Unusual metastatic spread of a malignant eccrine poroma
Published Web Locationhttps://doi.org/10.5070/D336r0r66z
Unusual metastatic spread of a malignant eccrine poroma
Department of Plastic and Reconstructive Surgery, Royal Free Hospital, Pond Street, London NW3 2QG
Ertan Erel, Gillian Tarr, Mark S Butterworth, and Peter EM Butler
Dermatology Online Journal 8(2): 7
Malignant eccrine poroma is a rare skin tumor which commonly presents as verrucous plaques or polypoid growths. We report an 83 year old male who presented with an ulcerating eccrine poroma on the left arm. Though the tumor was excised with wide margins, axillary lymphadenopathy due to regional metastasis developed four months later, followed by lymphangitic cutaneous spread at six months. Though the prognosis for this tumor is better than previously thought, it still must certainly be considered potentially fatal.
Malignant eccrine poroma is a rare cutaneous neoplasm, which originates from the intraepidermal ductal portion of the eccrine gland. Over 200 cases have been reported in the literature, since Pinkus et al. first described it in 1963.[1,2] The incidence is less than 0.01% of all skin biopsy specimens. It commonly presents as verrucous plaques or polypoid growths, which sometimes bleed with minor trauma. Though in the past a 50% metastasis rate, usually to regional lymph nodes, was reported, it is now realized that this percentage was inflated. Distant metastases to the lungs and other internal organs are less common.[3,4] The patients usually present in the fifth to eighth decades of life and the majority of cases show slow evolution of the neoplasm over an average of 1-5 years. Fifty-five percent of malignant eccrine poromas are found on the lower limbs, 20% on the trunk, 15% on the head, and 10% on upper limbs.
The standard treatment is wide local excision and, if clinically indicated, block dissection of regional lymph nodes. The local recurrence rate is in the range of 11-25%.[2,4,6] Lymph node metastasis occurs in 9.6-19%.[2,7] These data suggest that malignant eccrine poroma, though potentially fatal, has a better prognosis than previously reported. Important histological prognostic factors include mitoses, lymphoascular invasion, and tumor depth.
|Figure 1||Figure 2|
|Figure 1: Anterior chest wall multiple papular skin eruptions|
|Figure 2: Anterior chest wall multiple papular skin eruptions (closer view)|
An 83-year-old man presented with an ulcerating tumor on the left arm. A grey ulcerated nodule measuring 45x40x15 mm was excised with 2 cm margins. The histological features were interpreted as representing a malignant eccrine poroma. The tumor cells were strongly PAS positive and stained strongly immunohistochemically with CEA and AE1/AE3. Following a four month symptom-free period, the patient developed enlarged ipsilateral axillary and epitrochlear lymph nodes. He underwent axillary lymph node dissection which confirmed metastatic malignant eccrine poroma. Six months after axillary lymph node dissection, the patient presented with two further subcutaneous nodules in the axilla, and several unusual papules around the left anterior chest wall with surrounding erythema (Figure 1,2). The subcutaneous nodules were excised and biopsies were taken from the chest wall lesions. The skin biopsies showed tumor within dermal lymphatics (a "lymphangitic" pattern of spread) with focal invasion of the epidermis (Figure 3,4).
|Figure 3||Figure 4|
|Figure 3: Photomicrograph of eccrine poroma low power (x40), haematoxylin & eosin.|
|Figure 4: Photomicrograph of eccrine poroma medium power (x250), haematoxylin & eosin.|
The management of patients with metastatic malignant eccrine poroma is difficult. It has proven resistant to many chemotherapeutic agents and radiotherapy has not generally been effective. However, there are several cases reported that have responded to different chemotherapy regimens. A complete response was obtained with melphalan, intra-arterial infusions of 5-fluorouracil (5-FU), and hyperthermia. A partial response was reported with radiotherapy combined with 5-FU. A prolonged, complete remission was achieved with doxorubicin, mitomycin C, vincristine, and 5-FU alternating with cisplatin and bleomycin. In addition, some response was seen with isotretinoin and a remission lasting several months was obtained with docatexal.[12,13] Some local benefit has resulted from peri-lesional injection of interferon-alpha and interleukin-2. Our patient was given adjuvant chemotherapy and radiotherapy. Systemic cisplatin and 5-FU failed to produce a remission. Radiotherapy and 5-FU were able only to dry out the exudative lesions.
The development of multiple cutaneous deposits with a lymphangitic pattern and microscopic epidermotrophic spread is a rare pattern of metastasis in malignant eccrine poromas. There are only seven reported cases of similar modes of spread in the literature.[13,15,16,17] The tumor cells reportedly travel along dermal lymphatics and establish themselves within the epidermis. This histological picture simulates Paget's disease. In this case, lymphatic drainage would be expected to be from the arm or chest wall to the axilla and thence to the thoracic duct, rather than from the arm to the chest wall. The mechanism of dermal lymphatic spread in this case may be by retrograde spread.
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