Radiol Oncol 2005; 39(1): 55-9.
Cranium eroding sweat gland carcinoma: a case report
Mehmet Arslan, Ahmet N. Karadeniz, Görkem Aksu, Murat Güveli
Istanbul University, Institute of Oncology, Istanbul, Turkey
Background. Sweat gland carcinomas are rare tumors. Eccrine sweat gland carcinomas are also very rare, with only about 200 cases reported in the world literature and only one of them was eroding the cranium. Treatment modalities of these carcinomas are not well known.
Case report. Our patient was 47 years old female. Since 1989, she was operated on six times because of the tumour relapses. After each operation, the pathological results were: sweat gland adenoma, sweat gland tumour, cylindroma, turban tumour, malign cylindiroma. That was her seventh relapse. On examination, a lesion of the size 10 x 6 cm was observed in the left parietal region. Computed tomography showed the lesion had the size of 11 x 5 cm, and was destroying the tabula externa, diploic region and tabula. The tumour was invading the dura and causing periost reaction. Surgery and postoperative radiotherapy treatment was planned because of malign transformation and risk of recurrence.
Conclusions. Only one case with cranium erosion was reported in literature. In our case, also intracranial extension of the tumor was observed.
Key words: sweat gland neoplasms; parietal bone; neoplasms invasiveness
Introduction
Little is known about these rare sweat gland carcinomas and their treatment modalities. We reviewed the literature on sweat gland carcinomas to elucidate the nature of these tumours and best treatment course. The mean patients' age is 57 years with equal male-to-female distribution. Tumour distribu-Received 24 March 2004 Accepted 14 May 2004
Correspondence to: Prof. Mehmet Arslan, MD, Institute of Oncology, Istanbul University, C,apa, 34390, Istanbul, Turkey; Phone: +90 532 686 4659; Email: marslan19@yahoo.com
tion is: lower extremities, 32.9%; upper ex-tremities, 27.6%; trunk, 11.9%; head, 26.3%; neck, 1.3%. Metastasis sites are: lymph nodes, 30.2%; viscera, 22%. Malignant sweat gland carcinomas have a propensity for local and regional lymph node recurrence.1 The roles of lymph node dissection, radiation, and chemotherapy were reviewed.
Apocrine gland carcinoma is a rare sweat gland neoplasm with distinctive cytological appearance. Although the region of the axilla remains the most common site for these tu-mours, apocrine gland carcinoma of the anogenital region, eyelid, ear, chest, wrist, lip, foot, toe, and finger have been reported. Classically, these slow-growing lesions pres-ent as painless, colourless or reddish, firm or
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cystic nodules. More than half of the reported patients with apocrine carcinoma had lymph node metastases at the time of diagnosis. Wide local excision is standard therapy for these lesions. A therapeutic lymph node dis-section is indicated if lymph node metastases are confirmed. It may be effective in the set-ting of a large or highly aggressive tumour with narrow surgical margins. As apocrine gland carcinoma responds poorly to chemotherapy, adjuvant radiotherapy may be applied in advanced, local, or regional dis-eases.2
Malignant eccrine sweat gland tumours are rare and usually develop from pre-exist-ing eccrine appendage tumour of lesser matu-rity.3-6 Clinical features of these tumours are non-specific, and final diagnosis is always based on histology. The tumours are usually located on the head, neck, or extremities and manifest as slow-growing nodules or infiltrat-ed plaques. These tumours are rare, with on-ly about 200 cases reported in the world liter-ature.7 A review of the literature reveals only 25 published reports of malignancies arising from eccrine spiradenoma. These tumours have a metastasis rate of >50 per cent in re-ported cases with high mortality rates as a re-sult.3 The case that is reported by Ritter et al is the only porocarcinoma eroding the crani-um. A review of the literature failed to reveal any other such case.4
Dermal eccrine cylindroma or turban tu-mour is a rare benign tumours of the eccrine sweat glands. Despite its histological benign behaviour, the disease process is distressing for the patients and cylindromas rarely progress to cylindrocarcinoma.8
Case report
Our case is 47 years old women patient. She first visited the health care centre in 1989 for a solitary, colourless and painless lesion that she had on the scalp for a long time. On ex-Radiol Oncol 2005; 39(1): 55-9.
amination, colourless and painless 2 cm solid scalp lesion was palpated. The lesion was ex-cised and not examined pathologically at that time. For about two years later, the lesion re-lapsed. It was excised and diagnosed patho-logically as sweat gland adenoma. Adjuvant therapy was not needed. For about two years later, the lesion relapsed again. It was excised thirdly and diagnosed pathologically as sweat gland tumour. For about two years later, the lesion relapsed thirdly; this time, it was red-dish. It was excised fourthly and diagnosed pathologically as sweat gland cylindroma. For about one year later, the lesion relapsed mul-tifocally. The largest lesion was 4 cm long, reddish and painful. Other lesions were colourless, painless and solitary. The lesion was excised again and diagnosed as turban tumour. Adjuvant therapy was not needed.
