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| CASE REPORT |
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| Year : 2013 | Volume
: 5
| Issue : 4 | Page : 208-210 |
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Sweat gland adenocarcinoma of scalp
Pragya A Nair1, Kirti M Rathod2, Arvind H Chaudhary1, Abhishek P Pilani1
1 Department of Dermatology and Venereology, Pramukhswami Medical College, Karmasad, Gujarat, India
2 Department of Pahtology, Pramukhswami Medical College, Karmasad, Gujarat, India
| Date of Web Publication | 11-Apr-2014 |
Correspondence Address:
Pragya A Nair
Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad, Gujarat - 388 325
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0974-7753.130412
 Abstract | | |
Sweat gland adenocarcinoma is a rare tumor particularly over scalp. They have potential to be benign as well as distant metastasis. Usually presents with papules or nodules. Ulcerative morphology is uncommon. Wide surigical excision with regional lymph not dissection is the treatment of choice. A 42-year-old female with sweat gland adenocarcinoma of scalp is reported with cervical lymph node involvement. Keywords: Adenocarcinoma, scalp, sweat gland
How to cite this article:
Nair PA, Rathod KM, Chaudhary AH, Pilani AP. Sweat gland adenocarcinoma of scalp. Int J Trichol 2013;5:208-10 |
| Introduction | |  |
Sweat gland carcinoma (SGC) is a rare tumor particularly over scalp, which was reported first in 1865. [1] Berg and McDivitt provided a classification system for such tumor in 1968. [2] SGC occurs equally in both sexes [3] and in all races. Their most common sites are palm and sole. All metastatic SGC's spread through the lymphatics. [4] Lymphatic metastases are common followed by bone, lung and skin involvement. They show aggressive behavior with distant metastases. The overall prognosis is poor. The treatment of choice is wide excisional biopsy, with extensive excision and regional lymph node dissection. This decreases both the chances of local recurrence and metastatic lymphatic spread. We report a case of 42-year-old female with ulcerative lesion over scalp since 6 months proved as SGC on histopathology with local spread to cervical lymph nodes without any bony infiltration.
| Case Report | | |
A 42-year-old female presented in skin out-patient department (OPD) with history of (h/o) of pus filled lesions over scalp which ruptured leading to ulcer formation since almost 6 months. No h/o cough, cold, fever, vomiting, headache or hematuria present. No h/o weight loss, any GIT symptoms, breathlessness, chest pain or eye symptoms present. No H/o DM/HT/BT/TB/epilepsy/asthma/IHD/COPD/thyroid disease.
On examination single 4 × 5 cm non-tender ulcer with irregular and unhealthy margin, serous and foul-smelling discharge and slough, present over the occipital region of scalp [Figure 1]. Bilateral cervical lymph nodes enlarged and ruptured forming a well-defined round to oval ulcer of 2-3 cm in size with purulent discharge, rolled margin on left side and pus discharge with eschar formation on right side [Figure 2]. | Figure 1: Ulcer with irregular and unhealthy margin, serous discharge and slough over the occipital region of scalp
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 | Figure 2: Well-defi ned round to oval ulcer (a) with purulent discharge on left side (b) eschar formation on right side
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Routine investigations, liver function test (LFT) and renal function test (RFT) were in normal range. Chest X-ray/ECG/USG abdomen and computed tomography (CT) brain was normal. Patient was seronegative with normal venereal disease research laboratory (VDRL). Wedge shaped biopsy done from the ulcer over scalp by surgeon, showed epidermal ulceration, malignant epithelial cells arranged in cribriform pattern, nest, cords and in strands. The cells are pleomorphic with round to oval nuclei and dispersed chromatin, have eosinophilic and indistinct cytoplasm with nuclear and nucleolar pleomorphism. Few cells have foamy vacuolated cytoplasm, zone of necrosis and abnormal mitotic figures. Intraductal necrosis was also noted [Figure 3]. Changes were suggestive of adenocarcinoma of sweat gland over scalp. Wide local excision of ulcer was done with rotational flap and regional lymph node dissection. | Figure 3: Epidermal ulceration, malignant epithelial cells arranged in cribriform pattern, nest, cords and in strands, H and E, (a) ×4, (b) ×10 (c) Tumor island with central necrosis in strands. Pleomorphic cells with round to oval nuclei, dispersed chromatin, eosinophilic and indistinct cytoplasm with nulear and nucleolar pleomorhism. (H and E, ×40)
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| Discussion | | |
