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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 31  |  Issue : 3  |  Page : 263-269

Benign fibromatosis of submandibular gland - Radiological and immunohistochemical characteristic features


1 Department of Oral Medicine and Radiology, New Horizon Dental College and Research Institute, Sakri, Bilaspur, Chhattisgarh, India
2 Department of Oral Medicine and Radiology, Swami Devi Dayal Hospital and Dental College, Panchkula, Haryana, India

Date of Submission30-Apr-2019
Date of Acceptance13-Jun-2019
Date of Web Publication30-Sep-2019

Correspondence Address:
Dr. Ravleen Nagi
Department of oral Medicine and Radiology, Swami Devi Dayal Hospital and Dental College, Panchkula, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_98_19

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   Abstract 


Fibromatosis are benign well-differentiated fibroblastic proliferations that can occur anywhere in the body, but their occurrence in maxillofacial region is rare entity. Clinically, these tumors are intermediate grade neoplasms, present as firm nodular mass, are locally invasive, have high propensity of recurrence particularly in the head and neck area but do not metastasize. We report a rare case of 45-year-old female patient, who presented with the swelling in the left submandibular region which was confirmed as benign fibrous tumor, fibromatosis, histopathologically, and by immunohistochemical analysis.

Keywords: Fibromatosis, immunohistochemistry, management, spindle cell neoplasm


How to cite this article:
Naidu GS, Bhandari J, Nagi R, Makkad RS. Benign fibromatosis of submandibular gland - Radiological and immunohistochemical characteristic features. J Indian Acad Oral Med Radiol 2019;31:263-9

How to cite this URL:
Naidu GS, Bhandari J, Nagi R, Makkad RS. Benign fibromatosis of submandibular gland - Radiological and immunohistochemical characteristic features. J Indian Acad Oral Med Radiol [serial online] 2019 [cited 2019 Dec 14];31:263-9. Available from: http://www.jiaomr.in/text.asp?2019/31/3/263/268284




   Introduction Top


Fibromatosis are benign fibrous tumors that arise from connective tissue of muscle, overlying fascia, periosteum, or aponeurosis.[1],[2] They are considered as atypical uncommon variants of spindle cell neoplasms with an estimated incidence of approximately two to four cases per million patients per year and their biologic behavior lies between the benign fibromas and well-differentiated fibrosarcoma.[3],[4] A total of 12–15% of all fibromatoses occur in the head and neck area, of these 40–85% of tumors arise in the neck area. Their occurrence in the submandibular region and oral cavity is rare and mostly present as firm nodular painless mass fixed to underlying structures.[1],[4] Despite their benign nature, these tumors are locally aggressive, rarely metastasize, and tend to recur even after complete resection especially in the head and neck area due to proximity to vital structures.[4] This article report a case of 45-year-old female patient, with the swelling in the left submandibular region since 2 years and discusses its clinicopathological, radiological, and immunohistochemical (IHC) features for effective management.


   Case Report Top


A 45-year-old female patient reported to the Department of Oral Medicine and Radiology with a complaint of difficulty in opening mouth since 1 month associated with toothache in left mandibular posterior region. She also complained of swelling in the left submandibular region since 2 years that gradually progressed but was static since 1 year and was not associated with fever or weight loss but patient gave history of pain since 1 month that radiated to ear and temporal region. There was no associated history of trauma except the gudakhu (mixture of tobacco powder, molasses, and flavoring ingredients) massage by the patient on the gingiva since 2 years and she was also using it to relieve pain. Patient's medical and family history was non contributory. Patient's general appearance was normal, except for asymmetry of face owing to the swelling in submandibular region. All the vital signs were within the normal range. Extraoral and intraoral examination revealed that there was restricted mouth opening of 1 cm and extraorally well-defined solitary swelling was present on left submandibular region which was oval in shape, extending superiorly from left inferior border of mandible till submandibular region inferiorly, anteriorly 3 cm away from the corner of mouth to the tragus of ear posteriorly including the angle of mandible, it measured around 2 × 2 cm in size and no change in color was observed. Swelling was well-defined, tender, firm to hard in consistency, non compressible, nonfluctuant, and fixed to underlying structures on palpation [Figure 1]a and [Figure 1]b. Regional lymph nodes were palpable and non tender. On intraoral examination, no palpable mass was evident on bimanual palpation of left posterior floor of the mouth, normal salivary flow rate was expressed from the ductal orifice on milking of the glands thus excluding any ductal pathology causing enlargement of submandibular region. On hard tissue examination, 38 was deeply carious and non tender on percussion. Based on patient's history and clinical examination, provisional diagnosis of consolidated chronic odontogenic abscess was made. Subacute sialadenitis, chronic infective sialadenitis, lymphoepithelial or branchial cyst, and neoplastic pathologies, such as tumors of the submandibular gland mainly pleomorphic adenoma, adenoid cystic carcinoma, were considered under differential diagnosis. For further confirmation of diagnosis, patient was subjected to radiological and histopathological investigations.
Figure 1: (a and b) Extraoral photograph of the patient showing swelling in the left submandibular region

