Eccrine porocarcinoma: report of four cases and literature review

Abstracts

Porocarcinoma écrino é um tumor de pele raro derivado do acrossiríngeo. Quatro casos de porocarcinoma écrino são estudados. Os pacientes tinham idade entre 49 e 64 anos, sendo dois homens e duas mulheres. O tempo de crescimento dos tumores variou de um a 20 anos. As lesões mediam de 1,2 x 2 a 4 x 5 cm e se localizavam nas extremidades ou na face. Dois pacientes apresentavam carcinoma basocelular em sítios diferentes do porocarcinoma écrino. Duas das biópsias foram previamente descritas como poroma écrino; entretanto, malignidade foi observada em todos os casos após excisão ampla. Portanto, em toda biópsia de pele compatível com poroma écrino, deve-se considerar a possibilidade de malignidade.

Acrospiroma écrino; Adenocarcinoma; Carcinoma; Glândulas

CASE REPORT

Eccrine porocarcinoma: report of four cases and literature review

Fabiane Andrade Mulinari-Brenner^I; Maira Mitsue Mukai^II; Carlos Augusto Silva Bastos^III; Ezio Augusto Amaral Filho^IV; Jesus Rodriguez Santamaria^V; José Fillus Neto^VI

^IDermatologist, Assistant professor of Dermatology, Universidade Federal do Paraná, Head of the Dermatology Unit, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^IIDermatologist, Temporary lecturer of Dermatology, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^IIIDermatologist, preceptor of the Outpatient's Clinic, Hospital de Clínicas, Universidade Federal do Paraná and of the Centro de Dermatologia e Doenças Infecciosas Souza Araújo – Curitiba (PR), Brazil

^IVOncological surgeon, Centro de Dermatologia e Doenças Infecciosas Souza Araújo, master's degree in surgery from the Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^VDermatologist, Assistant professor of Dermatology, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^VIAdjunct Professor of Departamento de Patologia Médica do Hospital das Clínicas de Curitiba – Curitiba (PR), Brasil

^{Mailing Address}

ABSTRACT

Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.

Keywords: Acrospiroma, eccrine; Adenocarcinoma; Carcinoma; Eccrine glands

INTRODUCTION

Sweat gland carcinomas are rare and account for 0.005% of all skin neoplasias.¹ The eccrine porocarcinoma (EPC) or malignant eccrine poroma is an extremely rare skin tumor, although it is a relatively common among sweat gland carcinomas. They arise from the intraepithelial portion of the eccrine sweat gland, the acrosyringium, being a primary tumor or, even more common, a malignant transformation of an eccrine poroma (EP).² This tumor was first described in 1963 by Pinkus and Mehregan.³

CASE REPORT

We carried out a detailed review of the clinical and histopathology findings by light microscopy of four cases diagnosed with EPC between January 1995 and February 2000, at the Department of Dermatology, Universidade Federal do Paraná.

In each case we assessed clinical information including sex, age, phototype and occupation of each patient. We studied the tumor characteristics, such as symptoms, clinical course, size, site and histopathology.

The mean age at onset of skin tumors was 59.2 years (49-69 years). There were no differences as to sex in this small group, composed of two men and two women. All tumors had been growing for a long period - from one to 20 years (mean of 7.2 years) considering time the patient made the clinical identification.

The lesions had many clinical presentations: papules, verrucous, ulcerated nodular or vegetative ulcerated lesions, and sizes varied between 1.2 cm x 2 cm to 4 cm x 5 cm (Figures 1 and 2). Three of them were located on the limbs and one on the face (Figure 3). The patients had mild complaints, such as occasional difficulties to move the limbs, clear liquid drainage and bleeding upon trauma. None of them had clinically involved regional lymph nodes.

Two patients had associated basal cell carcinoma (BCC) - both on the nose. In one of them, the BCC was diagnosed upon EPC diagnosis (case 4) and the other had suffered a previous tumor resection with confirmed pathology of BCC. The last patient also had leprosy and was treated with polychemotherapy for two years (case 2).

All patients were submitted to biopsy - two of them with EP findings – with no evidence of atypical cells. After total tumor resection, histopathology showed malignancy in all cases (Figures 4 and 5).

One of the lesions was partially resected in the first attempt (case 2), and it recurred locally after two months, when it was completely excised by another surgical intervention. There was no evidence of metastases. All patients remained disease-free during a mean follow up of 7.5 months (6 to 9 months). Results are shown on Chart 1.

DISCUSSION

The EPC is more common in elderly patients and most cases occur in the fifth decade of life. Men and women are equally affected, and prevalence varies in both sex in diverse studies. ^3-5 Approximately 50% of EPC appear first in the lower limbs; in that, more than 40% occurr below the knees 2 - contrary to what happens to other adnexal tumors that tend to concentrate on the head and neck. Less than 5% involve the scalp.⁶ Other affected sites are face, ear, eyelids, abdomen, vulva, penis and pubis. In the cases described in this report, the mean age at tumor detection was 51 years. Time to make diagnosis varied between one and 20 years and the mean age of the patients was 51 years. The lesions involved the limbs (hands, feet and arms) and the face. Both sexes were affected; however, women (cases 1 and 3) had smaller and more long lasting lesions when compared to men.

The lesions usually measured less than 2 cm. They were clinically solitary circumscribed nodules or plaques on the face and limbs, pinkish or erythematous, covered by normal skin, which could be ulcerated or with hematic crusts. There are descriptions of lesions with a zosteriform pattern, and multiple with cobblestone distribution. Many are asymptomatic; however they can be associated with bleeding, pain or pruritus.

