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Malignant eccrine spiradenoma. (Case Reports).

In 1956, Kersting and Helwig (1) first described spiradenoma as a skin adnexal neoplasm with a slow growth pattern. Eccrine spiradenoma is now known as a benign sweat gland tumor that commonly affects young adults. The presentation is often that of a single nodule that may or may not be tender. In contrast, the malignant eccrine spiradenoma (MES; spiradenocarcinoma) is an extremely rare tumor, which almost always arises from a preexisting eccrine spiradenoma. Since the first reported case of this malignant transformation by Dabska in 1972, (2) fewer than 60 additional cases of MES have been reported. (3-12) In this report, we present an additional case of MES and recount the collective experience with this malignancy.

REPORT OF A CASE

A 70-year-old white man presented with an ulcerated lesion on his left upper arm. He reported that the lesion had started as a small lump, "about the size of a jelly-bean," a year before presentation. The lesion had abruptly started to enlarge, with recent ulceration and bleeding. On examination, a 3.0 x 2.5-cm left axillary mass was also noted. The skin lesion and the axillary mass were surgically excised. A 15 x 8 x 2-cm, tan-white skin ellipse was received in the surgical pathology laboratory. The skin surface was remarkable for a centrally located, ill-defined, indurated nodule. On serial sectioning, a 7 cm in diameter, partially encapsulated, multiloculated, cystic and solid, tan-yellow mass containing approximately 30 mL of serosanguinous fluid was noted. The lesion grossly involved the deep dermis and the subcutaneous tissue. However, the margins of resection were not involved. Nine lymph nodes retrieved from axillary dissection were submitted for histologic evaluation.

On microscopic examination, the dermis and subcutaneous tissue showed infiltration by a malignant adnexal neoplasm arranged in ragged sheets, solid masses with central necrosis, nests, cords, and occasional irregular glandular structures (Figure 1). Multiple areas of tumor necrosis, cystic degeneration, and old hemorrhage were present. The tumor cells had pleomorphic vesicular nuclei with variably prominent nucleoli and moderate amounts of amphophilic cytoplasm (Figure 2, A). Few small foci of squamous differentiation with central keratinization were noted (Figure 2, B). The mitotic activity was high, and a prominent desmoplastic inflammatory host response was observed. No lymphovascular invasion was recognized. This high-grade eccrine carcinoma was noted to be arising from a preexisting eccrine spiradenoma, seen as sharply demarcated lobules composed of 2 cell populations (peripheral basaloid cells and central pale cells) arranged in nests and interanastomosing trabecula with scattered glandular lumina and focal cystic changes (Figure 3). The overlying epidermis was unremarkable. Two of the 9 axillary lymph nodes were positive for metastatic carcinoma.

[FIGURES 1-3 OMITTED]

Metastatic workup (bone scan, computed tomographic scans of the chest, abdomen, and pelvis) was negative. In light of the positive estrogen receptor immunostain on the primary tumor (Figure 2, C), the patient was started on tamoxifen (20 mg/d). No adjuvant radiation or chemotherapy was administered. The patient has since been seen in follow-up every 3 months and has had yearly chest radiographs. After 41 months of follow-up, he is doing well and has no evidence of recurrent or metastatic disease. He continues to take tamoxifen, with plans for at least 5 years of treatment with this drug.

COMMENT

The clinical presentation of MES is similar in most cases (Table 1). It presents at an average age of 59 years (range, 21-92 years) and shows no sex predilection. It tends to preferentially involve trunk and extremities (92% of reported cases). There is also a single case report of MES arising in the breast. (7) The average size of MES at presentation is 3.9 cm (range, 0.5-15 cm). The malignancy almost always arises from a preexisting benign eccrine spiradenoma after a variable latent period, which may be as long as 75 years. (11) It generally begets medical attention when a preexisting undiagnosed lesion rapidly enlarges, changes color, ulcerates, or becomes painful and tender. This malignant transformation is fortunately extremely rare. According to the estimates of Marenda and Otto, (13) malignant sweat gland tumors account for only 0.005% of all skin tumors. To further attest to the rarity of this tumor, MES was not encountered in a large series of sweat gland carcinomas recently reported by Urso et al. (14)

