Malignant solitary cylindroma of the scalp

Eur J Plast Surg (2004) 27:246–248 DOI 10.1007/s00238-004-0650-x

C A S E R E P O R T

M. Hosnuter · O. Babuccu · E. Kargi · G. Numanoglu ·

R. Koca · B. Babuccu

Malignant solitary cylindroma of the scalp

Received: 3 November 2003 / Accepted: 6 May 2004 / Published online: 27 August 2004
Springer-Verlag 2004

Abstract

Cylindroma is a rare tumor which originates

from skin appendages and occurs predominantly on the

scalp and face. It is considered to be benign, but

malig-nant transformation has been reported. It has two distinct

clinical presentations, solitary and multiple. A review

of the literature revealed that the majority of malignant

cylindromas occur among multiple type cylindromas.

Only 36 malignant cylindromas have been reported, and

only 9 were of the solitary type. A case with a solitary

type of cylindroma exhibiting malignant transformation

of the scalp is presented.

Keywords

Cylindroma · Scalp · Solitary · Malignant

transformation

Introduction

The cylindroma was first described by Ancell [1] in 1842

and was observed frequently in the scalp and abdomen

[2]. In 1929 Wiedemann first described a malignant

cy-lindroma [5]. Cycy-lindroma is a benign tumor occurring

predominantly on the scalp. Their appearance is of

smooth pink and skin colored nodules and papules

rang-ing in size up to several centimeters; 90% occur on the

scalp and face. Growth usually begins during adult life

and they grow slowly over the years [4, 10, 15].

Cylin-droma is considered as a benign lesion, but malignant

transformation has been reported [9, 11]. Malignant

transformation of cylindroma is very rare, and this usually

originates from multiple cylindromas. The extensive

lit-erature review reveals overall only 36 cases with

malig-nant cylindroma. Only nine of these cases had a solitary

malignant cylindroma [6, 7, 14].

The special feature in this report that our case had a

solitary lesion 3225 mm in size on the scalp, and its

histological features were malignant.

Case report

A 73-year-old woman presented with a 15-year history of a firm, red to purple colored, nodular lesion on the scalp; this lesion had been necrotic and ulcerated for 1 year. She had a suspicious lesion on another part of the scalp, but she had no complaints related to this lesion and reported having had it for 15 years. On physical examination the noduloulcerative lesion was on the right parieto-occipital part of the scalp and measured 322510 mm (Fig. 1). The other lesion was on the left parietal part of the scalp and measured in 201510 mm, this was mobile and had cystic fea-tures. There were no lymph nodes in the neck or in other region regions. Her physical examination was normal. Radiographic ex-amination of the skull was negative; magnetic resonance imaging of the brain revealed no intracranial pathology.

Surgical excision was performed under general anesthesia, and the lesion was removed with 1 cm intact skin margins. The other lesion was removed with adequate margins. Immediate frozen sections suggested that the first lesion was a cylindroma containing malignant cells, and that other lesion was benign. Because of this report we removed the periosteum under the noduloulcerative le-sion, and the defect was covered with a rotation flap and the skin graft. Pathological examination was by light microscopy using hematoxylin and eosin stain and Alcian blue/periodic acid–Schiff histochemical stain. The latter revealed the hyaline sheets to be surrounding the tumor islands. Surgical margins were intact, and there was no aponeurotic invasion. The other specimen was stained with hematoxylin and eosin, and on microscopic examination it was a benign trichilemmal keratinous cyst.

At the end of 17months postoperatively there were no local or distant metastases, and all laboratory studies, chest radiographs, scintigraphic examination of bones, and cranial and thorax com-puted tomography scans revealed normal findings.

M. Hosnuter (

)

Zonguldak Karaelmas University, Zonguldak, Turkey

e-mail: hosnuter@yahoo.com Tel.: +90-372-2610169 ext 1574 Fax: +90-372-2610155

G. Numanoglu

Department of Pathology, Faculty of Medicine, Zonguldak Karaelmas University,

Zonguldak, Turkey R. Koca

Department of Dermatology,Faculty of Medicine, Zonguldak Karaelmas University,

Discussion

Cutaneous cylindroma is a rare tumor which originates

from the skin appendages. Typically the macroscopic

features of this tumor are nodular, smooth pink and skin

colored; it may be multiple or solitary and ranges in size

from a few millimeters to centimeters and may progress

up to 2150 g. The lesions are frequently painless, but

some patients complain of pain (35%) [4, 11, 17]. The

ratio of women to men is between 2:1 and 3:1. All

re-ported cases have been in whites. The lesions usually first

appear in second decade of life, and growth is gradual [6,

8, 18].

Cylindroma has two distinct clinical presentations. The

solitary cylindroma occurs characteristically on the scalp;

there is absence of familial history, and its frequency is

74%. The multiple type exhibits papules and nodules

which may cover the entire scalp resembling a turban.

Inheritance is autosomal dominant and may be associated

with multiple types of cutaneous tumors [4, 10, 18].

Cylindromas are also a component of the Brooke-Spiegler

tumor; this is a dominantly inherited form associated with

multiple facial trichoepitheliomas [3, 9, 13]. Malignant

transformation of cutaneous cylindromas is very rare and

is usually seen in multiple types [10, 11, 12].

Clinical-ly malignant change is suggested by ulceration, rapid

growth, bleeding, and blue to pink coloration (Fig. 2).