The cases reported showed the variety of EPC clinical presentations, ranging from a 1.5 cm to a more extensive lesion, with ulcerated areas and vegetating borders, making it very difficult to diagnose. Mild local symptoms were reported by all four patients.

There are a few descriptions of nevus sebaceous of Jadassohn and hydroacanthoma simplex exhibiting malignant transformation into EPC; nonetheless, their role as possible pre-invasive precursors of this lesion is still unknown.⁷ It seems that the upper portion of the dermal eccrine duct could have a role in the oncogenesis of the hydroacanthoma simplex. More recently it has been suggested that a mutation in the tumor-suppressive gene P53, could be involved in ECP carcinogenesis. ⁸

Many patients noticed the primary lesion years before diagnosis. This reinforces the fact that many EPCs may stem from an EP. ⁹ There is also a report describing the relation between diabetes and EPC, and one already established with clear cell syringoma. This suggests the use of clear cell EPC as a skin marker for diabetes mellitus. ¹⁰ Cases described on the nail plate and bed suggest trauma as the oncogenic factor. This series had two cases with BCC, which could suggest an association between EPC and sunlight exposure. Moreover, all patients were phototype II or III, like many patients from the literature review.

Differential diagnoses include basal and squamous cell carcinomas, seborrheic keratosis, amelanotic melanoma, verruca vulgaris, skin metastasis of neoplasms and other tumors, such as Bowen disease, Paget disease and hydroacanthoma simplex.¹¹ In general, this disease must be considered in patients with long standing tumors.

The definitive diagnosis is made by histopathology, since clinical findings vary. Neoplastic cells have a polygonal shape with clear cytoplasm, multiple nuclei or a large, hyperchromic and irregular nucleus; ductal structures, Paget-like growth of atypical cells on the epidermis and mitotic figures are usually observed. ² The epidermis can show acanthosis as a result of numerous well-defined nests of intertwined tumor cells.

The histological diagnosis may be difficult to make. The abundant presence of glycogen granules in the cytoplasm of tumor cells easily seen by the periodic acid Shiff (PAS), lack of keratinization and clusters of ducts, represent peculiar characteristics of EPCs, which help in the differential diagnosis(12). The EPC cells dye the carcinoembryonic antigen (CEA), usually found in the cytoplasm of tumor cells and in the lumen of malignant glands. Such findings help differentiate adnexal tumors from epidermal carcinomas, which are CEA-negative.

The EPC can develop from the intradermal or dermal eccrine poroma (EP). In such cases one may find areas composed of EP cells of benign appearance among anaplastic cells. 11 Therefore, it is possible for a small sample obtained by biopsy to show characteristics which are similar to those of EPs, which happened in two of the four cases presented. The cases 3 and 4 did not show malignancy in biopsy. Both had clear limits between the epidermis and the tumor with PAS-positive cuboidal cells. No cell atypia was seen in the biopsy fragments. The four cases showed PAS-positive atypical cells and this dye was decisive for diagnosis. No tumor was found among the lymphatic vessels.

Since 1963, in the first observation by Pinkus and Mehregan, it has been established that the initial EPC stage can originate from epidermal islets, and this stage may be followed by dermal involvement.^11,12 Some EPC cells invade lymphatic canals, which then become enlarged. Regional metastases are present in approximately 20% of cases 2. Multiple skin metastases, papules or nodules are peculiar characteristics. Visceral metastases are rarely present upon diagnosis and occur in approximately 12% of cases.^2,6,12,13 The prognosis of this carcinoma seems difficult to establish due to missed follow-up of cases described in the literature and tumor rarity. In about 59% of cases patients present cutaneous and visceral metastases, or regional lymph node involvement upon diagnosis.¹⁴ Approximately 20% of cases recur after excision and mortality rates can reach as far as 67% in five years if regional lymph nodes are involved.

Treatment of choice for EPC is total surgical excision with broad tumor margins. There is still insufficient literature data on cryosurgery and electrosurgery in order to accurately assess indication and tumor recurrence ⁷. Radiation therapy and chemotherapy seem to be ineffective to control tumor recurrence or metastasis ⁶. Chemotherapy with methotrexate, cisplatin, adriamycin and bleomycin or isotretinoin and interferon alpha have been used with partial response. There is no effective definitive treatment available for EPC at the moment. ¹⁵

All cases were surgically treated by broad excision with 0.3 cm to 0.5 cm margins, which seem to be a good treatment option. There was one recurrence – the biopsy showed involvement of the deep margins. This was a local recurrence after eight months, without involving regional lymph nodes.

EPC must be considered in the differential diagnosis of patients older than fifty years with long standing tumors in the limbs and head. Biopsy is indicated and, even with a diagnosis of EP, one must be attentive to risk of malignancy.

REFERENCES

Conflict of interest: None

Financial funding: None

How to cite this article: Mulinari-Brenner FA, Mukai MM, Bastos CAS, Amaral Filho EA, Santamaria JR, Fillus Neto J. Porocarcinoma écrino: relato de quatro casos e revisão da literatura. An Bras Dermatol. 2009;84(5):519-23.

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Mailing Address:

Fabiane Andrade Mulinari-Brenner

Rua General Carneiro, 181

SAM-4

80060 900 Curitiba PR

Tel.:/fax: [phone]

Publication Dates

• Publication in this collection
  07 Jan 2010
• Date of issue
  Oct 2009

History

• Accepted
  26 June 2009
• Received
  04 Oct 2004