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CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 4  |  Page : 255-258

A rare case report of an aggressive malignant spiradenoma chest wall masquerading as carcinoma of unknown origin - Course of events and review of literature


1 Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupathi, Andhra Pradesh, India
2 Department of Radiation Oncology, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupathi, Andhra Pradesh, India
3 Department of Pathology, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupathi, Andhra Pradesh, India

Date of Web Publication26-Dec-2017

Correspondence Address:
Dr. Ramana Reddy Naru
Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupathi, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_76_17

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  Abstract 


Malignant eccrine spiradenomas are exceedingly rare and aggressive tumours normally arising in long-standing benign eccrine spiradenomas. We present a case of malignant eccrine spiradenoma (MES) that was initially misdiagnosed as carcinoma of unknown origin in spite of extensive workup to look for primary and presented with a large exophytic tumour on the right side of his chest wall, which had fungated. Radiological imaging of the chest region revealed extensive involvement of the local tissues. Patient underwent wide local excision with a 1 cm margin along with right-sided axillary lymph node dissection. Histopathological findings were consistent with MES with tumour deposits in multiple lymph nodes in the axilla. Our case report shows MES is a rare and aggressive cancer, which is difficult to diagnose, highly aggressive with propensity to involve the lymph nodes. May present with local recurrence and also develop distant metastases significantly compromise the quality of life of the patient. It requires the surgeons to have a high index of suspicion for its initial diagnosis, and a multidisciplinary management with surgery, radiotherapy, and chemotherapy is required for complete treatment and needs close follow up.

Keywords: Chemotherapy, malignant eccrine spiradenoma, radiotherapy, wide local excision


How to cite this article:
Naru RR, Inamdar MB, Das P, Kottu R. A rare case report of an aggressive malignant spiradenoma chest wall masquerading as carcinoma of unknown origin - Course of events and review of literature. J NTR Univ Health Sci 2017;6:255-8

How to cite this URL:
Naru RR, Inamdar MB, Das P, Kottu R. A rare case report of an aggressive malignant spiradenoma chest wall masquerading as carcinoma of unknown origin - Course of events and review of literature. J NTR Univ Health Sci [serial online] 2017 [cited 2020 Jun 2];6:255-8. Available from: http://www.jdrntruhs.org/text.asp?2017/6/4/255/221532




  Introduction Top


Eccrine spiradenoma is a benign sweat gland tumor that commonly affects young adults. The presentation is often a single nodule that may or may not be tender. In contrast, the malignant eccrine spiradenoma (MES) is an extremely rare tumor, which almost always arises from a pre-existing eccrine spiradenoma. The overall prognosis of MES is poor.[1] Our case report describes one such aggressive presentation of MES with lymph nodal metastases that developed local recurrence as well as distant bony metastases.

Case description

A 67-year-old male presented to surgical oncology department at our tertiary care centre with chief complaints of swelling over right side of chest wall since 3 years; it was insidious in onset, gradually progressing in size but over the last one year it rapidly progressed. It was not painful. Patient does not have any history of shortness of breath, cough, and hemoptysis. There was no history of loss of appetite or loss of weight and his bowel and bladder habits are regular. He was a known smoker, 30-pack year duration. On local examination, a swelling of size 10 × 8 cm was present over right chest wall with skin involvement of area 4 × 4 cm, which was covered with slough [Figure 1]. It was variable in consistency - hard to firm, mobile, non tender. A single, mobile lymphnode of size 2 × 1 cm was palpable in the right axilla. Other systemic examination was unremarkable.
Figure 1: Intraoperative image showing tumor ulceration

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Biopsy from lesion showed possibility of adenocarcinoma deposits and Immunohisto chemistry (IHC) with CK7, CK20, and CEA were done. IHC with CK7 showed intense cytoplasmic positivity in tumor cells and CK20 and CEA were negative. We further suggested IHC with ER, PR, TTF-1 to rule out primary from adeno carcinoma lung and were all found to be negative.

Contrast-enhanced computed tomography (CECT) of the chest showed a heterogenously enhancing soft tissue density lesion with central necrosis and calcification measuring 6.6 × 5.3 cm over the anterior chest wall on right side [Figure 2]. Enlarged right axillary (2.6 × 1.8 cm) lymphnode. For metastatic workup, CECT abdomen, esophagogastroduodenoscopy, and colonoscopy were done and were normal. Tumour markers with serum carcino embryonic antigen (3.7 ng/ml), serum prostate specific antigen (0.3 ng/ml) were within normal limits. Other blood investigations were within normal limits.
Figure 2: CECT chest showing heterogenously enhancing Soft tissue density lesion with central necrosis and calcification measuring (6.6×5.3cm) is noted in anterior chest wall on right side

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Based on clinical, radiological, and pathological workup we diagnosed this case as carcinoma of unknown primary (adenocarcinoma) presenting as right chest wall mass with axillary lymphadenopathy.

