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  Table of Contents    
CASE REPORT  
Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 398-401
A rare case of malignant eccrine spiradenoma mimicking carcinosarcoma


1 Department of Pathology, M R Bangur Super Speciality Hospital, Kolkata, West Bengal, India
2 Department of Pathology, Vivekananda Institute of Medical Science, Kolkata, West Bengal, India
3 Department of Surgery, M R Bangur Super Speciality Hospital, Kolkata, West Bengal, India

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Date of Submission16-Apr-2020
Date of Decision22-May-2020
Date of Acceptance27-Jul-2020
Date of Web Publication9-Apr-2021
 

   Abstract 


Malignant eccrine spiradenoma is an extremely rare neoplasm of adnexal origin. It almost always originates from a preexisting long standing eccrine spiradenoma. We present a case of malignant eccrine spiradenoma arising from benign counterpart and having both carcinomatous and sarcomatous differentiation. Here we present a case of a 46 years old lady who presented with a long standing small nodule on her left leg of 7 years' duration with suddenly increase in size. Grossly the mass was partly solid and partly cystic measuring 11.5 cm in maximum dimension with cystic area forming the deeper plane. On microscopy, the superficial dermis showed well demarcated lobules of benign eccrine spiradenoma. Deeper dermis showed tumor cells with features of malignant transformation having both carcinomatous and sarcomatous component. After wide local excision patient is now doing well. The diagnosis of malignant eccrine spiradenoma requires a thorough histopathological examination of the lesion and requires finding a focus of benign spiradenoma within or adjacent to malignant tumour. Wide local excision and close follow-up for early detection of recurrence and metastasis is the mostly recommended management modality.

Keywords: Carcinosarcoma, malignant eccrine spiradenoma, neoplasm

How to cite this article:
Kundu A, Mondal TR, Ghosh S, Dey S, Mahapatra AS. A rare case of malignant eccrine spiradenoma mimicking carcinosarcoma. Indian J Pathol Microbiol 2021;64:398-401

How to cite this URL:
Kundu A, Mondal TR, Ghosh S, Dey S, Mahapatra AS. A rare case of malignant eccrine spiradenoma mimicking carcinosarcoma. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 8];64:398-401. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/398/313287





   Introduction Top


Eccrine spiradenoma is a benign skin adnexal neoplasm that reiterates primitive eccrine ductular and secretory epithelium. It usually presents as a single nodular swelling having slow growth pattern. Malignant eccrine spiradenoma is an extremely rare neoplasm of adnexal origin. It almost always originates from a preexisting long standing eccrine spiradenoma. The first such case of malignant transformation was reported by Dabska in 1972.[1] Since then, only 120 cases have been reported in the literature.[2] Morphological and histochemical behavior of this malignant tumour is variable. Most cases show only carcinomatous differentiation whereas fewer cases show carcinosarcomatous differentiation. Only 10 cases of carcinosarcoma have been found among 54 reported malignant eccrine spiradenoma till 2002.[3] We present an additional case of malignant eccrine spiradenoma arising from benign counterpart and having both carcinomatous and sarcomatous differentiation.


   Case Report Top


A 46-year-old lady presented with a long standing small nodule on her left leg of 7 years' duration with rapid increase in size over a period 6 months associated with pain. X-ray showed soft tissue lesion with no bony involvement. It was excised and sent.

The surgical specimen consisted of an elliptical skin covered soft tissue measuring 13.5 cm × 7 cm × 5 cm. The size of the mass which was partly solid partly cystic [Figure 1] was 11.5 cm × 6 cm × 4 cm and the cystic area forming the deeper plane. The lesion grossly involved the dermis and subcutaneous tissue.
Figure 1: Macroscopic view of tumour showing solid and cystic component

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On microscopic examination, epidermis was unremarkable. The superficial dermis showed well demarcated lobules composed of central pale cells and peripheral basaloid cells arranged in intertwining cords and forming lumina [Figure 2]a. These lobules were consistent with eccrine spiradenoma. In the deeper part of dermis pleomorphic, tumour cells were seen arranged in solid nests with central necrosis, occasional irregular glandular structure and papillary fronds [Figure 2]c. Areas of cystic degeneration and hemorrhage were present. The tumour cells had pleomorphic vesicular nuclei with variably prominent nucleoli with scanty cytoplasm [Figure 2]d. Foci of squamous differentiation with central keratinization were noted [Figure 2]b. The mitotic activity was very high in the atypical cells (10/10 HPF). Intervening stroma showed pleomorphic spindle cell along with bizarre tumor cells having marked atypia and multinucleated giant cells [Figure 2]e and [Figure 2]f. The pleomorphic sarcomatoid areas showed very high mitotic count (15/10 HPF). Osteoid formation was noted in a small area. No lymphovascular invasion was detected.
Figure 2: (a) Foci of spiradenoma in overlying dermis (H &E, X100) (b) tumour comprising of irregular malignant glands along with foci of keratinization surrounded by squamous cells (H &E, X100) (c) solid nests with and without central necrosis along with irregular glands (H &E, X100) (d) pleomorphic tumour cells having vesicular nuclei, conspicuous nucleoli and scanty cytoplasm (H &E, X400) (e) pleomorphic sarcomatous stroma along with irregular glands (H &E, X100)(f) sarcomatous