The diagnosis of MES is based on histopathologic examination of the lesion and requires finding a focus of benign spiradenoma within or adjacent to the malignant tumor. In the absence of a benign focus, the tumor can be confused microscopically with other skin malignancies and metastatic carcinoma. Histologically, proliferation of cells with hyperchromatic nuclei, increased mitoses, loss of periodic acid-Schiff-positive basement membrane, and invasion of the surrounding tissues characterize malignant transformation in eccrine spiradenoma. Malignant eccrine spiradenoma frequently shows focal squamous differentiation, which may be florid in rare instances. (11) It may also be highly vascular. Indeed, increased vascularity of MES and its benign counterpart may lead to diagnostic confusion with a vascular neoplasm. (5,15) Immunohistochemically, these tumors exhibit variable expression of cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, and S100 protein. (5-8,11,12,16) Overexpression of p53 protein in MES has been associated with malignant transformation. (11) The malignant transformation is usually into a carcinoma; however, carcinosarcomatous transformation has also been reported (Table 2). The mesenchymal elements are often nonspecific spindle cells, but rhabdomyosarcoma, osteosarcoma, leiomyosarcoma, and chondrosarcoma have been described in association with carcinoma arising in eccrine spiradenoma. (2-4,6-8,11,12)

The mainstay of therapy is surgical excision, which may be curative in some cases (Table 1). Regional lymph nodes should be dissected if tumor metastases are suspected clinically. Recurrence was reported in 7 (17.5%) of 40 cases. (3,11) A wider reexcision was performed in 4 of these 7 cases, of which 2 were on the extremities and 2 on the trunk. (11) Two patients also received local radiation therapy after reexcision. (11) Recurrences on the limbs were treated with amputation in the other 3 cases. (3,11) Malignant eccrine spiradenoma metastasizes to regional lymph nodes, lungs, brain, and liver (in descending order of frequency). While distant metastases of MES are uncommon, they generally portend an ominous prognosis. After a mean follow-up period of 35.23 months in 40 cases, 16 (40%) patients developed lymph node or distant organ metastases (Table 1). Seven (17.5%) of the 40 patients with MES developed distant metastases and died of disease after a mean of 11.1 months following diagnosis. (2-4,11) It is of interest that 4 of these patients (cases 2, 17, 18, and 51 in Table 1) had carcinosarcoma arising from eccrine spiradenoma. (2-4,11) Granter et al (10) recently suggested that the prognosis of patients with MES is generally not as grave as previously reported. In their series of 12 cases, albeit with limited follow-up, only 1 had developed metastases, but none had died of malignancy. Notably, there were no cases of carcinosarcoma arising in eccrine spiradenoma in their study. Perhaps MES is prognostically different from carcinosarcoma arising in eccrine spiradenoma.

Radiation therapy alone or in combination with chemotherapy has been used unsuccessfully in the treatment of patients with metastatic MES. (4,11) It is generally known that sweat gland tumors are radioresistant, and radiation therapy has little role in the management of these tumors. (17) Due to the rarity of these lesions, the experience with chemotherapy is limited. (11) Sridhar et al (18) reported symptomatic improvement and shrinkage of the tumor with tamoxifen therapy in a patient with estrogen receptor-positive eccrine adenocarcinoma. We also analyzed estrogen receptor status in our case of MES by immunohistochemistry and instituted tamoxifen therapy after obtaining positive results. The treatment has since been well tolerated, and no distant metastases have been detected after 41 months of follow-up. We believe that hormonal receptor status should be evaluated for potential therapeutic options. However, the roles of hormonal therapy and other modalities, such as localized postoperative radiation therapy, prophylactic lymph node dissection, and chemotherapy, still remain to be determined. Close follow-up of these patients for early detection of recurrence and metastases cannot be overemphasized.
Table 1. Cases of Malignant Eccrine Spiradenoma
Reported in the Literature *

Case Age, y/
No. Sex Site Size, cm Recurrence Metastases

 1 48/F E 10 ... ...
 2 76/F T 3 No Br, Bo, Lg
 3 74/F E 1.5 Yes No
 4 26/F T 3 No No
 5 67/F E >3 No LN
 6 50/M T 1.8 ... ...
 7 60/M H NA No No
 8 76/M E 3 Yes Lg

 9 36/M E 2 Yes No
 10 85/F E 3 No No
 11 59/M T 5 No No
 12 59/M E 3 No LN, Br
 13 50/F T 2 No No
 14 39/M T 0.8 No No
 15 35/F E 1 No No
 16 67/M E 3 No LN, Lg
 17 60/M E 3 Yes Lg
 18 52/F E 6 No LN, Mesen, Li
 19 45/F E 1 No No
 20 40/M E 2.5 No Lg
 21 22/F T NA Yes LN
 22 21/M E 4 No No

 23 52/F T 4 No No
 24 51/M T 1.5 No No
 25 68/M T 11 No LN
 26 48/F T NA ... No
 27 72/M E NA ... No
 28 72/F E 4 No LN
 29 67/M E 5 No No
 30 57/M E 3 No Lg
 31 72/M H 0.5 No No
 32 68/F T 2 ... ...
 33 68/F Br 4 ... ...
 34 60/F E 3 No ...
 35 72/F E 3 Yes LN, Bo, Lg

