Non-neural Granular Cell Tumor Research Articles

Pleomorphic Dermal Neoplasms

Pleomorphic neoplasms are typically associated with high-grade malignant behavior, but this does not readily apply to primary cutaneous tumors. Despite morphologic features suggestive of malignancy, atypical fibroxanthoma, the classic example of a pleomorphic dermal neoplasm, is characterized by indolent clinical behavior. Atypical fibroxanthoma is a distinctive clinicopathologic disease affecting sun-damaged skin of elderly males. Histologically, it is often ulcerated and dermal based with pushing growth, characterized by a sheet-like and fascicular growth of pleomorphic epithelioid, spindled, and multinucleated tumor cells with brisk and atypical mitotic activity. However, no positive discriminatory histologic or immunohistochemical features exist. Its diagnosis is one exclusion with a wide differential diagnosis, mainly including other mesenchymal, melanocytic, and epithelial neoplasms. Particular considerations are pleomorphic dermal sarcoma, invasive melanoma, squamous cell carcinoma, metaplastic carcinoma, poorly differentiated cutaneous angiosarcoma, cutaneous leiomyosarcoma, myxofibrosarcoma, variants of fibrous histiocytoma (FH), pleomorphic fibroma, and non-neural granular cell tumor. The behavior of these tumors is varied and ranges from outright malignant to entirely benign, requiring confident diagnosis to reliably predict behavior and guide treatment. Although challenging, because of significant clinical and pathologic overlap, it is usually possible to establish a definitive diagnosis when attention is paid to the often subtle differentiating features. This requires careful tumor sampling, recognition of the subtle distinguishing morphologic features, judicious use and analysis of immunohistochemistry, and interpretation of the findings in the appropriate clinical setting.

Primitive non-neural granular cell tumour of skin: report of a case

<h3>Background</h3> Granular cell tumour of skin is an uncommon benign neoplasm of neural origin. However, a rare histologically similar but S100 protein negative tumour designated by Le Boit <i>et al</i>. in 1991 as ‘primitive non-neural polypoid granular cell tumour' has also been recognised and its cell lineage remains uncertain. <h3>Case report</h3> A 77-year-old male presented with a painless nodule on his right cheek. Histolopathology revealed an ulcerated dermal tumour with nodular architecture formed by plump spindle and epitheloid cells with prominent granular eosinophilic cytoplasm, vesicular nuclei and numerous mitoses. Immunohistochemical staining showed strong positivity for CD10 and NKI/C3, and focal positivity for HMB45 and MITF. Staining for S100, MelanA, tyrosinase, MNF116, p63, panCK, CK5/6, desmin, SMA, SOX10, CD31 and CD34 were negative. A diagnosis of primitive non-neural polypoid granular cell tumour was made. <h3>Discussion</h3> The limited published data suggest primitive non-neural polypoid granular cell tumours usually pursue an indolent course. They are negative for S100 (unlike conventional granular cell tumour and melanoma), cytokeratin, desmin and SMA, indicating no obvious line of differentiation. However, they are consistently positive for NK1/C3, a marker for lysosomes in granular cells. This case highlights the value of immunohistochemistry in distinguishing this tumour from malignant entities.

Clear cell atypical fibroxanthoma: a case report and brief review of the literature

Aim/Background Clear cell tumours of the skin present a diagnostic challenge. We report a case of clear cell atypical fibroxan-thoma (clear cell AFX) and discuss differential diagnosis of clear cell tumours of skin. We also briefly review literature and immuno-histochemical testing pertaining to this group of lesions. Case presentation A 75-year-old male presented with an ulcerated lesion on the forehead/scalp. There was a past history of Gleason 3 + 4 = 7 prostatic acinar adenocarcinoma. The case was reported in consultation with a second expert dermatopathology opinion. Discussion The differential diagnosis of clear cell tumours of skin is broad and includes epidermal proliferations, clear cell adnexal lesions, melanocytic proliferations, mesenchymal lesions and metastases. In this case the lesion was pleomorphic and dermally based. This limited possible diagnoses to balloon cell melanoma, clear cell AFX, clear cell squamous cell carcinoma (SCC), clear cell basal cell carcinoma (BCC) and malignant skin adnexal neoplasms (sebaceous carcinoma, tricholemmal carcinoma and clear cell malignant eccrine neoplasms). Metastases, mesenchymal neoplasms (dermatofibroma with ‘monster’ cells and leiomyosarcoma with clear cell change) and non-neural granular cell tumour of the skin were also considered. Conclusion AFX is a diagnosis of exclusion. This case demonstrates how a comprehensive immunopanel and morphological features are used to eliminate other possibilities.

Granular Cell Variant of Epithelioid Cell Histiocytoma

Classic granular cell tumors (GCTs) stain strongly and uniformly positive for S100 protein and are believed to show Schwann cell derivation. Polypoid cutaneous tumors composed of cells with large nuclei and abundant granular cytoplasm that do not stain for S100 protein or show apparent Schwannian differentiation have been reported by several groups under names including "primitive polypoid granular cell tumors," "dermal nonneural granular cell tumor," and "primitive nonneural granular cell tumors of skin." We report a polypoid tumor composed of S100-negative epithelioid cells with abundant eosinophilic granular cytoplasm that meets diagnostic criteria for (primitive polypoid dermal) nonneural GCT but also meets criteria for a granular cell variant of epithelioid cell histiocytoma. We have identified a single previous report of a similar lesion. We report the immunohistochemical characteristics of these lesions and address how they are best classified.

