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Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 241
Malignant acrospiroma of chest and abdominal wall treated with chemotherapy

1 Department of Radiotherapy, Calcutta National Medical College and Hospital, Kolkata, India
2 Department of Pathology, IPGMER, Kolkata, India
3 Department of Dermatology, Calcutta National Medical College and Hospital, Kolkata, India

Date of Web Publication20-Apr-2013

Correspondence Address:
Anis Bandyopadhyay
Flat No. 102, P 756A, Padmapukur, Parnashree, Behala, Kolkata - 700 060, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.110849

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Acrospiroma denotes a group of benign ductal tumours of the eccrine sweat glands that may or may not be connected to the skin. Although various eccrine sweat gland tumours including benign acrospiroma are widely reviewed, malignant acrospiroma is rarely reported. Malignant acrospiroma have the propensity to recur locally and metastasizes to regional lymph nodes. The primary treatment is wide local excision with or without lymph node dissection. Local radiation is added in the presence of high risk features to reduce the risk of recurrence. We describe a case of a malignant acrospiroma involving wide areas of chest and abdominal wall with metastases to bilateral axillary lymph nodes in a 47 year old man showing minimal clinical response to combination chemotherapy and paclitaxel.

Keywords: Axillary lymphadenopathy, chemotherapy, eccrine, malignant acrospiroma, sweat gland carcinoma

How to cite this article:
Bandyopadhyay A, Das M, Jana S, Das A. Malignant acrospiroma of chest and abdominal wall treated with chemotherapy. Indian J Dermatol 2013;58:241

How to cite this URL:
Bandyopadhyay A, Das M, Jana S, Das A. Malignant acrospiroma of chest and abdominal wall treated with chemotherapy. Indian J Dermatol [serial online] 2013 [cited 2020 Sep 28];58:241. Available from: http://www.e-ijd.org/text.asp?2013/58/3/241/110849

What was known? Malignant acrospiroma is which rarely reported, surgical resection is treatment of choice, with radiotherapy for unresectable and margin positive cases

   Introduction Top

Acrospiroma denotes a group of benign ductal tumors of the eccrine sweat glands that may or may not be connected to the skin, ranging from solitary plaques to exophytic papules to dermal nodules. [1] Malignant acrospiroma comprises a group of rare epidermal, juxtaepidermal, and dermal ductal carcinomas that may coexist with their benign counterpart and have the potential for regional lymph node and, very rarely, distant metastases. The primary treatment is wide local excision with or without lymph node dissection. [2] We describe a case of a malignant acrospiroma involving wide areas of the chest and abdominal wall with metastases to bilateral axillary lymph nodes in a 47-year-old man, showing no clinical response to combination chemotherapy. Although various eccrine sweat gland tumors including benign acrospiroma are widely reviewed, malignant acrospiroma is rarely reported; also, literature on their response to chemotherapy is wanting.

   Case Report Top

A 47-year-old man presented at our dermatology outpatient department with multiple painful reddish raised nodular lesions over the chest and upper abdomen for the last four months. A year and a half back, he first noted a small single nodular lesion in the middle of the chest, gradually increasing in size with a rough surface overlying it which later became painful, but was not associated with any discharge. The lesion was subsequently excised in a medical college in September 2008. The site took a long time to heal. Within 3-4 months post surgery, he noticed multiple groups of raised lesions of skin close to the suture site and draining site in the abdomen. These lesions gradually increased in size within another 3-4 months, with no associated discharge or pain. Now for the past four months, he had noticed multiple crops of lesions on the chest and abdomen suddenly increasing in size and number. They were associated with pruritus, bleeds on scratch, and pain, associated with discharge. He complained of painful swelling in bilateral axilla and thickening of skin underneath and surrounding the existing lesion.