Short time later, the lesion relapsed multi-focally again. Dimensions of the lesions were up to 5 x 6 cm (Figure 1). The excision of the lesions, increasing in number and diameter and changing physically each time, was per-formed again. Pathologically, it was diag-nosed as cylindroma with malign features (Figure 2).
On physical examination of the patient with recurrence referred to our clinic, a mass of the size 10 x 6 cm was found on the left
Figure 1. General appearance of the lesion (5x6cm).
Arslan M et al / Cranium eroding sweat gland carcinoma
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Figure 2. Pathological appearance of the lesion (H&E, X100).
parietal region of the scalp. With cranial di-rect roentgen and computed tomography, a mass, measuring 11 x 5 cm, was destructing tabula, tabula externa, and diploic region, in-vading the dura and causing periost reaction was identified (Figure 3). Surgery and postoperative radiotherapy with surgical consulta-tion was planned because of malign transformation and risk of recurrence.
Discussion
Dermal eccrine cylindroma or turban tumour is a rare benign tumour of the eccrine sweat glands.9,10 Women are affected 4 times more frequently than men. About 10% of all cylin-dromas are hereditary, transmitted autoso-mally dominantly and with variable pene-trance. Papules, nodules, and tumours occur mainly on the scalp, but they may be found on the face and upper part of the trunk. If
nodules of cylindromas cover the entire scalp and are heaped up, they resemble a turban. Despite its histological benign behaviour, the disease process is distressing for the pa-tients.8 Cylindromas rarely progress to cylin-drocarcinoma.11-13 Most of these cases of cylindrocarcinomas have developed from
Figure 3. Radiological appearance of the lesion invad-ing the cranium (CT).
Radiol Oncol 2005; 39(1): 55-9.
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long persisting tumours of cylindroma. Histologically, the tumours are similar to cylindromas, but they are marked by large numbers of mitotic figures and atypical mi-toses. Cylindrocarcinomas are aggressive, with metastases to lymph nodes, bone, and visceral organs.6 In our case, despite its histo-logical benign features, the disease relapsed seven times and increased in dimension and number. Thus, despite its benign pathology, it was distressing for the patient. Some of them become malignant. Last pathological report of our case was cylindroma with malign features; so, it changed to a malign form from a long persisting cylindroma. A review of the literature reveals only 25 published reports of malignancy arising from eccrine spiradeno-ma.
These tumours are rare tumours, with only about 200 cases reported in the world litera-ture.7 The case that was reported by Ritter et al is the only porocarcinoma eroding the cra-nium. A review of the literature failed to re-veal any other such case.4 Our case with cra-nial erosion, dural invasion, and intracranial extension is the first scalp eccrine sweat gland tumour, cylindroma. Multiple resec-tions were required for local control and, fi-nally, the lesion changed to a growth with malign histology.
Wide local excision is standard therapy for these lesions. A therapeutic lymph node dis-section is indicated if lymph node metastases are confirmed, and it may be efficient in the setting of a large or highly aggressive tumour with narrow surgical margins. As sweat gland carcinoma responds poorly to chemotherapy, adjuvant radiotherapy may be used in advanced local or regional diseases.2 The treat-ment plan for our patient comprised wide lo-cal excision and postoperative radiotherapy because of malign transformation and risk of recurrence. The tumour invaded the dura and extended intracranially. Adjuvant treatment such as chemotherapy and craniospinal irradiation may be applied. But these tumours re-Radiol Oncol 2005; 39(1): 55-9.
spond poorly to chemotherapy and there is no experience of craniospinal irradiation.
Conclusions
The reported case with cranial erosion, dural invasion, and intracranial extension is the first scalp eccrine sweat gland tumour, cylin-droma. Cylindromas rarely progress to cylin-drocarcinoma. Most of these cases of cylin-drocarcinomas have developed from long persisting tumours of cylindroma. In our case, it also changed to malign cylindroma. Wide local excision is standard therapy for these lesions. Multiple resections may be re-quired in order to obtain local control even if the lesion is benign. Therapeutic lymph node dissection and adjuvant radiotherapy may be used in advanced local or regional diseases.
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