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ANNPLA-1106; No. of Pages 4 Annales de chirurgie plastique esthétique (2014) xxx, xxx—xxx
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CLINICAL CASE
Zosteriform configuration of multiple eccrine spiradenomas of the scalp ´ nomes eccrines multiples du scalp en configuration Spirade ´ rienne zoste T. Sorin a,*, J.-M. Vignaud b, T. Colson a, G. Gauchotte b, A. De Runz a, M. Brix a, J.-F. Cuny c, E. Simon a a
Department of Maxillofacial, Plastic Reconstructive and Aesthetic Surgery, Nancy University Hospital, ´ chal-de-Lattre-de-Tassigny, 54000 Nancy, France 29, avenue du Mare b ´ chal-de-Lattre-deDepartment of Anatomical Pathology, Nancy University Hospital, 29, avenue du Mare Tassigny, 54000 Nancy, France c ` s-Nancy, France Department of Dermatology, Nancy university Hospital, rue du Morvan, 54511 Vandoeuvre-le Received 13 August 2014; accepted 1 December 2014
KEYWORDS Forehead; Eccrine glands; Sweat gland neoplasms; Cell transformation; Trigeminal nerve
Summary Background. — Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area. Case report. — We describe an original case report of a 75-year-old Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. All the nodules appeared gradually on a one-year period. Biopsy revealed a nodular adnexal tumor in the dermis without malignant eccrine spiradenoma (MES) transformation. The surgical procedure was performed in a manner to protect the galea aponeurotica in the upper half on the first left trigeminal area. The frontalis muscle was raised with the surgical specimen in the lower half of the first trigeminal area. A split-thickness skin graft was applied on the surgical defect. Histological examination revealed multilobular well-defined tumors located in the dermis. Conclusion. — The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis is focused on clinical findings and the treatment is based on a large surgical excision. The histological examination is essential for not to fail a MES transformation. # 2014 Elsevier Masson SAS. All rights reserved.
* Corresponding author. E-mail address:
[email protected] (T. Sorin). http://dx.doi.org/10.1016/j.anplas.2014.12.001 0294-1260/# 2014 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Sorin T, et al. Zosteriform configuration of multiple eccrine spiradenomas of the scalp. Ann Chir Plast Esthet (2014), http://dx.doi.org/10.1016/j.anplas.2014.12.001
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MOTS CLÉS Front ; Glande eccrine ; Tumeur des glandes sudoripares ; Transformation cellulaire ; Nerf trigéminal
Re ´sume ´ Introduction. — Le spiradénome eccrine (SE) est une tumeur annexielle bénigne principalement localisée dans les régions cervico-céphaliques. Des SE multiples du scalp ont été rapportés mais jamais avec une configuration zostérienne sur la première aire trigéminale. Cas clinique. — Nous décrivons le cas d’un homme caucasien de 75 ans présentant des nodules sous-cutanés bleus et violets disséminés sur la première aire trigéminale gauche. Tous les nodules sont apparus en une année. La biopsie a révélé une tumeur annexielle nodulaire dermique sans transformation en spiradénocarcinome. L’exérèse chirurgicale a été effectuée en protégeant la galéa aponévrotique sur la moitié supérieure de la première aire trigéminale gauche. Le muscle frontal a été soulevé avec la pièce opératoire dans la moitié inférieure de la première aire trigéminale. Une greffe de peau mince a été appliquée sur le défait chirurgical. L’examen histologique a révélé des tumeurs multilobulaires dermiques bien définies. Conclusion. — La présence de multiples nodules sous-cutanés sur une aire trijuminale doit faire suggérer un SE multiple localisé. Le diagnostic est porté sur les données cliniques, et est facilité par un scanner. Le traitement est basé sur une excision chirurgicale avec de larges marges. L’examen histologique est indispensable pour ne pas manquer une transformation en spiradénocarcinome. # 2014 Elsevier Masson SAS. Tous droits réservés.
Introduction Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Isolated forms are frequently described but multiple tumors are rare with different presentations such as dermatomal, zosteriform or nevoid. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area.
Case report We describe an original case report of a 75-year-old healthy Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome, spreading to the glabellar area. All the nodules appeared gradually on a one-year period without traumatic or spontaneous bleeding (Fig. 1). At the examination, we noticed 23 nodules, ranged from 1 to 4 cm, covered with multiples telangiectasies. The palpation was pain-free associated with a spongy consistency. General clinical examination was normal as well as the corneal sensitivity. No lymphadenopathy of the head and neck was appreciated. There was no evidence of cutaneous tumors in patient’s family history. Computed tomography (CT) showed the multiple nodules with calcified areas and an heterogenous density (hypodense central area without enhancement). The regional imaging indicated no bone alteration or subcutaneous invasion (Fig. 1). Biopsy revealed a nodular adnexal tumor in the dermis and subcutaneous tissue with basaloid cells and without malignant eccrine spiradenoma (MES) transformation. The surgical excision was performed after an injection of peripheral vasoconstrictive solution and with at least 5 mm margins around the tumors. The dissection was divided in two parts: the cleavage was performed above the galea aponeurotica in the upper half on
the first left trigeminal area in order to maintain a good vascularization for the reconstruction. In the lower half of the first trigeminal area, the frontalis muscle was taken with the surgical specimen, respecting the periosteum because of the multiple adherences between the muscle and the tumors. The eyelid and the eyebrow muscles (procerus m., corrugator supercilii m., and orbital portion of the orbicularis oculi m.) were respected. The clinical examination of the surgical specimen highlighted greyish nodules with a gelatinous constistency. There was no significant bleeding. A split-thickness skin graft (STG) was applied on the surgical defect. The palpebral aesthetic subunit was grafted separately (Fig. 1). Histologic examination revealed a dermal multinodular epithelial tumor. The large, sharply delineated basophilic nodules, unattached to the epidermis were made by aggregates of cells in sheets and cords with a trabecular arrangement. There were small basaloid cells admixed with some larger cells with a pale nucleus, devoid of nuclear atypia or significant mitotic activity (Fig. 2). The strand of cells was cytokeratin positive (pan cytokeratin AE1—AE3 and CK7) and also expressed S-100 protein. The surgical course was uneventful after six months. The skin graft was fully integrated and the motricity of the upper eyelid was intact (Fig. 3).
