Multiple dermal cylindroma, appropriate treatment and literature review

ABSTRACT

Cylindromas are tumors of skin appendages. They present most commonly in the head and neck area. Many modalities have been described to treat them. We present a case of an elderly female who presented to us with recurrent lesions and was offered radical excision of all lesions versus excision of symptomatic lesions. She opted for the removal of symptomatic lesions. Surgical excision of symptomatic lesions is a reasonable alternative in elderly patients.

Keywords:
Second-look surgery; Clinical protocols; Skin neoplasms; Aged

RESUMO

Os cilindromas são tumores de apêndices cutâneos. Apresentamse normalmente na área da cabeça e pescoço. Muitas modalidades foram descritas para seu tratamento. Apresentamos aqui o caso de uma idosa que se apresentou com lesões recorrentes e que recebeu a opção de fazer uma excisão radical de todas as lesões versus a excisão das lesões sintomáticas. Ela optou por remover as lesões sintomáticas. A excisão cirúrgica das lesões sintomáticas é uma alternativa razoável para pacientes idosos.

Descritores:
Cirurgia de Second Look; Protocolos clínicos; Neoplasias cutâneas; Idoso

INTRODUCTION

Multiple dermal cylindromas are rare benign tumors of skin appendages. The exact incidence is not known. About 91% are present in the head and neck region¹. They can be present in salivary glands, bronchus, lungs, extremities, breast, prostate and kidney. They can present as a solitary or multiple lesions. Multiple lesions involving the whole scalp is popularly known as turban tumour, first described by Ancell².

CASE REPORT

We present a case of a 62-year-old female who presented with multiple cylindromas in the head and neck region. She had complaints of pain from the swelling in the posterior part of her head and off and on bleeding from the lesion. Examination revealed multiple pink rubbery hairless tumors in the region of the head and neck (Figures 1 and 2). Wedge biopsy from a lesion was suggestive of cylindroma. Patient was given the option of complete excision of all the lesion with reconstructive procedures and resection of symptomatic lesions and primary closure. She opted for resection of the lesions causing discomfort only.

Figure 1
Before 3^rd resection.

Figure 2
Before 3^rd resection - note skin graft over forehead done in earlier surgery.

After being investigated for anesthetic fitness, she underwent resection of those lesions under general anesthesia. Raw areas were closed primarily. Lesions over forehead were resected followed by application of thick graft over the raw area. Postoperative period was uneventful and patient was discharged on 3^rd postoperative day.

Histopathology was suggestive of Cylindroma. Follow-up after 3 months showed the development of new lesions of which those causing discomfort on the right side of face and in the post auricular area were resected. She again underwent a 3^rd resection of new symptomatic lesions on her occipital region (Figures 3 and 4). None of the lesions showed a malignant transformation.

Figure 3
After 3^rd resection - Vertex view.

Figure 4
After 3^rd resection - skin graft on occipital area.

DISCUSSION

Dermal Cylindromas may occur sporadically or may be inherited in Autosomal Dominant manner on Cyld 1 gene on chromosome 16³. Women are affected more than men with a female to male ratio of 3:1 to 9:1⁴. Immunohistochemical markers show their derivation from apocrine and eccrine glands.

Surgery remains the mainstay of treatment for dermal cylindromas. Solitary tumors can be dealt with simple surgical excision and primary closure. Small lesions may be dealt with carbon dioxide laser³. Multiple lesions may require serial excision or excision of the symptomatic lesions. Extensive lesions like turban tumor may be dealt with excision followed by split thickness skin grafting. Total excision of the scalp has also been done⁵.

Procedures such as dermabrasion and ablation with electrocautery are associated with a high recurrence rate² as they do not result in complete removal of dermal appendages. These tumors are associated with Brooke, syringiomas, spiredinomas. Malignant transformation is rare. They may present with lymph node metastasis. These are treated with neck dissection along with radiotherapy and chemotherapy⁶.

CONCLUSION

Dermal Cylindromas are rare tumors which present in a variety of ways. The awareness of the various treatment options is necessary and helpful in providing satisfactory surgical outcome. Simple serial excision of the symptomatic lesions in elderly patients is a good option with minimal morbidity.

REFERÊNCIAS

• ¹
  Freedman AM, Woods JE. Total scalp excision and auricular resurfacing for dermal cylindroma (turban tumor). Ann Plast Surg. 1989;22(1):50-7. PMID: 2538102 DOI: http://dx.doi.org/10.1097/00000637-198901000-00010
  » https://doi.org/10.1097/00000637-198901000-00010
• ²
  De Backer A, Houte KV, Duinslaeger L, Wylock P. Familial dermal eccrine cylindroma (turban tumor) - A report of two cases with emphasis on the surgical approach. Eur J Plast Surg. 1986;9(3):113-6. DOI: http://dx.doi.org/10.1007/BF00298953
  » https://doi.org/10.1007/BF00298953
• ³
  Gupta R, Jain R, Sood S, Mohan H. Dermal cylindroma presenting as mass lesion in superomedial orbit. Indian J Ophthalmol. 2003;51(3):257-9. PMID: 14601853
• ⁴
  Fredrich RE. Dermal cylindroma of scalp (turban tumor) and subjacent calvarial defect. Anticancer Res. 2010;30(5):1793-7.
• ⁵
  Alix T, Labbé D, Caquant L, Comoz F, Compère JF, Bénateau H. Management of whole-scalp tumour: the Poncet-Spiegler cylindroma or turban tumour. Rev Stomatol Chir Maxillofac. 2009;110(2):109-12. PMID: 19328506 DOI: http://dx.doi.org/10.1016/j.stomax.2009.01.005
  » https://doi.org/10.1016/j.stomax.2009.01.005
• ⁶
  Akgul GG, Yenidogan E, Dinc S, Pak I, Colakoglu MK, Gulcelik MA. Malign cylindroma of the scalp with multiple cervical lymph node metastasis-A case report. Int J Surg Case Rep. 2013;4(7):589-92. DOI: http://dx.doi.org/10.1016/j.ijscr.2013.02.027
  » https://doi.org/10.1016/j.ijscr.2013.02.027

Publication Dates

• Publication in this collection
  29 May 2023
• Date of issue
  Jan-Mar 2017

History

• Received
  09 Sept 2016
• Accepted
  21 Feb 2017