SGC represents a rare group of tumors with potential for destructive local tissue infiltration and regional as well as distant metastasis. The sweat gland neoplasms comprised 0.05% of all surgical pathological specimens, [5] and only 12% of these tumors are carcinomas. It is found frequently in the elderly [3],[4],[6],[7] with an average age of about 53 year. The incidence is slightly higher in females. [8]
SGC are very rare malignant tumors that were first described by Cornil in 1865. [1] They have been reported to occur at various sites, including eyelids, scalp, foot digits, breast, axilla, and nose. Head and neck region lesions were found to be 33.6%. [8] The majority of the lesions start as painless red or violet papules slowly growing and progressing to form solid nodules infiltrating subcutaneous tissue. Ulceration of skin is uncommon, occurring mainly in recurrent or large lesions at the back or gluteal region. Increase in size, ulceration and bleeding following minor trauma prompts the patients to seek medical advice. The molecular pathogenesis is poorly understood. A low incidence of loss of heterozygosity at chromosome 17p has been noticed along with p53 alterations. These tumors are more aggressive than squamous or basal cell carcinoma. [9] SGC are broadly divided into: [8]
- Tumors bearing resemblance to their benign counterparts
- Those without or minimal resemblance to their benign counterparts are further classified in to:
- Eccrine adenocarcinomas
- Mucinous eccrine carcinomas
- Adenoid cystic eccrine carcinomas
- Aggressive digital papillary adenoma/carcinoma.
Sweat gland adenocarcinoma belongs to the latter group and is defined as a tumor without any pathological characteristics. Pathogenesis of this neoplasm is not precisely understood, mainly due to the limited number of case reports. It usually appears as a moderate to poorly differentiated adenocarcinoma with regional variation, ranging from true ductule formation to an infiltrative anaplastic cellular zone.
The histological features of malignancy are the same as most epithelial tumors, i.e. increased cellularity, enlarged nuclei, cellular pleomorphism and presence of bizarre mitotic figures. Distinction between metastatic adenocarcinoma and primary SGC can be very difficult at times. Identification of adenocarcinoma arising from eccrine glands is difficult unless lesion has invasive characters. Tumors like sweat gland adenocarcinoma invading bone that are extremely rare has been reported. [10] Tumors show an asymmetry with cords and lobules of polygonal cells, limited to epidermis or extend into dermis. There will be cystic lumina within tumor nests, nuclear atypia with frequent mitosis and necrosis. Malignant cells have large hyperchromatic, irregularly shaped nuclei and may be multi-nucleated and are rich in glycogen. [11]
The recommended treatment of all subtypes of sweat gland carcinomas is wide surgical excision along with regional lymph node dissection in clinically positive nodes. SGCs are regarded as resistant to radiotherapy. Chemotherapy has been very infrequently employed. [12]
Prognostic factors for SGC are difficult to identify, again owing to the very small number of reported cases, but includes size, histological type, lymph node involvement, and distant metastasis. [13]
Our patient was a middle-aged female, presented with ulcer over scalp which clinically caused confusion, as squamous or basal cell carcinoma; but histopathology eliminated it to be adenocarinoma of sweat gland, which has spread to regional lymph nodes but not yet infiltrated the bone, with no metastasis elsewhere was present. Patient was referred to surgeon for further management.
Adenoid SGC were reported over abdomen, chest, axilla vulva and forearm [11] but not over scalp. A case of sweat gland tumor over arm is reported with lung metastasis. [9] Two cases of metastatic sweat gland adenocarcinoma one of eccrine and the other one of apocrine origin) have been reported. [13]
| Conclusion | | |
SGC can be considered as clinico-pathological dilemmas with an unpredictable biological behavior, rarely diagnosed clinically.
| References | | |
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| 11. | Urso C, Bondi R, Paglierani M, Salvadori A, Anichini C, Giannini A. Carcinomas of sweat glands: Report of 60 cases. Arch Pathol Lab Med 2001;125:498-505.
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| 12. | Voutsadakis IA, Bruckner HW. Eccrine sweat gland carcinoma: A case report and re-view of diagnosis and treatment. Conn Med 2000;64:263-6.
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| 13. | Chintamani, Sharma R, Badran R, Singhal V, Saxena S, Bansal A. Metastatic sweat gland adenocarcinoma: A clinico-pathological dilemma. World J Surg Oncol 2003;1:13.
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[Figure 1], [Figure 2], [Figure 3]
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