Discussion ES are rare adnexal tumors especially as multiples localizations (Only 3% of the tumors are multiple) [1]. These forms are gathered together as 3 different patterns: linear, zosteriform and nevoid [2]. The mean age of the patients cases is 29.8 years. Solitary-ES demonstrate a male preponderance with a 2/1 male to female ratio whereas multiple-ES show a female preponderance with a 3/1 female to male ratio [2]. Usually, ES are centimetric painful nodules on the upper half of the trunk and of the head. The tumor growth is considered as extremely slow over a several years period [3,4].
Please cite this article in press as: Sorin T, et al. Zosteriform configuration of multiple eccrine spiradenomas of the scalp. Ann Chir Plast Esthet (2014), http://dx.doi.org/10.1016/j.anplas.2014.12.001
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Figure 1 Above and right, below and left: multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. Above and left: computed tomography (CT): nodules with calcified areas and heterogenous density (hypodense central area without enhancement because the vascular acquisition seemed to be too precocious). Below and right: the surgical procedure was divided in two parts: the cleavage was performed above the galea aponeurotica in the upper half on the first left trigeminal area. In the lower half of the first trigeminal area, the frontalis muscle was removed with the surgical specimen, respecting the periosteum because of the multiple adherences between the muscle and the tumors. The eyelid and the eyebrow muscles (procerus m., corrugator supercilii m., and orbital portion of the orbicularis oculi m.) were respected. A splitthickness skin graft was applied on the surgical defect for the reconstructive procedure. The palpebral aesthetic subunit was grafted separately.
ES associated with cylindroma and trichoepithelioma may raise the possibility of Brooke-Spiegler syndrome [5]. The autosomal dominant familial forms of ES is described usually on a zosteriform pattern’s expression [6]. Yoshida and al. suggest a classification based on ES’s clinical finding (Solitary, multiple localized or multiple multifocal ESs) and histological analyses (Benign or malignant forms, common, vascular and cystic ESs) [2]. Immunohistochemically, tumor cells expressed S-100 protein, pancytokeratin and CK7 [7]. In our study, we found two cell types: small basaloid cells admixed with some larger cells with a pale nucleus. Previous studies mention another cell type: smaller with hyperchromatic nucleus and scanty cytoplasm [6—9]. Contrast-enhanced CT shows irregular enhanced areas at the center of the tumors due to the presence of dilated
vessels [2]. These areas are partially enhanced and heterogeneous unlike hemangiomas. No contrast enhancement was seen in the patient CT, because the images of the vascular acquisition seemed to be too precocious. Malignant eccrine spiradenomas (MES) can develop from an ES with a latency period before cancerous transformation. This can usually range from 20 to 30 years [8]. De novo MES are also described [3]. MES appearances can be clinically identified as a rapid growth associated with ulcerations and bleeding. Lymphatic spread is the first metastatic site followed by the lung, liver, brain and bones [4]. MES without metastasis need an aggressive surgical procedure (estimated survival rate of 100% with a mean followup of 33 months) and a sentinel lymph node biopsy may be useful in the absence of clinical relevances [4]. While wide local excision with a 1 cm margin and depth down to the
Please cite this article in press as: Sorin T, et al. Zosteriform configuration of multiple eccrine spiradenomas of the scalp. Ann Chir Plast Esthet (2014), http://dx.doi.org/10.1016/j.anplas.2014.12.001
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Figure 3 Six months after surgery, the skin graft was fully integrated and the motricity of the upper eyelid remained intact.
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
References Figure 2 Above: hematoxylin and eosin: 4 magnification. The tumor is composed of several large, sharply delineated basophilic nodules, unattached to the epidermis and sometimes extending into the subcutis. Tumor lobules has a thin fibrous capsule. Below: hematoxylin and eosin: 40 magnification. The tumor nodules are composed by aggregates of cells and cords with a trabecular arrengement. There are small basaloid cells admixed with some larger cells.
fascia is described like for squamous cell carcinomas surgical treatment [10,11]. ES surgical approach is the best treatment to avoid recurrences. As a surgical alternative, CO2 laser ablation can be proposed for small and discrete lesions [6]. After tumor excision, we prefer the STG reconstruction technique in order to perform the clinical surveillance. It enables an accurate and early screening of an eventual recurrence.
Conclusions The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis focused on clinical findings, is facilitated by a contrast-enhanced CT. The treatment is based on a surgical excision with broad margins. The histological examination is essential for not to fail a MES transformation.
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Please cite this article in press as: Sorin T, et al. Zosteriform configuration of multiple eccrine spiradenomas of the scalp. Ann Chir Plast Esthet (2014), http://dx.doi.org/10.1016/j.anplas.2014.12.001