We referred the case to medical oncology for further management; they started the patient on chemotherapy with carboplatin and paclitaxel. However, after one cycle of chemotherapy with the above mentioned drugs the lesion was progressing in size, the ulcer with foul smelling discharge had increased. As the lesion was increasing in size after having interdisciplinary discussion in tumour board, it was decided to have a second opinion of the histopathology slides and blocks. The slides and blocks were sent to a higher tertiary care centre there it was opined as possibility of skin adnexal neoplasm probably of sweat gland origin suggestive of benign tumor; however, possibility of low grade malignancy cannot be ruled out. After extensive workup, patient was taken up for surgery and a wide local excision and right-sided axillary lymph node dissection was done. Microscopic sections showed stratified squamous epidermis with an infiltrating ill circumscribed lesion in the dermis comprising of lobules, sheets, islands, ill-defined ducts, tubules, and nests of tumor cells displaying two distinct cellular patterns peripherally placed dark basaloid cells displaying moderate bluish cytoplasm. Centrally placed large cells with round to mild pleomorphic vesicular nucleus, abundant pale eosinophilic to vacuolated cytoplasm [Figure 3] and [Figure 4]. Lymphovascular emboli were present. The final histopathology impression was spiradenocarcinoma of chest wall with 5/27 axillary lymphnodes showing tumor deposits. Postoperative recovery was uneventful [Figure 5].
Figure 3: Photomicrograph(H & E 4X) showing tumor cells along with necrosis in dermis

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Figure 4: Photomicrograph(H & E 20X) showing small lymphocyte like cell with hyperchromatic nuclei(orange arrow)larger epithelioid cell with open chromatin(green arrow)

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Figure 5: Postoperative image

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After having an interdisciplinary discussion post surgery adjuvant radiotherapy was decided and with inclusion of chest wall, right axilla, and right supraclavicular fossa in the radiotherapy field. But the patient defaulted from receiving adjuvant radiotherapy and was lost to follow up for one year. One year later he presented to surgical oncology department with history of recurrence. On examination, a swelling of size 8 × 4 cms is present over the right chest wall, which was fixed to underlying ribs clinically, located from clavicle superiorly till the 4th intercostals space. Another swelling on the lower aspect of right side of the neck measuring 6 × 5 cms, fixed and hard at level V cervical lymph node region on right side. We further investigated with FNAC, right cervical node and left axillary node it was opined as recurrence. Hence, we further investigated with Bone scan F18; it revealed local infiltration of 1st and 2nd ribs, with evidence of distant metastases involving left ala of sacrum and left ilium.

In view of metastatic disease, patient was referred to medical oncology for further management. Patient received 3 cycles of chemotherapy with cisplatin and paclitaxel.

Defaulted for 3 months and presented with a large mass over right side of the chest, which was fungating and foul smelling and thereafter received 2 more cycles of chemotherapy, thereby completing a total of five cycles. Palliative radiotherapy was planned in view of symptomatic bony metastases with 39 gray in 13 fractions, patient completed radiotherapy and patient is on follow up at present and doing well.


  Discussion Top


Spiradenocarcinoma (also known as malignant spiradenoma and sweat gland carcinoma ex eccrine spiradenoma)[2] was first described by Dabska [3] in 1972, 16 years after Kersting and Helwig [4] originally described its benign precursor. Malignant eccrine spiradinoma is a rare adnexal carcinoma of the skin. It is normally considered to be a malignant transformation of a pre-existing benign eccrine spriradenoma.[5] MES presents at an average age of 59 years (range: 21–92 years) and shows no sex predilection.[6] It tends to preferentially involve the trunk and extremities (92% of reported cases),[7] but there are also published case reports of MES arising in the breast,[6] scalp,[8],[9] and eyelids. Overall prognosis of malignant eccrine spiradenoma is poor.[2]

Primary treatment includes local excision, with or without regional lymphadenectomy,[1] with recurrence reported in 17.5% of the cases.[6] Malignant eccrine spiradenoma metastasizes to regional lymph nodes,[5] lungs, brain, skin,[4] bone,[4] and liver [7] (in descending order of frequency). Distant metastases of MES are uncommon even in extensive tumours, such as the one reported here, metastasis are rare but carry a poor prognosis.[7] Radiation therapy alone or in combination with chemotherapy has been used with no benefit in the treatment of patients with metastatic MES.[9] Sridhar et al. reported symptomatic improvement and shrinkage of the tumor with tamoxifen therapy in a patient with estrogen receptor-positive eccrine adenocarcinoma.[10] However, the role of hormonal therapy still remains to be determined. Close follow-up of these patients for early detection of recurrence and metastases cannot be overemphasized.