 36 54/F T 2.8 No No
 37 60/M T 15 No LN, Lg
 38 60/F H 1.9 No No
 39 46/F T 3.2 No LN
 40 38/F E 4 ... ...
 41 79/F T >6 ... ...
 42 38/F T 4 ... ...
 43 83/M E 5.5 ... ...
 44 78/F T 10 No No
 45 51/M E 5 No No
 46 88/F H 3 ... ...
 47 60/M T 5 No No
 48 48/M H 2.4 ... ...
 49 77/M T 2.3 ... ...
 50 60/M T 4 No No
 51 37/F T >3 Yes LN, Lg, Li,
 Ki, Br, Sk
 52 72/M T 2.8 ... ...
 53 92/M T 5 No N
 54 70/M E 7 No LN

Case Follow-up, Reference
No. Treatment Status mo No.

 1 Res NA ... 2
 2 Res DOD 11 2 ([dagger])
 3 Res, RT ANED 24 11 ([dagger])
 4 Res ANED 6 11 ([dagger])
 5 Res, LND ANED 84 11 ([dagger])
 6 Res NA ... 11 ([dagger])
 7 Res DOUD 120 11 ([dagger])
 8 Res, Amput, AWD 36 11 ([dagger])
 Chemo, RT
 9 Res, Amput ANED 15 11 ([dagger])
 10 Res ANED 8 11 ([dagger])
 11 Res ANED 96 11 ([dagger])
 12 Res, LND DOD 7 11 ([dagger])
 13 Res ANED 8 11 ([dagger])
 14 Res ANED 2 11 ([dagger])
 15 Res ANED 19 11 ([dagger])
 16 Res, LND, RT DOD 5 11 ([dagger])
 17 Res, Amput DOD 2 11 ([dagger])
 18 Res, RT DOD 26 11 ([dagger])
 19 Res ANED 156 11 ([dagger])
 20 Res, RT ANED 144 11 ([dagger])
 21 Res, LND ANED 2 11 ([dagger])
 22 Res, LND, ANED 24 11 ([dagger])
 Amput
 23 Res ANED 9 11 ([dagger])
 24 Res ANED 97 11 ([dagger])
 25 Res, LND ANED 29 11 ([dagger])
 26 Res NA ... 5 ([dagger])
 27 Res NA ... 5
 28 Res, LND ANED 5 11 ([dagger])
 29 Res ANED 6 11 ([dagger])
 30 Res ANED 6 11 ([dagger])
 31 Res ANED 24 11 ([dagger])
 32 Res NA ... 6
 33 Res NA ... 7
 34 Res ANED 15 12 ([dagger])
 35 Res, LND, DOD 20 11 ([dagger])
 Chemo, RT
 36 Res ANED 11 11 ([dagger])
 37 Res, LND AWD 3 11 ([dagger])
 38 Res ANED 14 9
 39 Res, LND ANED 76 10
 40 Res NA ... 10
 41 Res NA ... 10
 42 Res NA ... 10
 43 Res DOUD ... 10
 44 Res ANED 18 10
 45 Res ANED 24 10
 46 Res NA ... 10
 47 Res ANED 24 10
 48 Res ANED ... 10
 49 Res NA ... 10
 50 Res ANED 144 10
 51 Res, LND, DOD 7 11
 Chemo, RT
 52 Res NA ... 12
 53 Res DOUD 9 12
 54 Res, LND, HT ANED 41 Present case

* E indicates extremity; T, trunk; H, head and neck; Br, brain;
NA, not available; ... (ellipses), data not available; Bo, bone;
Lg, lung; LN, lymph node; Mesen, mesentery; Li, liver; Ki, kidney;
Sk, skin; Res, surgical resection; Rt, radiation therapy; LND,
lymph node dissection; Amput, amputation; Chemo, chemotherapy; HT,
hormonal therapy (tamoxifen); DOD, died of disease; ANED, alive
with no evidence of disease; DOUD, died of unrelated disease;
and AWD, alive with disease.

([dagger]) Reference listed therein.
Table 2. Malignant Eccrine Spiradenoma With Sarcomatous
Component (Carcinosarcoma)

Case No. * Morphology Reference No.

