later, it recurred, and

the patient underwent wide resection and coverage with a

skin graft. This time, it was diagnosed as an adenoid

sclerosant ulcerated and multicentric basal cell carci-

noma. One year later, it recurred, and the immunohis-

tological report showed recurrent SEC. A year after that,

it recurred again, and she underwent wide resection in-

cluding the upper third of the right auricle with the same

diagnosis of SEC. The tumor reappeared one year later,

and the oncologic surgeon proposed wide resection with

total amputation of the auricle and resection of part of the

temporal bone followed by coverage with a free flap. The

patient declined this ablation and requested palliative

treatment without surgery or radiotherapy. After pallia-

tive treatment, she reported no systemic signs of illness,

weight loss, or functional impairment. Her examination

results were normal.

The patient rejected surgical treatment, including

Mohs micrographic surgery (Figure 1). She underwent

five sessions of palliative photodynamic therapy for re-

current SEC of the auricle using topical aminolevulinic

acid HCL 20% (ALA) (Levulan Kerastick; Stiefel Coral

Gables Fl USA) and between 25 and 33 pulses using

Level Intense Pulsed Light (Quantum Lumenis SR 560).

The ALA incubation time was 3 hours, and 4 weeks

passed between each session. This treatment helped to

control ulceration, pain, and swelling. We saw the patient

1 month after the last treatment (Figure 2), after which

time she moved and we lost contact.

Figure 1. A 75-year-old female with a 7-year-history of re-

current ulcerated syringoid eccrine carcinoma. Pretreat-

ment view.

Figure 2. After five sessions of palliative photodynamic

therapy with satisfactory control of local ulceration and

inflammatory reaction. One month after the last session.

3. Discussion

After many recurrences and extensive surgeries including

partial auricle amputation, the patient declined further

surgical treatment. Without therapy, the course of SEC is

very slow and of long duration, but multiple recurrences

are described [1,7].

Local recurrence occurs in 14% to 20% of cases. Most

patients survive beyond 10 years and usually die of an-

other cause, although notable exceptions of death within

6 months of diagnosis due to metastasis to the lymph

nodes and lungs have been reported [4,8].

Therapy of SEC consist mainly of surgical excision [9-

17]. Nowadays, Mohsmicrographic surgery is the method

of choice for SEC [11,16,18,19] if there is no “skip” area

or evidence of multifocality and no evidence of distant

metastases. Nishiwara reports the use of chemotherapy as

initial treatment in a case that exhibited multiple distant

metastases at diagnosis. In general, chemotherapy and/or

radiation therapy have been used for metastatic sweat

gland carcinomas. Radiation treatment for sweat gland

carcinoma has been reported to be ineffective, but more

recent reports have found a role for radiation in the local

control of this disease [20-24].The role of chemothera-

phy in sweat gland carcinoma is also under debate. Both

single-agent and combination chemotherapy have been

used infrequently and, at best, have shown only a tempo-

rary benefit [22-26]. There have been a few reports in

which patients with SEC were treated with radiotherapy

for local control and bone metastases [11,27]. Nishizawa

et al. reported an extended SEC treated with seven cycles

of combination chemotherapy [28].

Photodynamic therapy for superficial skin tumors, in-

cluding basal cell carcinoma, following local application

of ALA has been described by several authors [29-31].

Photodynamic Therapy as Palliative Therapy for Invasive Syringoideccrine Carcinoma of the Auricle: A Case Report 1093

However, it has not been described for the treatment of

SEC. We believe that this case shows the utility of

photodynamic therapy as a palliative treatment for tem-

poral control of tumor activity, even if it may not work in

other clinical contest except the ones previously reported.

This alternate approach was used because of patients’

refusal to radical surgery, and there is not any previous

report of palliative treatment in this rare neoplasm.

4. Disclosure

The authors have no commercial associations or financial

disclosures with regard to this manuscript.

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