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      Large Dermal Non Neural Granular Cell Tumor on the Surgical Wound Site

      case-report

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          Abstract

          Granular cell tumors (GCTs) can be divided into neural type with S-100 reactivity and non-neural type without that. The latter has not been widely recognized and there are only fewer reports available when compared to conventional GCT. A 65-year-old man was presented with the presence of a painless mass on his back. The mass had developed into a small nodule on the scar developed because of previous surgery carried out 2 years ago. The tumor consisted of large, polygonal cells comprising of an enormous number of faintly eosinophilic small granules in the cytoplasm. The cytoplasmic granules were stained positively for periodic acid-Schiff stain. Immunohistochemical stains for S-100 protein and neuron-specific enolase were found to be negative. Herein, we report the appearance of a very rare case of non neural GCT developed on the surgical scar in support with relevant literature reviews.

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          Primitive nonneural granular cell tumors of skin: clinicopathologic analysis of 13 cases.

          A rare subset of distinctive cutaneous nonneural granular cell tumors was described by LeBoit et al in 1991 and termed "primitive polypoid granular-cell tumor." Herein, we report our experience with 13 similar, distinctive nonneural granular cell tumors. Affected patients included 7 males and 6 females ranging in age from 5 to 83 years (mean, 25 years; median, 16 years). These cutaneous lesions involved the back (5 cases), neck, shoulder, thigh (2 cases each), chin, and elbow (1 case each). Clinically described as smooth, nontender cutaneous nodules, the tumors ranged in size from 0.2 to 2.8 cm (median, 0.8 cm) and were present from months to years before excision. Mitoses numbered from 1 to 6 per mm (median, 2). Eight of the lesions were polypoid, based in the papillary dermis with extension to the superficial dermis and associated with an epithelial collarette. Five of the lesions were situated deeper in the reticular dermis with limited extension into the subcutis but clinically were also nodular. All the tumors were well circumscribed and composed of spindled to ovoid cells with abundant granular, eosinophilic cytoplasm and vesicular nuclei with small prominent nucleoli. Immunohistochemistry revealed reactivity only for NKI-C3 (11 of 12 cases), CD68 (7 of 11 cases), and NSE (5 of 10 cases); S-100 protein as well as other melanocytic, epithelial, and myoid markers were uniformly negative. All 13 of the lesions were locally excised and in the 8 cases with adequate follow-up ranging from 13 to 126 months (mean, 68 months; median, 41 months), none has recurred locally. However, one tumor (case no. 11) gave rise to a local lymph node metastasis 25 months after presentation, but the patient is currently disease-free 70 months after lymphadenectomy. These cutaneous granular tumors do not appear to be neural or Schwannian in nature, but their precise line of differentiation is unknown.
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            Primitive polypoid granular-cell tumor and other cutaneous granular-cell neoplasms of apparent nonneural origin.

            Most cutaneous and noncutaneous granular-cell tumors are currently thought to be of Schwann-cell derivation. We present seven unusual cutaneous granular-cell lesions in which Schwann-cell origin can be excluded or is inapparent. Four of these lesions are of a previously undescribed type, and, unlike conventional granular-cell tumors of the skin, show a polypoid configuration, numerous mitoses, cytologic atypia, and a primitive immunophenotype. We propose the term "primitive polypoid granular-cell tumor" for these lesions. One occurred in a child, and three in adults. There have been no metastases to date, with follow-up periods of 2, 4, 4, and 16 years, respectively, although one tumor recurred locally. Additional cases and longer follow-up may be required to rule out the possibility that primitive polypoid granular-cell tumor is a low-grade malignancy. Two other granular-cell lesions represent variants of leiomyosarcoma, one of which widely metastasized. The last case is a granular-cell form of nodular basal-cell carcinoma. Cutaneous granular-cell neoplasms can show varying differentiation and behavior. Pathologists should not equate the occurrence of cytoplasmic granularity in a cutaneous neoplasm with the diagnosis of granular-cell schwannoma.
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              Dermal non-neural granular cell tumour (so-called primitive polypoid granular cell tumour): a distinctive entity further delineated in a clinicopathological study of 11 cases.

              Cutaneous and soft tissue granular cell tumour is a well-characterized benign neoplasm of neural origin. However, there remains a subcategory of granular cell tumour, first described by Le Boit as 'primitive polypoid granular cell tumour', that shows no obvious line of differentiation. The aim of this study is to further the characterization of this lesion by undertaking a clinicopathological review. Eleven cases of dermal non-neural granular cell tumour were retrieved from one of the authors referral archives (E.C.) and both the histology and immunohistochemistry reviewed. Clinical data with follow-up were obtained from the referring pathologists. The lesions most commonly occurred in young to middle-aged adults (nine cases, median = 33 years, age range 6-56 years), with a slight female predominance. They presented as painless nodules, mainly on the extremities or face. Local excision was the treatment of choice and up to date follow-up reveals no sign of recurrence. Histologically, eight cases were polypoid, while three cases were endophytic. The tumours were composed of elongated spindle-shaped to polygonal or round cells with prominent granular cell change, and tumour nuclei showing mild focal atypia to rare moderate atypia. Mitotic activity ranged from one to nine mitoses per 10 high-power fields (median = 2, mean = 3.8). Immunohistochemical labelling of the tumour cells demonstrated expression for NKI-C3 (n = 11), focal, weak positivity for CD68 (n = 10) and FXIIIa (n = 2). There was negative staining for S100 protein, smooth muscle actin, Melan-A, CD34, desmin and cytokeratin. This analysis of 11 cases contributes to the characterization of this recently described entity, which despite some atypical histological features and no obvious line of differentiation, behaves in a completely indolent fashion.
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                Author and article information

                Journal
                Ann Dermatol
                AD
                Annals of Dermatology
                Korean Dermatological Association; The Korean Society for Investigative Dermatology
                1013-9087
                2005-3894
                October 2011
                31 October 2011
                : 23
                : Suppl 2
                : S147-S150
                Affiliations
                Department of Dermatology, College of Medicine, Dankook University, Cheonan, Korea.
                Author notes
                Corresponding author: Byung-Cheol Park, M.D., Department of Dermatology, College of Medicine, Dankook University, San 16-5, Anseo-dong, Cheonan 330-715, Korea. Tel: 82-41-550-6485, Fax: 82-41-552-7541, 4exodus@ 123456hanmail.net
                Article
                10.5021/ad.2011.23.S2.S147
                3229052
                22148037
                078d008e-6c94-4bd8-8def-25b60cf0d5a0
                Copyright © 2011 Korean Dermatological Association; The Korean Society for Investigative Dermatology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 03 August 2010
                : 27 December 2010
                : 27 December 2010
                Categories
                Case Report

                Dermatology
                scar,granular cell tumors,s-100
                Dermatology
                scar, granular cell tumors, s-100

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