| Abstract|| |
Proliferative fasciitis is a pseudo-sarcomatous myofibroblastic proliferation characterized by the presence of unusual giant cells resembling ganglion cells, which usually occurs in the adult population with a mean age of 54 years. It usually affects the upper extremities and is rare in head and neck region with only 11 cases described in the literature so far. Because of rapid growth and histological similarity to various malignant tumors such as rhabdomyosarcoma, recognition of this benign condition is a matter of utmost importance when a pathologist is concerned. In this study, we report a case of a 11-year-old boy presented with a lesion on the right side of chin, along with a literature review involving those cases reported in the head and neck region.
Keywords: Chin, ganglion cells, proliferative fasciitis
|How to cite this article:|
Jyothi H, Sudha S, Nair RG, Remya K. Proliferative fasciitis of the chin: A report of the rare case and review of literature. Indian J Dent Res 2019;30:630-3
|How to cite this URL:|
Jyothi H, Sudha S, Nair RG, Remya K. Proliferative fasciitis of the chin: A report of the rare case and review of literature. Indian J Dent Res [serial online] 2019 [cited 2020 Jul 5];30:630-3. Available from: http://www.ijdr.in/text.asp?2019/30/4/630/271092
| Introduction|| |
Proliferative fasciitis (PF), a term coined by Chung and Enzinger in 1975 is a pseudo-sarcomatous myofibroblastic proliferation characterized by the presence of unusual giant cells resembling ganglion cells. It belongs to a heterogeneous group of reactive soft tissue proliferative lesions, including nodular fasciitis and proliferative myositis. Proliferative myositis is a lesion which is having the same cellular composition occurring within skeletal muscle. PF develops more frequently in the upper extremities, particularly the forearms, followed by the lower extremities and the trunk. It usually grows rapidly and may clinically simulate a malignant mesenchymal tumor. The lesion is of unknown etiology, although a history of trauma can be elicited on the site of the onset of the lesion has been described in the literature. The exact incidence of this lesion in head cannot be assessed using available data. It usually affects adult population with a mean age of 54 years and is seen rarely in children below 15 years. In this study, we report a case of a 11-year-old boy presented with a nontender soft tissue swelling on the chin along with a literature review of the cases involving the head and neck region.
| Case Report|| |
An 11-year-old boy referred from a private hospital, presented in the Department. of Oral Pathology and Microbiology, Government Dental College, Kozhikode with a swelling of the right side of the chin which started about 2 months back after being hit by another child [Figure 1]. Initially, the swelling was soft and small and it rapidly enlarged and reached the present size. Physical examination revealed a firm flesh colored swelling of about 2 × 3 cm size which was freely movable and was non-tender. No intraoral findings were present [Figure 2]. His past medical history was unremarkable and routine laboratory examinations showed no abnormalities. Radiographic examination revealed no abnormal findings.
|Figure 1: Extra oral photograph of the patient showing soft tissue swelling on the right chin|
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Fine needle aspiration cytology of the lesion was done, which yielded a hemorrhagic fluid. Smears from the aspirate were cellular, which showed spindle-shaped cells with abundant eosinophilic cytoplasm as well as uni- and bi-nucleated ganglion-like cells. Considering these findings, a provisional diagnosis of nodular fasciitis was made.
After a week, complete excision of the lesion was performed and sent for histopathological analysis. Histologic sections showed a high cellularity with the proliferation of mainly two population of cells consisting of fibroblastic/myofibroblastic spindle cells and large ganglion-like cells with abundant amphophilic cytoplasm, and large vesicular nuclei containing 1 to 2 prominent nucleoli. The stroma varies from myxoid to collagenous with diffuse inflammatory infiltrate intermingling with numerous endothelium lined vascular channels [Figure 3], [Figure 4], [Figure 5].
|Figure 3: Low-power view showing both ganglion like and spindle cells in a loosely collagenous stroma|
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Immunohistochemical studies were performed to determine the cellular origin of this lesion using smooth muscle actin (SMA), vimentin, and pan-cytokeratin. Spindle-shaped cells showed SMA positivity, and ganglion-like cells and some spindle-shaped cells showed vimentin positivity [Figure 6] and [Figure 7]. Pancytokeratin was negative. In accordance with the immunohistochemical and histologic findings, a diagnosis of PF was made.
|Figure 6: Low-power view of ganglion-like cells showing vimentin positivity|
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|Figure 7: Low-power view of spindle cells showing smooth muscle actin positivity|
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After the surgery, patient recovered well and has been followed up for about a year with no evidence of recurrence.
| Discussion|| |
PF is a psuedosarcomatous lesion characterized by large ganglion-like cells in addition to plump fibroblastic/myofibroblastic cells similar to those seen in nodular fasciitis. Although it was considered to be similar to nodular fasciitis, a clear distinction was made by Chung et al. in 1975. PF is considered to be a lesion of adult life with most of the patients being in the age group ranging from 40–70 years (mean age-54 years). The childhood lesions were generally well circumscribed, lobulated, extremely cellular with less collagen production, and often associated with acute inflammation and microscopic foci of necrosis. It is of uncommon occurrence in a patient younger than 15 years. The age of the patient in the present case is 11 years [Table 1].
|Table 1: Consolidated data after reviewing the literature of case reports of Proliferative fasciitis on head and neck|
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PF over the head and neck are of rare occurrence, so far only 11 cases were reported including the present case. Considering the cases reported in sites other than head and neck, a slight male predominance can be noted. However, in the head and neck region, no such deduction can be made. According to Honda et al., more than 50% of the patients had pain associated with the lesion; in their study, Chung and Enzinger  found out that about two- third of patients had pain. Here, the patient presented with a non tender swelling in the right side of chin.