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Intraoral periapical radiograph [Figure 2]and panoramic radiograph [Figure 3] were taken for the patient that showed carious 38 with periapical rarefaction. Patient was prescribed systemic antibiotics (amoxicillin clavulanic acid 625 mg three times a day) and analgesics (ibuprofen 400 mg three times a day) for 5 days. On follow-up visit, we found symptomatic relief in pain but there was no reduction in size of swelling. Further, to rule out nonodontgenic and other neoplastic pathologies, ultrasound scan (US) was done which revealed, relatively bulky submandibular gland with well-defined mixed echoic soft tissue mass lesion of approximately 3.1 × 1.9 cm seen arising from anterior aspect of the gland [Figure 4]. On color Doppler, minimal internal vascularity was noticed. Computed tomography (CT) axial view demonstrated encapsulated, well-defined hyperdense mass measuring approximately 2.4 × 1.7 cm in size in the left submandibular region. Contrast-enhanced CT revealed minimally enhancing soft tissue density mass lesion in the left submandibular region. Fat plane between lesion and gland was not maintained suggestive of it arising from submandibular gland[Figure 5]. Based on the findings, benign tumor of the submandibular gland, such as pleomorphic adenoma, adenoid cystic carcinoma was considered under radiological diagnosis. After all the routine blood investigations that were found to be within normal range, surgical excision of the lesion was done under general anesthesia [Figure 6]a and [Figure 6]b. Risdon approach submandibular incision was given, then mass and left submandibular gland was surgically removed [Figure 6]a. Excised tissue specimen was sent for histopathological examination [Figure 6]b. Microscopically, lobules of serous salivary gland with fibrous connective tissue septae and glandular tissue composed of serous acini and intercalated ducts were seen. Fibrous septae was made of collagen fibers, fibroblasts, blood vessels, and mild chronic inflammation. Other than that, highly cellular interlacing and streaming fascicles of fibroblasts were seen. Fibroblasts were spindle-shaped with large, oval, and pale nucleus. Normal fibrous connective tissue with few skeletal muscle fibers and serous acini at one end were also noted [Figure 7]a, [Figure 7]b, [Figure 7]c, [Figure 7]d. IHC analysis was performed on tissue specimen andIHC markers assessed were smooth muscle actin (SMA), caldemson, β-catenin, CD34, S-100, and cytokeratin [Figure 8]. The specimen was found to be immunoreactive only for SMA indicative of presence of the smooth muscle cells in the glandular epithelium [Figure 8]d and tumor was finally diagnosed as fibromatosis of submandibular gland. Patient was followed-up after 6 weeks and no relapse of the lesion was observed.
Figure 2: Intraoral periapical radiograph shows carious 38 with periapical rarefaction

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Figure 3: Panoramic radiograph showing carious 38 with periapical rarefaction (white arrow)

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Figure 4: US revealing bulky submandibular gland with well-defined mixed echoic soft tissue mass arising from anterior aspect of gland

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Figure 5: Axial CTrevealing encapsulated, well-defined hyperdense mass in the left submandibular region.Minimally enhancing soft tissue density mass lesion seen in the left submandibular region on the contrast-enhanced CT (white arrow)

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Figure 6: (a) Surgical excision of the lesion (b) Excised surgical specimen (macroscopic appearance)

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Figure 7: Histopathology showing (a and c) cellular connective tissue stroma composedofcollagen fibers, inflammatory cells, blood vessels, and slit-like clear spaces (H and E,10×. (b and d) Interlacing and streaming fascicles ofspindle-shaped fibroblasts with large, oval, and pale nucleus (H and E,100×

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Figure 8: IHC staining reveals (a) strong expression for smooth muscle actin and negative expression for (b)β catenin,(c) caldesmon,(d) CD 34,(e) cytokeratin, CK, and (f) S100