There are only 36 reports of malignant cylindroma, and

only 9 developed from the solitary type [5, 7, 8, 14].

Malignant solitary cylindroma has been reported by

nu-merous authors [2, 6, 7, 10,17]. The histological features

of malignant changes are loss of hyaline sheets,

promi-nent large cells, nuclear anaplasia and pleomorphism,

stromal invasion, loss of jigsaw puzzle arrangement, focal

areas of necrosis, squamous cell metaplasia, and loss of

pallisading at the tumor island periphery [8, 11].

Ag-gressive behavior is associated with loss of hyaline sheets

and loss of pallisading at the periphery of the cell islands

[17]. Malignant tumors can spread along draining

lym-phatic vessels to the related viscera [8]. There may be

metastases to regional lymph nodes, stomach, thyroid,

liver, lung, and bones [5]. Transcranial erosion and

in-tracranial invasion by cylindromas has also been reported

[18].

Indications for surgery are ulceration, infection,

bleed-ing, and cosmesis. The reported treatment modalities are

simple excision, scalping, Mohs’ micrographic surgery,

laser ablation. Simple surgical excision and

reconstruc-tion with local flaps and grafts suffices for the treatment

Fig. 1 The lesion with an ulcerative appearance on the scalp. The tumor masses in front of the malignant tumor were diagnosed epidermal cysts

Fig. 2 Diagnostic findings in specimens were considered as ma-lignant cylindroma. a The specimen consisted of tumor island surrounded by two types of cells. Most of these cells were dark staining nuclei and, less commonly, clear large nuclei and cyto-plasm in palisade arrangement. Two layered epithelial island with a central necrosis. Hematoxylin and eosin stain, original magnifica-tion 100. b There was a certain amount of pleomorphism of the nuclei and most of them characterized by atypically mitotic fea-tures. In the center of the tumor islands were seen with necrosis, i.e., two mitosis in every 10 high-power fields. Hematoxylin and eosin stain, original magnification 400

of benign cylindromas [6]. Very large tumors can be

excised by scalping. The defects are allowed to heal by

second intention or can be covered by split thickness skin

grafts or and free flaps [11, 15, 18]. Laser ablation is

another treatment approach [13]. Radiation has been used

in the past to treat benign cylindromas [8].

In conclusion, a solitary malignant cylindroma is rare,

as was its size of 3225 mm. There were no other

asso-ciated lesions. At 17 months’ follow-up there was no

evidence of recurrence.

References

1. Ancell H (1842) History of a remarkable case of tumors, de-veloped on the head and face, accompanied with similar disease on the abdomen. Med Chir Trans 25:227

2. Bondeson L (1979) Malignant dermal eccrine cylindroma. Acta Derm Venereol 59:92–94

3. Burrows NP, Jones RR, Smith NP (1992) The clinicopatho-logical features of familial cylindromas and trichoepitheliomas (Brooke-Spiegler syndrome): a report two families. Clin Exp Dermatol 17:332

4. Crain RC, Helwig EB (1961) Dermal cylindroma (dermal ec-crine cylindroma). Am J Pathol 35:504

5. Durani BK, Kurzen H, Jaeckel A, Kuner N, Naeher H, Hart-schuh W (2001) Malignant transformation of multiple dermal cylindromas. Br J Dermatol 145:653

6. Freedman MA, Woods JE (1989) Total scalp excision and auricular resurfacing for dermal cylindroma (Turban tumor). Ann Plast Surg 22:50

7. Gerretsen AL, Putte SCJ, Deenstra W, Vloten WA (1993) Cutaneous cylindroma with malignant transformation. Cancer 72:1618

8. Hammond DC, Grant KF, Simpson WD (1990) Malignant de-generation of dermal cylindroma. Ann Plast Surg 24:176 9. Iyer PV, Leong ASY (1989) Malignant dermal cylindromas. Do

they exist? A morphological and immunohistochemical study and review of the literature. Pathology 21:269

10. Lin PY, Fatteh MS, Lloyd MK (1987) Malignant transforma-tion in a solitary dermal cylindroma. Arch Pathol Lab Med 111:765

11. Lo JS, Peschen M, Snow SN, Oriba HA, Mohs FE (1991) Malignant cylindroma of the scalp. J Dermatol Surg Oncol 17:897

12. Lotem M, Trattner A, Kahanovich S, Rotem A, Sandbank M (1992) Multiple dermal cylindroma undergoing a malignant transformation. Int J Dermatol 31:642

13. Martins C, Bartolo E (2000) Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser. Dermatol Surg 26:877

14. Pizinger K, Michal M (2000) Malignant cylindroma in Brooke-Spiegler syndrome. Dermatology 201:255

15. Spauwen PHM, Molenaar WM, Dhar BK (1987) Cylindroma of the scalp. Neth J Surg 39:101

16. Tosun Z, Hosnuter M, Senturk S, Avunduk MC, Savaci N (2002) Cylindroma of the foot. Ann Plast Surg 48:220 17. Urbanski SJ, From L, Abramowiez A, Joaquin A, Luk SC

(1985) Metamorphosis of dermal cylindroma: possible relation to malignant transformation. J Am Acad Dermatol 12:188 18. Wyld L, Bullen S, Browning FSC (1996) Transcranial erosion

of a benign dermal cylindroma. Ann Plast Surg 36:194 248