Physical examination revealed multiple grouped nodular erythematous to skin-colored translucent papules and nodules, a few of them hyperkeratotic, brownish to black in color of varying sizes ranging from less than 1 cm to about 10 × 8 cm distributed over the anterior chest wall and upper abdomen [Figure 1]. There were also ulceroproliferative lesions with pus discharge rising from the excision scar overlying the epigastric region. The lesions were painful and there was purulent yellowish discharge from the lesions. The skin around the nodular masses was infiltrated and tender. Examination of the axilla revealed bilateral hard, tender, and mobile lymphadenopathies (single, about 8 × 6 cm in the right axilla, and single, about 3 × 4 cm in the left axilla). The initial cytopathological report before the first excision was consistent with benign adnexal tumor and histopathology of the excised lesion revealed it to be eccrine poroma. Based on the clinical and pathological features of a repeat biopsy, done after two years, a provisional diagnosis of cutaneous malignancy/malignant adnexal tumor was made. The repeat histopathology revealed a tumor arising within the lower portion of the epidermis with extension downward into the dermis. The tumor was present in lobules and broad anastomosing bands. The border between the epidermis and the tumor was readily apparent.
Figure 1: Multiple noduloulcerative lesions, brownish to black in color over the chest wall and abdomen

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The tumor was predominantly solid [Figure 2]; however, narrow ductal lumina could be seen focally. The cells were oval to cuboidal with a deeply basophilic nucleus and a clear-to-eosinophilic cytoplasm. Focally large hyperchromatic nuclei were seen. The mitotic rate was very high (10 per 10 high power field) with atypical mitosis [Figure 3]. No cytoplasmic keratinization, intracytoplasmic pigment, or peripheral palisading of nuclei was noted. Necrosis was also not visible. The tumor displayed an infiltrative growth [Figure 4] and reached up to the subcutaneous plane. Focal surface ulceration was noted. The margins of resection were free. Histochemically, the cells were PAS-positive (PAS; periodic acid-Schiff) indicating the presence of glycogen. The cells were positive for pancytokeratin and negative for HMB-45 (HMB: Human Melanoma Black) and S-100. A final diagnosis of malignant eccrine acrospiroma or acrospirocarcinoma was made on histopathology, based on these findings.
Figure 2: Low-power view showing islands of cells extending from the epidermis into the dermis. The surface shows hyperkeratosis (Magnification LP 5×, H and E staining )

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Figure 3: Atypical mitosis is seen (arrow), HP 40×, H and E staining

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Figure 4: The cells are seen streaming down from the epidermis with a clear zone of differentiation between the normal epidermis and the tumor cells

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A computed tomography (CT) evaluation of the abdomen and thorax revealed multiple heterogeneous, hyperdense nodular lesions of varying sizes with irregular margins rising from the skin of the anterior abdominal wall and anterior chest wall [Figure 5]. Some were continuous and others rose as discrete exophytic nodules. The lesions could be grouped into three types, those rising from the skin and growing exophytically with little or no deeper component, some that were primarily rising deep to the epidermis in the subcutaneous fatty tissue as discrete nodules, and other bigger lesions extending from the surface of the skin across subcutaneous fatty tissue and invading into the underlying muscles of the anterior abdominal wall and thorax. The largest nodule measured around 6.2 × 6.0 cm rising from the epidermis. There was no extension into the abdominal cavity. There was also the presence of bilateral multiple enlarged lymph nodes, of around 5 × 6 cm on the right and around 1.3 × 2.5 cm on the left [Figure 6]. A radiological diagnosis of dermal appendage tumor (?malignant) with infiltration into the subcutaneous tissue and underlying muscle over the upper anterior abdominal wall and upper anterior chest wall with bilateral axillary nodal involvement was made.
Figure 5: CT scan showing all three subtypes of lesions in the same section, the ones rising from skin and growing exophytically and invading into deeper tissues (a), those rising mainly from the deep subcutaneous tissue as discrete nodules (b), and superficial lesions that have only exophytic component (c)

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Figure 6: CT scan showing enlarged bilateral axillary lymphadenopathy, more marked on the right side

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The patient was treated with systemic chemotherapy, as wide local excision was not deemed feasible by the surgeon citing widespread and disseminated nature of the lesions. He was initially treated with a combination chemotherapy regimen consisting of adriamycin 50 mg/m2 Day 1, vincristine 1 mg/m2 Day 1 and Day 5, 5-fluorouracil (5FU) 500 mg D1-D5, cisplatin 20 mg/m2 and bleomycin 5 IU/m2 from Day15-Day19, a 28-day cycle for two cycles with no significant clinical response. Thereafter, the chemotherapy regimen was changed to paclitaxel 175 mg/m2 D1, a 21-day cycle which showed minimal clinical response with the appearance of new lesions. However, there was significant amelioration of pain. The patient subsequently received two more cycles of paclitaxel, with sustained pain relief but minimal or no regression of the lesions. He was planned for locoregional radiation using electron beam but defaulted and was lost to follow-up thereafter.