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   Discussion Top


Fibromatoses are benign fibrous tumors that originate from the musculoaponeurotic structures in the body and histologically reveal spindle shaped fibroblasts arranged in fascicles with abundant collagenous stroma. The important therapeutic concern is their local aggressiveness and tendency to recur even after complete resection.[1] A total of 12–15% of fibromatosis occur in the head and neck region in children and young adults, of which 26% have been reported to involve submandibular area and 3% parotid gland.[4] They are atypical variants of spindle cell carcinoma that predominantly consist of spindle or pleomorphic fibroblasts and their occurrence in the submandibular region poses diagnostic challenge for a clinician [Table 1].[3] Several classification systems have been proposed for fibromatosis, Enzinger and Weiss have subdivided fibromatosis into two types: superficial and deep. Superficial fibromatoses are slow growing, of small size, and encompass palmar, plantar, penile, and knuckle pad lesions. Deep fibromatoses are rapidly growing, of large size and have a high recurrence rate.[2] They are also known as desmoids tumors, aggressive fibromatosis, or aggressive type fibrosarcoma and are further divided into extra abdominal that include head and neck, abdominal, and intra abdominal subtypes.[4]
Table 1: Classification of spindle cell neoplasms of oral cavity[3]

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Pathogenesis of fibromatosis is multifactorial, local trauma, endocrine disturbances, such as puberty and pregnancy, inappropriate gene expression, chromosome alterations have been found to be associated with the development of fibromatosis.[4],[5] Many clinicians have found that patients usually give history of local trauma as immature fibroblasts engaged in healing process begin uncontrolled proliferation but no above-mentioned specific cause was detected in our patient except the association of gudhaku usage.[1],[4] Clinically, fibromatosis is common in elderly females and presents as painless palpable slow-growing mass, fixed to underlying soft tissues. Chang et al. reported a case of fibromatosis in the right submandibular region in a 42-year-old female patient who presented with right submandibular mass that slowly increased in size, was nontender, firm, and fixed.[1] Rao and Dinesh reported another case in a 50-year-old female patient of aggressive fibromatosis, who presented with swelling on left side of the face which was nontender on palpation.[6] The clinical findings were consistent to the current case as our patient was 45-year-old female and swelling was slowly progressive and nontender on palpation. [Table 2] summarizes previously reported cases of fibromatosis from the literature.
Table 2: Summary of previously reported cases of fibromatosis from the existing literature

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Histologically, fibromatosis is characterized by spindle-shaped fibroblasts arranged in fascicles with an abundant collagenous component. There is usually absence of any cellular atypia or mitotic figures.[1] Our case also comprised streaming fascicles of spindle-shaped fibroblasts in fibrous connective tissue. It is essential for a clinician to differentiate this neoplasm histologically from myxoma, reactive fibrosis, nodular fasciitis, fibrosarcoma, and fibrous hamartoma to prevent under diagnosis and for better prognosis [Table 3].[7],[8],[9],[10],[11],[12]
Table 3:Differential diagnosis offibromatosisbased on clinical and histopathological features

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Still further techniques such as IHC analysis are necessary to characterize the biological behavior, nature, and aggressiveness of the neoplasms.[7] In the present case, SMA marker showed strong positivity on IHC staining, which identifies myoepithelial cells in normal or neoplastic salivary glands.[13] SMA positivity isalsoseen in myofibromas and benign fibrous histiocytoma; hence, it is important for a clinician to differentiate these pathologies from low grade fibromatosis. Myofibroma is afibroblastic, uncommon spindle cell neoplasm, consisting of benign proliferation of myofibroblasts and typically occurs in early infancy with few cases in adults. The tumor has predilection for head and neck areas with cases involving mandible, tongue, lip, buccal mucosa, maxilla and floor of mouth. Clinically, lesion grows as asymptomatic swelling unless ulcerated. Microscopically, characteristic feature is biphasic pattern, i.e., outer periphery of spindle-shaped cells with long oval nuclei resembling smooth muscle and central zone consisting of more primitive spindle cells with hemangiopericytoma like vascular pattern. Definitive diagnosis is usually made by positivity of various markers S100, vimentin, CK, SMA, and desmin.[14] Benign fibrous histiocytomais a fibrohistiocytic tumor arising in buccal spaces with few cases in tongue, gingiva, mandible, maxilla, lower and upper lip, and soft palate. Rarely, the lesions have been seen involving parotid and submandibular glands. The lesions, occur in sun exposed areas, are common in young- and middle-aged women and usually presents as a painless mass. Microscopically, the lesion comprises fascicular arrangement of spindle-shaped fibroblasts with intermixed plump, polygonal histiocytic cells, and show no demonstrable mitotic figures. Usually, these tumor cells are positive for CD34, CD68, and SMA.[15] The features of benign fibrous histiocytoma can be correlated to the present tumor based on patient's age and location. Moreover, the lesion was asymptomatic and but microscopically plump proliferating histiocytocytes were not noticed in our case. Features of myofibroma, i.e. occurrence of lesion in infancy, long-term association with ulceration, demonstration of myofibroblasts, and biphasic pattern histopathologically lead to exclusion of this benign neoplasm.