 1 Adenocarcinoma with sarcomatous
 differentiation 2
 2 Carcinoma with squamous cell and
 sarcomatous differentiation 2
 17 Carcinoma with sarcomatous
 differentiation 3
 18 Carcinoma with osteocartilagenous
 and rhabomyosarcomatous
 differentiation 4
 19 Carcinoma with osteosarcomatous
 differentiation 4
 32 Carcinoma with leiomyosarcomatous
 differentiation 6
 33 Adenocarcinoma with osteosarcomatous
 and rhabdomyosarcomatous
 differentiation 7
 37 Carcinoma with sarcomatous
 differentiation 8
 51 Carcinoma with chondrosarcomatous
 differentiation 11
 52 Carcinoma with squamous cell and
 sarcomatous differentiation 12

* As shown in Table 1.


References

(1.) Kersting E, Helwig EB. Eccrine spiradenoma. Arch Dermatol. 1956;73:199-227.

(2.) Dabska M. Malignant transformation of eccrine spiradenoma. Pol Med J. 1972;11:388-396.

(3.) Meriggi F, Tagliabo R, Morone G, et al. The potential malignancy of eccrine spiradenoma. Ital J Surg Sci. 1989;19:265-268.

(4.) McKee PH, Fletcher CDM, Stavrinos P, et al. Carcinosarcoma arising in eccrine spiradenoma: a clinicopathological and immunohistochemical study of two cases. Am J Dermatopathol. 1990;12:335-343.

(5.) Malhotra R, Kumar W, Wilbum S, et al. Malignant eccrine spiradenomas with hemangiomatous elements: DNA ploidy, light microscopy, and immunohistochemical studies of two cases [abstract]. J Cutan Pathol. 1993;20:555.

(6.) Itoh T, Yamamoto N, Tokunaga Y. Malignant eccrine spiradenoma with smooth muscle differentiation: histological and immunohistochemical study. Pathol Int. 1996;46:887-893.

(7.) Saboorian MH, Kenny M, Ashfaq R, et al. Carcinosarcoma arising in eccrine spiradenoma of the breast. Arch Pathol Lab Med. 1996;120:501-504.

(8.) McCluggage WG, Fon LJ, O'Rourke D, et al. Malignant eccrine spiradenoma with carcinomatous and sarcomatous elements. J Clin Pathol. 1997;50:871-873.

(9.) Beekley AC, Brown TA, Porter C. Malignant eccrine spiradenoma: a previously unreported presentation and review of the literature. Am Surg. 1999;65: 236-240.

(10.) Granter SR, Seeger K, Calonje E, et al. Malignant eccrine spiradenoma (spiradenocarcinoma): a clinicopathologic study of 12 cases. Am J Dermatopathol. 2000;22:97-103.

(11.) Ishikawa M, Nakanishi Y, Yamazaki N, et al. Malignant eccrine spiradenoma: a case report and review of the literature. Dermatol Surg. 2001;27:67-70.

(12.) Fernandez-Acenero MJ, Manzarbeitia F, Mestre de Juan MJ, et al. Malignant spiradenoma: report of two cases and literature review. J Am Acad Dermatol. 2001;44:395-398.

(13.) Marenda SA, Otto RA. Adnexal carcinomas of the skin. Otolaryngol Clin North Am. 1993;26:87-116.

(14.) Urso C, Bondi R, Paglierani M, et al. Carcinomas of sweat glands: report of 60 cases. Arch Pathol Lab Med. 2001;125:498-505.

(15.) Cotton DWK, Slater DN, Rooney N, et al. Giant vascular eccrine spiradenomas: a report of two cases with histology, immunohistology, and electron microscopy. Histopathology. 1986;10:1093-1099.

(16.) Swanson PE, Cherwitz DL, Neumann MP, et al. Eccrine sweat gland carcinoma: an histologic and immunohistochemical study of 32 cases. J Cutan Pathol. 1987;14:65-86.

(17.) Morris DM, Sanusi ID, Laneheart WH. Carcinoma of eccrine sweat gland: experience with chemotherapy, autopsy findings in a patient with metastatic eccrine carcinoma and a review of the literature. J Surg Oncol. 1986;31:26-30.

(18.) Sridhar KS, Benedetto P, Otrakji CL, et al. Response of eccrine adenocarcinoma to tamoxifen. Cancer. 1989;64:366-370.

Accepted for publication September 7, 2001.

From the Departments of Pathology (Drs Mirza and Sieber) and Clinical Hematology and Oncology (Dr Kloss), Danbury Hospital, Danbury, Conn.

Reprints: Steven C. Sieber, Department of Pathology & Laboratory Medicine, Danbury Hospital, 24 Hospital Ave, Danbury, CT 06810 (e-mail: steven.sieber@danhosp.org).
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Author:Mirza, Imran; Kloss, Robert; Sieber, Steven C.
Publication:Archives of Pathology & Laboratory Medicine
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Date:May 1, 2002
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