   Discussion Top

Acrospiromas are distinct sweat gland tumors that present as solitary plaques, nodules, or exophytic papules. The term eccrine acrospiroma was first coined by Johnson and Hewig, in 1968, because on the histologic and histochemical studies, the cells of these tumors were believed to mimic those of the eccrine sweat gland. [3] These tumors usually occur in adults. Histologically, these lesions are subclassified according to the location of the tumor in relation to the epidermis, with those confined primarily in the epidermis as epidermal acrospiroma and those involving both the epidermis and dermis as juxtaepidermal acrospiroma or just eccrine poroma. Those which are confined exclusively to the dermis or have minimal connection to the epidermis are termed as dermal acrospiroma or hidradenoma. [1] Malignant acrospiroma comprises a group of rare epidermal, juxtaepidermal, and dermal ductal carcinoma occurring over the head and neck, anterior trunk, or extremities. [4],[5] One series described an incidence of only five cases in a group of 750,000 cases over an eight-year period. [6] Another series from Russia described malignant transformation in d six cases out of 105 cases of acrospiroma [7] They follow a predictable pattern from the tumor site to regional lymph node and ultimately to systemic metastases. [2],[8]

The present case showed some unique features that need documentation. Malignant acrospiromas tend to be of moderate size; the largest reported malignant tumor was 4 cm. [3],[9] In the present case, the lesion was widespread with the largest nodule measuring about 11 cm and the full extent of the lesions on the thorax and abdomen were of about 30 cm. Reports in the literature describe acrospiroma to be a slowly growing tumor both for benign and malignant variants. [9] However, in the present case, the history was quite short with lesions appearing in crops over seven to eight months. The histology of the first lesion was benign eccrine poroma and the subsequent local recurrence and its malignant histology and biological aggressive behavior point to the evidence of malignant transformation in a benign lesion.

Malignant acrospiromas are treated by wide local excision, with a local recurrence rate of around 50%. [10] Holden described the use of wide local excision with adjuvant radiotherapy for malignant eccrine acrospiroma of the scalp and left parotid, which eventually had local recurrence in the parotid region after two years. [2] Headington, et al. describes a more radical surgical approach of amputation of the leg and regional lymph node dissection that was required for clinical control of extremity acrospiroma. [11] William, et al. reported the case of a 66-year-old female with a recurrent malignant acrospiroma of the chest treated by wide radical resection, including chest wall excision, followed by reconstructive surgery and radiotherapy. After 16 months, there was no evidence of local recurrence or distant metastasis. [12]

Harari, et al. described the role of radiotherapy in malignant eccrine acrospiroma, wherein three cases of malignant acrospiroma were treated with postoperative radiotherapy with doses of around 71-76 Gy to the primary surgical bed and about 50 Gy to the draining lymph node basin, with modest disease-free survival (27 and 35 months) in two of the three cases. They suggested that certain histological features like dermal lymphatic invasion, nerve sheath involvement, deep structure infiltration, positive resected margin, and extracapsular lymph node extension may identify a high risk of recurrence and merit postoperative radiotherapy. [13] The role of chemotherapy in eccrine sweat gland carcinomas, and especially malignant acrospiromas is not clear. Various case reports and case series have reported the use of a multitude of drugs in various sweat gland carcinomas like cyclophosphamide and doxorubicin, bleomycin, cisplatin, mitomycin C, and so on, with partial response and a median duration of response of 4 to 16 months. [14],[15],[16] Isolated reports of response to taxanes (docetaxel and paclitaxel) are also present.

