|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 1 | Page : 26-28
Granular cell tumor: An uncommon tumor of Schwann cell origin
Anup Kumar Tiwary, Dharmendra Kumar Mishra
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
|Date of Web Publication||14-Jun-2016|
Anup Kumar Tiwary
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Tiwary AK, Mishra DK. Granular cell tumor: An uncommon tumor of Schwann cell origin. Indian J Dermatopathol Diagn Dermatol 2016;3:26-8
|How to cite this URL:|
Tiwary AK, Mishra DK. Granular cell tumor: An uncommon tumor of Schwann cell origin. Indian J Dermatopathol Diagn Dermatol [serial online] 2016 [cited 2020 Oct 28];3:26-8. Available from: https://www.ijdpdd.com/text.asp?2016/3/1/26/184008
Granular cell tumor (GCT) is a rare benign tumor of Schwann cell origin that was first reported by Weber in 1854 and described as a clinical entity termed as “granular cell myoblastoma” by Abrikossoff in 1926. It can occur at any site, but most common locations are oral cavity (most commonly tongue), skin, and subcutaneous tissue. It usually presents as an asymptomatic, solitary, skin-colored, firm or hard nodule in middle-aged persons. Sometimes, the lesions can be multiple and on rare occasions, malignant transformation has also been reported.,, Clinically it can be misdiagnosed as dermal appendageal tumor or soft tissue tumor. Definite diagnosis can only be made by histopathological examination and immunohistochemistry (IHC). Here, we document a case of benign GCT in a 45-year-old woman.
A 45-year-old woman presented with an asymptomatic, solitary, well-circumscribed, hyperpigmented, hard nodule of size 2. 5 cm × 2. 5 cm on the right side of lower abdomen. One year back, it started as an asymptomatic, skin-colored papule which gradually increased in size. There was no history of trauma, infection, or preexisting dermatosis at that site and no significant family history. On cutaneous examination, the lesion was a solitary, well-circumscribed, hyperpigmented, dome-shaped, mobile, nontender hard nodule [Figure 1]. Examination of the mucosae, hair, nails, and teeth was also normal. Regional lymph nodes were not enlarged. General physical, and systemic examinations were normal and all routine laboratory parameters were within normal limits.
|Figure 1: Non-tender, well-circumscribed, hyperpigmented, dome-shaped, hard nodule|
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During excision, tumor was found to be ill-defined and located in deep dermis with yellowish-white cut surface. It was completely excised with wide safe margins and specimen was sent for histopathological examination.
Histopathological evaluation revealed non-encapsulated broad fascicles containing irregular sheets of large, round tumor cells with indistinct borders and pale cytoplasm [Figure 2]. The tumor cells had oval nuclei, and cytoplasm was filled with abundant eosinophilic coarse granules. Within few tumor cells, eosinophilic bodies with a clear halo known as “pustulo-ovoid bodies of Milian” were seen [Figure 3]. Based on these distinct histopathological features, diagnosis of GCT was made.
|Figure 2: Broad fascicles of large, polygonal, tumor cells arranged in nests or sheets infiltrating the dermis and dermal structures (H and E, ×10)|
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|Figure 3: The tumor cells containing round-to-oval nuclei and small, eosinophilic granules uniformly filling the cytoplasm (H and E, ×40) and eosinophilic pustulo-ovoid bodies of Milian (arrow) are surrounded by a clear halo|
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To confirm this diagnosis, IHC staining with S-100 was done which was also positive due to the presence of Schwann cells [Figure 4]. The patient was asked to follow-up biannually to look for any recurrence.
GCT is a rare benign tumor, previously thought to be of muscular origin but now accepted to be originating from Schwann cell due to strong positivity for S-100 protein.,, Apart from oral, cutaneous, and subcutaneous involvement, this tumor may occur at other sites such as breast, scalp, abdominal wall, back, extremities, inguinal region, lymph node, mediastinum, soft palate, orbit, salivary glands, respiratory tract, vulva, and gastrointestinal tract.,,,, In skin, it usually presents as a single, painless, mobile, well-circumscribed, slow-growing, skin-colored, grayish, firm to hard nodule of size between 5 mm and 30 mm, with smooth or hyperkeratotic surface, located in the dermis., It is more common in women in the age group of 20–50 years. GCT has been reported to be associated with certain conditions such as Noonan's syndrome, Watson's syndrome, osteomuscular malformations, oculo-facial alterations, neurofibromatosis, squamous cell carcinoma of esophagus, adenocarcinoma of prostate and small cell lung carcinoma. It was classified by Fanburg-Smith et al. as benign, malignant, or atypical on the basis of six histopathologic criteria, namely:
- Increased mitotic activity(>2 mitoses/10 hpf at ×200 magnification)
- High nucleo-cytoplasmic ratio
- Nuclear polymorphism
- Spindling of tumor cells and
- Vesicular nuclei with prominent nucleoli.
Tumors fulfilling ≥3 criteria were classified as malignant; those having one or two criteria as atypical and cases displaying none of the six criteria were classified as benign GCT. Clinical picture is usually simulated by dermatofibroma, fibroxanthoma, lipoma, keratoacanthoma, neurofibroma and leiomyoma, which needs to be ruled out on histopathologic evaluation.,, On histopathology, GCT is characterized by noncapsulated, irregular sheets of large, round or polygonal cells with indistinct cytological border containing abundantly eosinophilic, granular, pale staining cytoplasm, and small, round or oval nuclei.,,,,, The granularity of cytoplasm is due to massive accumulation of lysosomes which may sometimes be larger and surrounded by a clear halo called 'pustulo-ovoid bodies of milian' as seen in our case.,, Non-specifically, epidermis is hyperkeratotic and may show downward proliferation forming horn pearls due to pseudocarcinomatous hyperplasia.
Other diseases which need to be differentiated by histopathology are rhabdomyoma, alveolar soft tissue sarcoma, paraganglioma, hibernoma, and melanoma. IHC further aids in the correct diagnosis of GCT as granular cells are positive for S-100, neuron specific enolase, vimentin, periodic acid-schiff, inhibin-α, protein gene product 9.5, and CD68 and negative for epithelial membrane antigen, cytokeratin, desmin, myogenin, smooth muscle actin, and Human melanoma black-45(HMB-45).
For management, fine needle aspiration cytology should preferably be undertaken to make pre-operative diagnosis so that wide local excision with a safe margin can be done in order to reduce the risk of recurrence.
Advice for follow-up must be given to the patients to look for any recurrence and malignant transformation. Adjuvant radiotherapy and chemotherapy may be tried in case of recurrent and metastatic forms of GCT, respectively.
The authors sincerely thank Dr.Uday Khopkar for their contribution by giving valuable suggestions for the histopathological diagnosis.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]