Surgical excision with positive margins is mainly preferred to treat fibromatosis and preoperative CT or magnetic resonance imaging is mandatory to identify tumor margins and for treatment planning. In cases of high recurrences, radiotherapy or other treatments, such as chemotherapy, hormonal therapy, nonsteroidal anti-inflammatory drugs, steroids, percutaneous cryoablation have been recommended. Radiation therapy is avoided to small areas of head and neck as it can cause tissue fibrosis or secondary neoplasm. Recent therapies such as percutaneous cryoablation or medicinal treatment should be encouraged in such cases. Follow up of fibromatosis patients is mandatory as this tumor has high recurrence rate although it is benign but is locally invasive and has tendency to invade vital structures.[1],[4] Hence, thorough evaluation is important by correlation between the clinicoradiological and histological findings for better management and in future, further reports or trials should be encouraged for early diagnosis of these rare neoplasms.


   Conclusion Top


Fibromatoses are benign fibrous neoplasms consisting of fibroblastic cells in the spindle-shaped morphology. Although they have no metastatic or malignant potential, but their treatment is challenging for the clinicians due to local infiltration and high tendency of recurrence. Therefore, complete analysis of these tumors in combination with advanced imaging modalities and recent IHC staining methods is essential for early detection and better management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Chang YN, Chaung YC, Lin YS. Fibromatosis of the submandibular region.J Chin Med Assoc 2009;72:492-4.  Back to cited text no. 1
    
2.
Seper L, Burger H, Vormoor J, Joos U, Kleinheinz J. Aggressive fibromatosis involving the mandible: Case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:30-8.  Back to cited text no. 2
    
3.
Shamim T. The spindle cell neoplasms of the oral cavity. Iranian J Pathol 2015;10:175-84.  Back to cited text no. 3
    
4.
Burlini D, Conti G, Bardellini E, Amadori F. Rare case of desmoid-type fibromatosis of the mandibular region in a child: Diagnosis and surgical management. Eur J Paediatric Dent 2013;14:333-34.  Back to cited text no. 4
    
5.
Varekar AA, Patil SB, Patil A, Golgire SM, Patil PD, Magdum DB. Juvenile aggressive fibromatosis of the oral cavity treated conservatively: A case report. Curr Pediatr Res 2016;20:301-3.  Back to cited text no. 5
    
6.
Rao S, Dinesh BS. Aggressive Fibromatosis of the oral cavity. Indian J Dent Res 2012;23:435.  Back to cited text no. 6
    
7.
James L, Shetty A, Jaypal N, Okade D. Oral soft tissue myxoma.J Indian Acad Oral Med Radiol 2012;24:152-4.  Back to cited text no. 7
  [Full text]  
8.
Damasceno LS, da Silva Gonçalves F, Silva ES, Zenóbio EG, Souza PEA, Horta MCR. Stromal myofibroblasts in focal reactive over growths of the gingival. Braz Oral Res (São Paulo) 2012;26:373-7.  Back to cited text no. 8
    
9.
Jang EA, Park TI, Nam SC, Park JY. Nodular fasciitis in the submandibular gland. Diagn Cytopathol 2008;36:805-8.  Back to cited text no. 9
    
10.
Peng WX, Kudo M, Yamamoto T, Inai S, Fujii T, Teduka K, et al. Nodular fasciitis in the parotid gland: A case report and review of the literature. Diagn Cytopathol 2013;41:829-33.  Back to cited text no. 10
    
11.
Yuwanati MB, Tupkari JV. Fibrosarcoma of mandible-A diagnostic dilemma. J Clin Diagn Res 2013;7:1804-5.  Back to cited text no. 11
    
12.
Kang G, Suh YL, Han J, Kwon GY, Lee SK, Seo JM. Fibrous hamartoma of infancy: An experience of a single institute. J Korean Surg Soc 2011;81:61-5.  Back to cited text no. 12
    
13.
Al-Bayaty H, Balkaran RL. Spindle cell carcinoma of the mandible: Clinicopathological and immunohistochemical characteristics. J Oral Biol Craniofac Res 2016;6:160-3.  Back to cited text no. 13
    
14.
Brasileiro BF, Martins-Filho RCS, Piva MR, da Silva LCF, Nonaka CFW, Miguel MCD. Myofibroma of the oral cavity. A rare spindle cell neoplasm. Med Oral Patol Oral Cir Bucal 2010;15:e596-600.  Back to cited text no. 14
    
15.
Khalkhali K, Azizi MR, Atighechi S. A fibrous histiocytoma of intermediate malignancy arisen from the parotid gland. Arch Iranian Med 2007;10:100-3.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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