PF and many other psuedosarcomatous lesions are associated with bizarre histological features and history of trauma, both clinical and subclinical. Trauma or injury may cause proliferation of fibroblasts and cytokines or other factors secreted through the tissue repair, or the reactive process of the trauma may also contribute to cytological alteration of these immature fibroblasts, including the possible fusion of the cells that results in bi-nucleated giant cells, but further 2 months before investigation is required for clarification. In our case, the patient had a history of trauma 2 months before the occurrence of the lesion.
The fine-needle aspiration cytology (FNAC) features of nodular fasciitis and PF are essentially the same, except that PF shows lower cellularity, more collagen fragments, and abundant ganglion-like cells. FNAC of the lesion revealed the presence of spindle shaped cells as well as uni- and bi-nucleated ganglion-like cells. The diagnosis is usually depending on the histopathologic examination of the excised tissue. The lesion can be considered as a biphasic one consisting of two populations of cells; spindle shaped cells as well as ganglion-like giant cells in either a myxoid or collagenous stroma.
Histopathological examination of the present case revealed both spindle shaped cells and ganglion-like cells in a myxoid stroma. The cells usually stain positive for vimentin , and SMA, which also holds true in our case. Because of its high cellularity and increased growth rate the lesion can be misdiagnosed as sarcomas, so a careful diagnosis should be made. Treatment is total excision of the lesion, which was performed in the present case, and the patient showed complete resolution of the lesion. To the best of our knowledge, no recurrences are reported in the head and neck region so far after the complete excision of the lesion. The patient is on follow-up for about a year without any evidence of recurrence.
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.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Goldblum JR, Weiss SW. Enzinger and Weiss's Soft Tissue Tumors. 5th
ed. Cleveland: Elsevier Health Sciences; 2008.
Christopher DM, Fletcher, Unni KK. World Health Organization classification of tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press: WHO; 2002.
Meis JM, Enzinger FM. Proliferative fasciitis and myositis of childhood. Am J Surg Pathol 1992;16:364-72.
Honda Y, Oh-i T, Koga M, Serizawa H. A case of proliferative fasciitis in the Abdominal region. J Dermatol 2001;28:753-8.
Chung EB, Enzinger FM. Proliferative fasciitis. Cancer 1975;36:1450-8.
Sasano H, Yamaki H, Ohashi Y, Ohtsuki S, Nagura H. Proliferative fasciitis of the forearm: Case report with immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature. Pathol Int 1998;48:486-90.
Satish S, Shivalingaiah SC, Ravishankar S, Vimalambika MG. Fine needle aspiration cytology of pseudosarcomatous reactive lesions of soft tissues: A report of two cases. J Cytol 2012;29:264-6.
] [Full text]
Park SY, Kim GY, Chun YS. Sonographic appearance of proliferative fasciitis – A case report. J Clin Ultrasound 2017;45:445-9.
Morgan K, Batcup G, Aparicio S, Spicer RD, Marsden HB. Proliferative fascitis in childhood: A case report. Pediatr Pathol 1990;10:431-8.
Kiryu H, Takeshita H, Hori Y. Proliferative fasciitis. Report of a case with histopathologic and immunohistochemical studies. Am J Dermatopathol 1997;19:396-9.
Ushigome S, Takakuwa T, Takagi M, Koizumi H, Morikubo M. Proliferative myositis and fasciitis. Report of five cases with an ultrastructural and immunohistochemical study. Acta Pathol Jpn 1986;36:963-71.
Wong NL, Di F. Pseudosarcomatous fasciitis and myositis: Diagnosis by fine-needle aspiration cytology. Am J Clin Pathol 2009;132:857-65.
Magro G, Michal M, Alaggio R, D'Amore E. Intradermal proliferative fasciitis in childhood: A potential diagnostic pitfall. J Cutan Pathol 2011;38:59-62.
Stanzione B, Cozzolino I, Arpino G, Vigliar E, Virginia SF, Zeppa P, et al.
Multiple metachronus proliferative fasciitis occurring in different anatomic regions: A case report and review of the literature. Pathol Res Pract 2012;208:126-30.
Dr. H Jyothi
Jyothi Nilayam, TC-16/1448 (1), Thycaud PO, Trivandrum - 695 014, Kerala
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]