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Pan cytokeratin (AE1/AE3) showed positivity for glandular and squamous component and negative staining in sarcomatous area [Figure 3]a. Tumour cells in carcinomatous area were positive for EMA, CEA, CK7 [Figure 3]b and negative for vimentin. Tumour cells are found to be positive for vimentin and negative for EMA, CEA, CK7 in sarcomatous area. Immunohistochemistry for P53 was also positive in malignant cells [Figure 3]c.
Figure 3: (a) Membranous and cytoplasmic positive staining of Pan cytokeratin (AE1/AE3) in glandular and squamous area (X100) (b) CK7 positivity in carcinomatous areas with negative staining in sarcomatous stroma (X400) (c) P53 staining in malignant cells (X400)

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After wide local excision patient was followed up for last eight months and no recurrence, regional lymph node involvement, distant metastasis or PET avid areas could be detected on subsequent investigations. Patient is currently doing well.


   Discussion Top


In most cases of malignant eccrine spiradenoma clinical features are very much similar. It usually presents between 21-92 years of age with no sex predilection. This tumour can occur at any skin surface. 92% of reported cases involve extremities and trunk.[3] Rare sites of occurrence are breast, vulva etc.[4],[5],[6] The average size at the time of presentation is 3.9 cm (range 0.5-15).[3] The malignant transformation may occur from a previous benign eccrine spiradenoma after a long time, which may be as long as 75 years.[7] It generally comes to medical attention when preexisting lesion rapidly increases in size, changes color, ulcerates, or becomes painful and tender. Fortunately this malignant transformation is extremely rare. Malignant sweat gland tumours account for only 0.005% among skin tumours.[8]

The diagnosis of malignant eccrine spiradenoma requires a thorough and extensive histopathological examination of the lesion and requires finding a focus of benign spiradenoma within or adjacent to malignant tumour. In absence of benign focus, tumour may be confused with other skin malignancies and metastatic disease. Carcinosarcoma can be a primary tumour within the skin without a benign sweat gland neoplasm or may be metastatic deposit.[9] Histological proliferation of cells with high mitotic rate, loss of periodic acid Schiff (PAS) positive basement membrane and invasion of surrounding tissue characterize malignant features. Malignant eccrine spiradenoma usually show only carcinomatous, only sarcomatous or rarely both carcinomatous and sarcomatous differentiation.[1],[2],[3],[4],[5],[6],[7],[9],[10],[11],[12] It frequently shows focal squamous differentiation, which may be florid in some cases.[13] It can be highly vascular and cause confusion with vascular neoplasm.[14] The mesenchymal elements are often non specific spindle cells but rhabdomyosarcoma, osteosarcoma, leiomyosarcoma and chondrosarcoma have been described along with carcinoma. Immunohistochemically, these tumours exhibit variable expression of cytokeratins, carcinoembryonic antigen, epithelial membrane antigen and S100 protein in carcinomatous area, whereas sarcomatous areas exhibit variable expression of vimentin, desmin etc.[2],[6],[7],[10],[11],[12],[14] Overexpression of p53 protein has been demonstrated association with malignant transformation.[6],[7]

Most cases have been treated with wide local excision.[15] Lymphnode dissection has been done during primary surgery in case of nodal metastasis at the time of presentation in some cases. Recurrence in the limbs treated with amputation has occurred in a few cases. Malignant eccrine spiradenoma metastasizes to regional lymphnode, lung, liver, brain, and bone. Fewer cases have been treated with chemotherapy or radiotherapy or both postoperatively mainly in metastatic disease, but it has not shown to increase overall survival or provide palliation significantly.[2],[7],[16] Role of post operative radiation therapy, chemotherapy and prophylactic lymphnode resection still remain to be determined.[16] Close follow up of patients for detection of early recurrence and metastasis cannot be overemphasized. Prognosis of malignant eccrine spiradenoma is variable. Many cases show disease free survival without any recurrence or metastasis but few cases have early metastasis leading to early death.[1],[2],[7],[16] It appears that tumors with sarcomatous component are aggressive in nature with poor prognosis.

