|Year : 2019 | Volume
| Issue : 5 | Page : 411-413
|Zosteriform lymphangitic metastases of eccrine porocarcinoma
Yunfang Zhang1, Ming Zhang2, Weiwei Wu2, Jiejie Lu2
1 Department of Oncology, Hainan Provincial Hospital of TCM, Haikou, China
2 Department of Dermatology, Hainan Provincial Hospital of Skin Disease, Haikou, China
|Date of Web Publication||5-Sep-2019|
No. 33, Southern Road of Longkun, Haikou
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Eccrine porocarcinoma (EPC) is a rare malignant neoplasm which originates from the intraepidermal portion of eccrine sweat glands or acrosyringium. Here, we report a unique case of cutaneous metastases of EPC presenting with an erosive plaque on the left thumb and multiple nodules on left forearm. The histopathological examination of the wrist lesion revealed islands of basaloid tumor cells with eosinophilic cytoplasm, downward infiltrating growths, ductal differentiation, and intracytoplasmic lumen formations and focally connected to the epidermis. Acrosyringeal differentiation was confirmed by positive immunohistochemical staining using antibodies to carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) in some of porocarcinoma cells. Lymphatic embolization was shown by immunostaining of podoplanin. To the best of our knowledge, this is the first reported EPC case with a primary lesion on the left thumb and zosteriform growth pattern of lymphangitic metastases on left forearm. Our case highlights the diagnostic challenges and reveals lymphangitic metastasis mode of EPC.
Keywords: Eccrine porocarcinoma, lymphangitic metastasis, zosteriform
|How to cite this article:|
Zhang Y, Zhang M, Wu W, Lu J. Zosteriform lymphangitic metastases of eccrine porocarcinoma. Indian J Dermatol 2019;64:411-3
| Introduction|| |
Eccrine porocarcinoma (EPC) is a rare malignant neoplasm which originates from the intraepidermal portion of eccrine sweat glands or acrosyringium. Here, we report a unique case of EPC with a primary lesion on the left thumb and zosteriform growth pattern of lymphangitic metastases on left forearm.
| Case Report|| |
In May 2018, a 53-year-old man was referred to our department with a 1-year history of erosive plaque on the left thumb and multiple nodules on left forearm. One year ago, he noticed the asymptomatic plaque on left thumb without obvious predisposing factors. The lesion was gradually enlarging, and ulcerated. The patient was treated with antibiotics and daily dressing at a local clinic. However, his symptoms did not improve. Two months ago, multiple papules and nodules developed progressively on the dorsum of left hand and forearm. His past medical history was unremarkable.
On physical examination, there was an erosive plaque on the pulp of his left thumb with nail bed involved [Figure 1]a. The patient was also found to have multiple well-demarcated papules and nodules on the dorsum of the left thumb, hand, and forearm, measuring approximately from 0.2 to 1.5 cm in diameter [Figure 1]b. There was no palpable regional lymphadenopathy. No obvious abnormality was detected in routine tests of blood, urine and stool, hepatic and renal function tests, blood sugar, lipid profile, and electrocardiogram. Histological findings presented as islands of basaloid tumor cells with eosinophilic cytoplasm, downward infiltrating growth pattern, ductal differentiation, and intracytoplasmic lumen formations, and focally connected to the epidermis [Figure 1]c. Immunohistochemistry (IHC) staining pattern was consistent with malignant appendage tumor of eccrine differentiation. IHC showed positive expression of epithelial membrane antigen (EMA) [Figure 2]a, carcinoembryonic antigen (CEA) [Figure 2]b, and podoplanin [Figure 2]c.
