|Year : 2018 | Volume
| Issue : 5 | Page : 452-454
Very rare childhood tumor: Giant cell fibroblastoma
Aditya Pratap Singh, Arun Kumar Gupta, Maryem Ansari, Sunil Kumar Mehra, Dinesh Kumar Barolia
Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||5-Sep-2018|
Aditya Pratap Singh
Near The Mali Hostel, Main Bali Road, Falna, Dist.Pali, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
Giant cell fibroblastoma (GCF) is an intermediate grade, soft-tissue tumor of childhood with a high incidence of local recurrence. GCF merits attentive interpretation as they have been misdiagnosed as sarcoma in the past leading to erroneous remedies. Here, we are reporting the case of GCF in 13-month-old boy who presented with a painless swelling over the left gluteal region for the past 3 months.
Keywords: Fibroblastoma, giant cell tumor, gluteal region, soft tissue tumor
|How to cite this article:|
Singh AP, Gupta AK, Ansari M, Mehra SK, Barolia DK. Very rare childhood tumor: Giant cell fibroblastoma. Med J DY Patil Vidyapeeth 2018;11:452-4
|How to cite this URL:|
Singh AP, Gupta AK, Ansari M, Mehra SK, Barolia DK. Very rare childhood tumor: Giant cell fibroblastoma. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2020 Nov 30];11:452-4. Available from: https://www.mjdrdypv.org/text.asp?2018/11/5/452/240369
| Introduction|| |
Giant cell fibroblastoma (GCF) is an unusual childhood fibrohistiocytic tumor of intermediate malignancy. It was originally described by Schmookler and Enzinger in 1982, following which around 100 cases have been reported.,, GCF is a rare soft-tissue tumor that occurs almost exclusively in children younger than 10 years of age and exceptionally in adults. The lesions are mostly located in the superficial soft tissues of the back and thighs.
| Case Report|| |
A 13-month-old boy presented with painless swelling at the left gluteal region for the past 3 months. Initially, the swelling was small and gradually increased to the present size of 6 cm × 6 cm. There was no history of trauma, fever, restriction of movements, or any other swelling. However, there was a history of the biopsy in the same region 6 months back. The patient was operated for the provisional diagnosis of the abscess at the periphery, where they found a swelling on the cruciate incision and took biopsy. That time histopathological report showed neurofibroma. Then, the patient referred to us with gradual increase in the size of the swelling and for further management [Figure 1]a. On local examination, a soft, nontender, freely mobile swelling of 6 cm × 6 cm was present at the left gluteal region. The swelling was not fixed to the skin except previous operative site, and there was no sinus or ulcer present over it. Hematological profile and all other investigations were within normal limits. Fine-needle aspiration cytology of the swelling showed acute and chronic inflammatory cells suggestive of the granulation tissue. Magnetic resonance imaging (MRI) gluteal region showed well-defined homogeneous lesion of size approximately 37 mm × 78 mm × 73 mm in the left postero-supero-lateral gluteal region involving cutaneous–subcutaneous regions with maintained fat planes with underlying gluteal muscles without infiltration [Figure 2]. MRI suggested benign mesenchymal tumor and most likely large benign neurofibroma. We planned for the surgical excision. On exploration, it was not adherent to any structures except previous operative site [Figure 1]b and [Figure 1]c. We dissected the swelling all round with blunt and sharp dissection and excised completely.
|Figure 1: (a) Preoperative clinical photograph, (b) peroperative photograph, (c) excised specimen|
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Histopathologically, the section showed GCF [Figure 3]. The patient was regularly followed up and doing fine for the past 1 month.
|Figure 3: Histopathological slide show spindle-shaped cells in collagenous background with multinucleated floret-like giant cells (black arrow)|
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| Discussion|| |
GCF characteristically occurs in males during the first decade of life, with more than half of cases seen before the age of 5 years. This case was also a male child at the age of around 1 year.
Metastasis has never been reported. Clinically, the patient usually presents with a small, painless, slow-growing soft-tissue swelling which has a predilection for the back and thighs. Other less common sites include anterior chest, shoulder, perineum, and extremities., In our case, it was located in the left gluteal region.
On gross examination, GCF appears as an unencapsulated, gray-white, gelatinous lesion. Histologically, these lesions show a variable degree of cellularity with a fibromyxoid to hyalinized stroma containing bland spindle cells and multinucleated giant cells. The spindle cells may demonstrate mild-to-moderate pleomorphism and are arranged diffusely, or in vague fascicles, their elongated nuclei have vesicular to hyperchromatic chromatin. The cytoplasm is usually scanty and eosinophilic. The giant cells contain a variable number of round to oval vesicular nuclei and have abundant amphophilic cytoplasm with irregular cytoplasmic contours. Slit-like sinusoidal spaces lined by either a continuous or discontinuous layer of the multinucleated giant cell is a unique and diagnostic feature of GCF.,
The histogenesis of GCF has been a topic of considerable debate among researchers. Ultrastructural studies have, however, demonstrated the fibroblastic differentiation. Previously, GCF was considered to be a juvenile form of dermatofibrosarcoma protuberans (DFSP), but recent studies suggest that both these lesions are on a spectrum of the same entity as confirmed by clinical, morphological, immunophenotypical, and molecular studies., Both lesions share common clinical features of male predominance, slow growth, painlessness, and anatomical location on mainly the trunk. Morphologically, GCF and DFSP show predominantly dermal or subcutaneous location and rarely superficial skeletal muscle involvement, honeycomb and parallel growth patterns, sparing of adnexa, myxoid changes, and prominent vasculature. By using immunohistochemistry, both lesions show positive staining for CD34 and negative reaction to keratin, S-100, HMB-45, smooth muscle actin, and desmin. Cytogenetically, t (17;22) translocation is seen in GCF and DFSP. Although GCF and DFSP share several clinical as well as morphological features, we can still differentiate them solely on the basis of histological findings. The presence of pseudovascular spaces lined by giant cells, solid areas with stromal giant cells, consistent hemorrhage, perivascular, and onion skin-like chronic inflammation without storiform pattern favors the diagnosis of GCF.
Vimentin is the only immunohistochemical detection marker that has been found to consistently stain GCF. Treatment includes removal of the tumor with wide excision, and these patients do not require any form of chemotherapy. Mohs' micrographic surgery has been advocated for recurrent GCF. This is a margin controlled surgical resection technique. Although local recurrence is common, long-term outcome of this tumor is very good after reexcision of the tumor.
| Conclusion|| |
GCF is a rare childhood tumor of intermediate-grade malignancy which shares several features with DFSP. It is very important to keep it as a differential diagnosis in swelling in children below the age of 10.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The authors would like to thank Dr. Ranjana Solanki, Professor, MD in Pathology, Department of Pathology, SMS Medical College Jaipur, Rajasthan, India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]