In a meta analysis review,[11] The majority of non-metastatic disease with no known lymph node involvement were treated with local resection, with 3 patients undergoing additional lymph node dissections. Of 36 total cases of non-metastatic disease, the 35 patients treated with local surgical resection alone yielded a disease-free survival rate of 100% with a mean follow-up period of 33 months. The remaining patient underwent an incomplete superficial excision for initial treatment because the etiology of the tumor was not fully understood at that time. The persistent locoregional disease was later treated unsuccessfully with radiotherapy, necessitating a subsequent more radical surgical intervention 10 months later to remove the residual tumor. Twelve patients showed lymph node metastasis without distant metastasis. All of these patients underwent surgical resection of the tumor, with 7 patients receiving additional lymph node dissection. Of the 12 total patients, 3 patients without lymph node dissection died of metastatic disease (mean survival, 45 months); 1 patient was alive with disease 24 months after lymph node dissection; and 8 patients (6 of who underwent lymph node dissection) lacked evidence of recurrent disease at final follow-up evaluation. Twenty-four patients presented with distant metastasis, with a median survival of 16 months. The treatments varied widely in this subset of patients: 13 patients underwent local resection (1 patient underwent lymph node dissection in addition) and 11 were given adjuvant therapy in addition to surgery. These adjuvant modalities included five instances of radiotherapy alone, 3 cases of chemotherapy alone, and 3 occurrences of chemoradiotherapy. The median survival time for the surgical group was 12 months. Surgery with adjuvant therapy yielded a median survival time of 20 months. However, the survival curve did not significantly differ between the surgery and surgery plus adjuvant therapy treatment groups.


  Conclusion Top


Our case report shows malignant spiradenoma is a rare and aggressive cancer, which is difficult to diagnose, highly aggressive with propensity to involve the lymph nodes. May present with local recurrence and also develop distant metastases significantly compromising the quality of life of the patient. It requires the surgeons to have a high index of suspicion for its initial diagnosis, and a multimodality mode of treatment with surgery, radiotherapy, and chemotherapy is required for complete treatment and needs close follow up.

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.
Meyer TK, Rhee JS, Smith MM, Cruz MJ, Osipov VO, Wackym PA. External auditory canal eccrine spiradenocarcinoma: A case report and review of literature. Head Neck 2003;25:505-10.  Back to cited text no. 1
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2.
Agarwal S, Khanna R, Arya NC, Khanna AK. Malignant eccrine spiradenoma. An unusual presentation. Indian J Dermatol Venereol Leprol 2002;68:290-1.  Back to cited text no. 2
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3.
Dabska M. Malignant transformation of eccrine spiradenoma. Pol Med J 1972;11:388-96.  Back to cited text no. 3
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4.
Kersting E, Helwig EB. Eccrine spiradenoma. AMA Arch Derm 1956;73:199-227.  Back to cited text no. 4
    
5.
Yildirim S, Akoz T, Akan M, Ege GA. De novo malignant eccrine spiradenoma with an interesting and unusual location. Dermatol Surg 2001;27:417-20.  Back to cited text no. 5
    
6.
Mirza I, Kloss R, Sieber SC. Malignant eccrine spiradenoma. Arch Pathol Lab Med 2002;126:591-4.  Back to cited text no. 6
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Russ BW, Meffert J, Bernert R. Spiradeno carcinoma of the scalp. Cutis 2002;69:455-8.  Back to cited text no. 7
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8.
Gupta S, Radotra BD, Kaur I, Handa S, Kumar B. Multiple linear eccrine spiradenomas with eyelid involvement. J Eur Acad Dermatol Venereol 2001;15:163-6.  Back to cited text no. 8
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Otero-Garcia JE, Carlo VJ, Trinidad-Pinedo J. Malignant eccrine spiradenoma of the neck: A case report. Otolaryngol Head Neck Surg 2001;125:428.  Back to cited text no. 9
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10.
Sridhar KS, Benedetto P, Otrakji CL, Charyulu KK. Response of eccrine adenocarcinoma to tamoxifen. Cancer 1989;64:366-70.  Back to cited text no. 10
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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. 11
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    Figures

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



 

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