|Figure 1: Clinicopathological features of EPC in our case. A 53-year-old man presented erosive plaque on left thumb (a) and multiple nodules with lymphangitic metastasis of left forearm (b). The histopathological examination of the wrist lesion revealed islands of basaloid tumor cells with eosinophilic cytoplasm, downward infiltrating growths, ductal differentiation, and intracytoplasmic lumen formations and focally connected to the epidermis (c) (H and E, ×100)|
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|Figure 2: Immunohistochemical findings of the left wrist tumor. Acrosyringeal differentiation was confirmed by positive immunohistochemical staining using antibodies to CEA (a) (×100) and EMA (b) (×100) in some of porocarcinoma cells. Lymphatic embolization was shown by immunostaining of podoplanin (c) (arrow) (×100) |
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Based on clinicopathological features, we diagnosed lymphangitic metastasis of EPC. The patient refused further examination and treatment in our hospital, and then underwent thumb amputation and wide surgical excision of the tumor lesions in another hospital in June 2018. The patient died from brain and lung metastasis of EPC in October 2018.
| Discussion|| |
Until now, approximately 300 cases of EPC have been reported worldwide since this disease was first described by Pinkus and Mehregan in 1963. The pathogenesis of EPC was unclear and usually occurs in elderly patients with major involvement in the lower extremities. Only 8% of EPC occur on the upper extremity, and 3% are on the hand. In this case, the primary lesion occurred on the thumb, which was the second case reported.
The lesions of EPC had various but nonspecific morphologies, so EPC is a challenge to diagnose because it can mimic many other dermatological diseases. We report a unique case of cutaneous metastases of EPC clinically presenting with a zosteriform pattern of multiple reddish and well-demarcated papules and nodules. Zosteriform presentation of papules and plaques have been reported in several kinds of skin diseases, such as sporotrichosis, leishmaniasis, seborrheic keratosis, palisaded-encapsulated neuroma, eccrine spiradenoma, cutaneous leiomyoma, zosteriform cutaneous metastases from malignant tumor, and so on.,,,,,,,
The accurate diagnosis and differential diagnosis of EPC relies on specific histological features. Current immunohistological markers were reported to be helpful. CEA and EMA are often used as the principal indicators of ductal differentiation owing to their high sensitivity. Positive immunohistochemical staining to human cytokeratin (CK), CEA, and EMA can confirm acrosyringeal differentiation. In this report, immunohistochemical studies showed that CEA and EMA were expressed in some of the porocarcinoma cells, which confirmed the diagnosis of EPC. Podoplanin, recognized by monoclonal antibody D2-40, is a useful marker for lymphatic endothelial cells and lymphangiogenesis. Immunostaining of podoplanin clearly revealed extensive lymphatic invasion by the tumor cell. In this case, podoplanin staining demonstrated intralymphatic neoplastic cells, which proved pathologically that metastasis of EPC occurs primarily through the lymphatic system.
EPC has a high tendency of metastatic spread. Local recurrence, nodal recurrence, and distant metastases were observed in 17%–20%, 10.4%–20%, and 8.3%–11.4% of cases, respectively.,, Cutaneous metastasis can be explained by the direct lymphatic infiltration of cancer cells and posterior reinvasion of the epidermis. Zosteriform dissemination was usually regarded as the initial lymphatic spread of the tumor cells,, which also was observed in our case.
There is no established standard modality of treatment for EPC, especially in cases of metastasis. Conventional wide-local excision with broad tumor margins was currently the first-line treatment. Mohs micrographic surgery seems to be effective in eradicating early EPC and minimizing the risk of recurrence., In the case of lymphadenopathy, sentinel lymph node biopsy might be useful in determining subclinical metastasis in the regional lymph nodes. In cases of metastasis, various combinations of excision, radiotherapy, and chemotherapy, including paclitaxel, docetaxel, interferon-α, and cetuximab, have been used.,,, However, the long-term prognosis is still unfavorable. In case of recurrent or metastatic EPC, the prognosis is actually poor, and mortality rate of metastatic EPC patients can be up to 67%. Tumor thickness, tumor size, mitosis, lymphovascular invasion, lymphnode metastasis, and infiltrative growth pattern are correlated with poor prognosis.,
To the best of our knowledge, this is the first reported EPC case with a primary lesion on the left thumb and zosteriform growth pattern of lymphangitic metastases on left forearm. Our case highlights the diagnostic challenges and reveals lymphangitic metastasis mode of EPC.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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