The present case had widespread lesions involving the upper abdomen and chest wall. Radical excision was deemed not possible and not feasible. The role of definitive radiotherapy is not described in the literature. Hence the option of chemotherapy to reduce the bulk of the lesions followed by a trial of definitive radiotherapy was planned for this patient. However, all of the chemotherapy regimens used including paclitaxel failed to show any significant clinical response.

Analyzing all the available literature, we conclude that wide local excision is the treatment of choice for these rare skin appendage tumors, when localized, and adjuvant radiotherapy provides some additional benefit in local control. Multiagent chemotherapy thought of as an option for more extensive lesions, as in the present case, did not show much benefit, indicating a relative incurability of the tumor. However, paclitaxel as a single agent could provide a viable option for palliation, though more evidence in the form of case series and case reports is needed to establish the same.

   References Top

1.Fletcher CD. Diagnostic histopathology of tumors. 3 rd ed. Philadelphia: Churchill Livingstone; 2000. p. 1457-8.  Back to cited text no. 1
2.Holden B, Colome-Grimmer M, Savage C, Stierman K, Pou AM. Malignant eccrine acrospiroma with metastases to the parotid. Ear Nose Throat J 2002;81:352-5.  Back to cited text no. 2
3.Johnson BL, Helwig EB. Eccine acrospiroma. A clinic-pathologic study. Cancer 1969;23:641-57.  Back to cited text no. 3
4.Cooper PH. Carcinomas of sweat glands. Pathol Annu 1987;22:83-110.  Back to cited text no. 4
5.Santa Cruz DJ. Sweat glands carcinomas: A comprehensive review. Semin Diagn Pathol 1987;4:38-74.  Back to cited text no. 5
6.Abenoza P, Ackerman AB, Di Leonardo M. Porocarcinomas. Chapter 15. In: Neoplasms with eccrine differentiation. Philadelphia: Lea and Febiger; p. 415-31.  Back to cited text no. 6
7.Poroshin KK, Vavilov AM, Ostep NM, Seredniakova NI. Acrospiroma (clinic-morphological characteristics and histogenesis). Arkh Pathol 1986;48:70-6.  Back to cited text no. 7
8.Ogilvie JW. Malignant transformation of eccrine tumours. J Cutan Pathol 1987;14:15-22.  Back to cited text no. 8
9.Hunt SJ, Santa Cruz DJ, Kerl H. Giant eccrine acrospiroma. J Am Acad Dermatol 1990;23:663-8.  Back to cited text no. 9
10.Wilson KM, Jubert AV, Joseph JI. Sweat gland carcinoma of the hand (malignant acrospiroma). J Hand Surg 1989;14:531-5.  Back to cited text no. 10
11.Headington JT, Niederhuber JE, Beals TF. Malignant clear cell acrospiroma. Cancer 1978:41:641-7.  Back to cited text no. 11
12.William PL, Dupin C, Levine EA. Recurrent malignant Acrospiroma: Treatment by chest wall excision. Dermatol Surg 1998;24:908-12.  Back to cited text no. 12
13.Harari PM, Shimm DS, Bangert JL, Cassady JR. The role of radiotherapy in the treatment of malignant sweat gland neoplasms. Cancer 1990;65:1737-40.  Back to cited text no. 13
14.Coonley CJ, Schauer P, Kelsen DP, Sordillo P, Huvos AG. Chemotherapy of metastatic sweat gland carcinoma. A retrospective review. Am J Clin Oncol 1985;8:307-11.  Back to cited text no. 14
15.Briscoe KE, Grage T, Kennedy BJ. Sustained complete remission of metastatic sweat gland carcinoma. JAMA 1978;240:51-2.  Back to cited text no. 15
16.Piedbois P, Breau JL, Morere JF, Isreal L. Sweat gland carcinoma with bone and visceral metastases-prolonged complete remission lasting 16 months as a result of chemotherapy. Cancer 1987;60:170-2.  Back to cited text no. 16

What is new? Extensive involvement, unresponsive to multiagent chemotherapy. Good palliation with single agent Paclitaxel


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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