Malignant eccrine spiradenoma is an extremely rare tumour having variable carcinomatous and/or sarcomatous component without any specific immunohistochemical signature. Diagnosis stands upon meticulous grossing and extensive sampling to find out foci of benign spiradenoma and excluding differentials by sound histomorphological examination along with a comprehensive panel of immunohistochemistry. The challenge is to rule out metastatic deposit, the prognosis and treatment of which is radically different.

A further molecular profiling of number of cases of this entity is desirable to resolve the mystery of its morphological diversity and behavioural variation. Hence reporting these cases and their recognition is important for archiving and further molecular testing.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Dabska M. Malignant transformation of eccrine spiradenoma. Polish Med J 1972;11:388-96.  Back to cited text no. 1
    
2.
Staiger RD, Helmchen B, Papet C, Mattiello D, Zingg U. Spiradenocarcinoma: A comprehensive data review. Am J Dermatopathol 2017;39:715-25.  Back to cited text no. 2
    
3.
Mirza I, Kloss R, Sieber SC. Malignant eccrine spiradenoma. Arch Pathol Lab Med 2002; 126:591-4.  Back to cited text no. 3
    
4.
de Andrés Gómez A, Moratalla CN, Ferrer FV, Sabater Marco V, García-Vilanova A, Fuster Diana C, et al. Malignant eccrine breast spiradenoma. A case report and literature review. Int J Surg Case Rep 2015;15:81-4.  Back to cited text no. 4
    
5.
Chen G, Cheuk W, Cheung JS, Chan JK. Carcinosarcoma ex eccrine spiradenoma of the vulva: Report of the first case. Int J Gynecol Pathol 2011;30:301-5.  Back to cited text no. 5
    
6.
Catteau X, D'haene N, Noël JC. Low grade malignant eccrine spiradenoma of the vulva: Case report, review of the literature and discussion about the role of p53 and HPV. Diagn Pathol 2020;15:24.  Back to cited text no. 6
    
7.
Ishikawa M, Nakanishi Y, Yamazaki N, Yamamoto A. Malignant eccrine spiradenoma: A case report and review of the literature. Dermatol Surg 2001;27:67-70.  Back to cited text no. 7
    
8.
Marenda SA, Otto RA. Adnexal carcinomas of the skin. Otolaryngol Clin North Am 1993;26:87-116.  Back to cited text no. 8
    
9.
Biernat W, Kordek R, Liberski PP, Wozniak L. Carcinosarcoma of the skin: Case report and literature review. Am J Dermatopathol 1996;18:614-9.  Back to cited text no. 9
    
10.
Itoh T, Yamamoto N, Tokunaga M. Malignant eccrine spiradenoma with smooth muscle cell differentiation: Histological and immunohistochemical study. Pathol Int 1996;46:887-93.  Back to cited text no. 10
    
11.
McCluggage WG, Fon LJ, O'rourke D, Ismail M, Hill CM, Parks TG, et al. Malignant eccrine spiradenoma with carcinomatous and sarcomatous elements. J Clin Pathol 1997;50:871-3.  Back to cited text no. 11
    
12.
Fernández-Aceñero MJ, Manzarbeitia F, de Juan MJ, Requena L. Malignant spiradenoma: Report of two cases and literature review. J Am Acad Dermatol 2001;44:395-8.  Back to cited text no. 12
    
13.
Lee HH, Lee KG. Malignant eccrine spiradenoma with florid squamous differentiation. J Korean Med Sci 1998;13:191-5.  Back to cited text no. 13
    
14.
Malhotra R. Malignant eccrine spiradenomas with hemangiomatous elements: DNA ploidy, light microscopic and immunohistochemical studies of two cases. J Cutan Pathol 1993;20:555.  Back to cited text no. 14
    
15.
Sharma A, Sengupta P, Das AK, Nigam MK, Chattopadhya S. Eccrine spiradenoma in knee. Indian J Dermatol 2014;59:513-5.  Back to cited text no. 15
[PUBMED]  [Full text]  
16.
Andreoli MT, Itani KM. Malignant eccrine spiradenoma: A meta-analysis of reported cases. Am J Surg 2011;201:695-9.  Back to cited text no. 16
    

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Correspondence Address:
Ayan Kundu
Department of Pathology, M R Bangur Super Speciality Hospital, 247, Deshpran Sasmal Road, Tollygunge, Kolkata, West Bengal - 700 033
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_390_20

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