Primitive polypoid granular cell tumor

ejd.2012.1768 Auteur(s) : Francesco Ricci1 fraric1984@libero.it, Andrea Paradisi1, Sara Lanza Silveri1, Barbara Fossati1, Ilaria Pennacchia2, Guido Massi2, Rodolfo Capizzi1, Cristina Guerriero1 1 Department of Dermatology, 2 Department of Histopathology, Catholic University of Sacred Heart L.go A Gemelli 8 00168 Rome, Italy First described as primitive polypoid granular cell tumor (PPGCT) in 1991 by LeBoit [1], dermal non-neural granular cell tumor is a skin neoplasm of uncertain differentiation. An [...]

An infiltrative variant of non‐neural granular cell tumor: a case report

Dermal non-neural granular cell tumors are rare tumors of indeterminate lineage that typically present as well-circumscribed tumors with nuclear pleomorphism and mitotic activity. We describe a dermal non-neural granular cell tumor with a distinctive growth pattern with granular cells interspersed between collagen bundles. This asymptomatic papule arose on the scapula of a 46-year-old woman and consisted of a mixture of epithelioid and spindled granular cells. The immunohistochemical characteristics were similar to those of previously reported dermal non-neural granular cell tumors. Despite mild nuclear pleomorphism and dispersion of lesional cells among collagen bundles, mitoses were not present and Ki-67 staining indicated a low proliferative rate. In addition to being S-100 protein negative and NKI/C3 positive, our case was positive for PGP9.5 and weakly positive for neuron-specific enolase, a staining pattern similar to what has been observed for cellular neurothekeomas. Our case could represent a dermal non-neural granular cell tumor with unique architecture, a granular cellular neurothekeoma or a granular cell dermatofibroma. As both dermal non-neural granular cell tumor and cellular neurothekeoma are of indeterminate lineage, our case with features characteristic of both entities may suggest a common precursor or lineage for dermal non-neural granular cell tumor and cellular neurothekeoma.

Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?

We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15-year-old girl. Histologically, the lesion was composed of a dermal population of medium- to large-sized, short spindle- to round- to epithelioid-shaped cells with eosinophilic cytoplasm containing numerous and small diastase-resistant periodic acid-Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of 'solitary cutaneous histiocytosis with granular cell changes' was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so-called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially 'granular cell tumor' and 'dermal non-neural granular cell tumor'.

Primitive Nonneural Granular Cell Tumor (So-called Atypical Polypoid Granular Cell Tumor). Report of 2 Cases With Immunohistochemical and Ultrastructural Correlation

Primitive nonneural granular cell tumors (so-called atypical cutaneous granular cell tumors) were first described in 1991, followed by few case reports, and 2 recent larger series. We report here 2 additional cases in 2 women aged 73 and 74, who presented with 0.6- and 0.4-cm skin nodules on the right side of the jaw and the forearm, respectively. Biopsies showed cutaneous granular cell neoplasms with epithelioid morphology. The cells exhibited nuclear pleomorphism and brisk mitotic activity with atypical mitoses. Immunohistochemically, the tumor cells expressed vimentin and PGP 9.5 but lacked S-100 and CD34 expression. Ultrastructurally, both cases showed primitive cells packed with large secondary lysosomes. Primitive nonneural granular cell tumors seem to consist of neoplastic proliferating cells that fail to break down uncharacterized cellular material within the lysosomes. They are, however, different from classic granular cell tumor by lacking neural differentiation. Despite reported worrisome cellular atypia, these rare tumors seem to pursue a favorable outcome.

Granular Cell Atypical Fibroxanthoma: Report of Two Cases

Two cases of an uncommon histopathological variant of atypical fibroxanthoma (AFX) are described. Even though both lesions presented as clinically conventional atypical fibroxanthoma, histopathology disclosed a neoplasm composed of cells with granular change that was negative for S100 staining, and showed prominent pleomorphism, severe nuclear atypia, and a high mitotic index. Degenerative change may explain the granular phenotype in these two cases of AFX. The differential diagnosis with primitive nonneural granular cell tumor is discussed.

Primitive Nonneural Granular Cell Tumors of Skin: Clinicopathologic Analysis of 13 Cases

Primitive Nonneural Granular Cell Tumors of Skin: Clinicopathologic Analysis of 13 Cases

Nonneural granular cell tumor of the oral cavity: a case report and review of the literature

Nonneural granular cell tumors (NNGCTs) are rare benign neoplasms originally described in 1991 by Leboit et al. Typical granular cell tumors (GCTs) are commonly encountered in the oral cavity, but NNGCTs, unlike GCTs, are S-100 negative and may display cytologic atypia, allowing for misdiagnosis as a more aggressive lesion. We report a case of a 43-year-old male with a lesion of the mandible that we believe to be the first intraoral example of an NNGCT.

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.

Primitive Nonneural Granular Cell Tumors of Skin

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.