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 Table of Contents  
Year : 2021  |  Volume : 33  |  Issue : 3  |  Page : 344-347

Aggressive fibromatosis: A diagnostic paradox of 68 years old male patient-A case report

1 Department of Oral Medicine and Radiology, People's Dental Academy, Bhopal, Madhya Pradesh, India
2 Department of Oral and Maxillofacial Surgery, People's Dental Academy, Bhopal, Madhya Pradesh, India

Date of Submission28-Jan-2021
Date of Decision05-Aug-2021
Date of Acceptance08-Aug-2021
Date of Web Publication28-Sep-2021

Correspondence Address:
Dr. Saumya Khare
Department of Oral Medicine and Radiology, People's Dental Academy, Bhopal - 462010, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaomr.jiaomr_26_21

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Fibromatosis develops in the soft tissue and is the proliferation of well-differentiated fibroblasts and myofibroblasts. It is present most commonly in the abdominal region. Aggressive fibromatosis (AF) of the oral or para oral structures has been reported occasionally but, it is quite uncommon in the maxilla. We report a case of AF in a 68-year- old male in the maxilla, who came with a chief complaint of swelling in his upper left back tooth region which extended to the retromolar region and was clinico-histopathologically diagnosed as AF. All proliferative indurated growths are not always clear-cut malignancies.

Keywords: Aggressive fibromatosis, case report, desmoid tumor, maxilla

How to cite this article:
Khare S, Ramachandra Reddy G V, Pillai AK, Khare P. Aggressive fibromatosis: A diagnostic paradox of 68 years old male patient-A case report. J Indian Acad Oral Med Radiol 2021;33:344-7

How to cite this URL:
Khare S, Ramachandra Reddy G V, Pillai AK, Khare P. Aggressive fibromatosis: A diagnostic paradox of 68 years old male patient-A case report. J Indian Acad Oral Med Radiol [serial online] 2021 [cited 2023 Jan 31];33:344-7. Available from: http://www.jiaomr.in/text.asp?2021/33/3/344/326882

   Introduction Top

Aggressive fibromatosis (AF) is an intermediate tumor between benign and malignant, which is in propinquity to fibrosarcomas. They are locally invasive but never metastasize.[1] The terminology given by the World Health Organization (WHO) is extra-abdominal fibromatosis, other terms like extra-abdominal desmoid tumor and grade I fibrosarcoma are also used.[2] It is common in the shoulder girdle, thigh, and buttock of young adults, often associated with Gardner's syndrome. Fibromatosis of the head and neck are only 10% to 12% of reported cases of AF[3] and commonly affects the mandible in the maxillofacial region. This article reports a unique case of a 68-year-old male patient, with growth in the left maxillary posterior region, and gives an insight into clinicopathological and radiological features for effective management.

   Patient Information Top

A 68-year-old male patient reported to our department with swelling and pain in his upper left back tooth region since 2 months. He underwent extraction of decayed upper left back tooth two months back followed by which he developed swelling in the same region. The swelling was initially of pea size and gradually increased to present size, which was associated with moderate intensity, intermittent throbbing pain. The patient's medical and family history were non-contributory. The history of tobacco chewing was present for 6 years. All vital signs were within normal range.

Timeline of history

Clinical description

Extraoral examination revealed a diffuse swelling in the left middle third of the face with stretched overlying skin. On palpation, the local temperature was not raised, the swelling was tender, firm in consistency, non-compressible, and non-fluctuant. Regional lymph nodes were palpable, tender, and firm in consistency. Intraorally a solitary lobulated growth was present on the left posterior maxillary region, extending anteroposteriorly from the distal aspect of 26 to 28, bucco-palatally from mucogingival sulcus to approximately 2 cm away from the midline of the hard palate, measuring approximately 3 × 3 cm, with defined borders, the indentation of lower teeth, and ulcerated surface [Figure 1]. On palpation, it was tender, firm in consistency, sessile and fixed to the underlying structure. Hard tissue examination revealed a root stump of 45 and missing 11, 17, 28, and 46.
Figure 1: A solitary lobulated growth was present on the left posterior maxillary region with defined borders, indentation of lower teeth with ulcerated surface

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Diagnostic assessment

Based on history and clinical findings, a provisional diagnosis of benign non-odontogenic tumor in the left maxillary posterior region was made. Inflammatory hyperplasia, peripheral ossifying fibroma, peripheral giant cell lesion, malignant lesions, and minor salivary gland tumors were considered under differential diagnosis [Table 1]. Orthopantomogram revealed interdental bone loss extending from the distal aspect of 26 to the region of 28, giving a saucer-shaped appearance measuring approximately 2 × 1 cm [Figure 2]. Hematological investigations were within normal limits. Incisional biopsy was performed, histopathological examination revealed spindle cells arranged in whorls, fascicles, and wavy bundles with superficial necrosis of mucosa, which gave an impression of the benign mesenchymal lesion. Because of the pandemic, patient reported after a gap of 6 months with a further increase in the size of growth [Figure 3]. 3D CE-CT (contrast-enhanced computed tomography) revealed evidence of ill-defined heterogeneously attenuating lesion in upper left retromandibular trigone, measuring approximately 3.6 cm × 3.7 cm × 3.6 cm. Superiorly there was the erosion of superior alveolar ridge and inferior wall of the left maxillary sinus, antero-inferiorly displacement of a molar tooth, posteriorly abutting medial pterygoid muscle, and laterally mild infiltration in the posterior buccal mucosa, giving the impression of the malignant lesion [Figure 4].
Table 1: Differential diagnosis

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Figure 2: Orthopantomogram shows interdental bone loss extending from the distal aspect of 26 to the region of 28 giving saucer shaped appearance

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Figure 3: Overall Increase in the size of the growth after 6 months

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Figure 4: CECT (Contrast Enhanced Computed Tomography) Axial view -Bone window: Showing ill-defined heterogeneously attenuating lesion in upper left retromandibular trigone (White arrow), measuring approximately 3.6 × 3.7 × 3.6 cm, antero-inferiorly displacement of molar tooth (Red arrow) is seen giving impression of malignant lesion

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Therapeutic intervention

Complete excision of the lesion was done and histopathological examination revealed a cellular proliferation of spindle-shaped cells, arranged in fascicles with interspersed collagen, cells devoid of pleomorphism, and mild inflammatory cell infiltrate [Figure 5]. Based on these findings, a final diagnosis of AF in the left posterior maxillary region was made.
Figure 5: Histopathological picture after complete excision of growth showing cellular proliferation of spindle shaped cells (Black arrow), arranged in fascicles (Red arrow) with interspersed collagen and cell devoid of pleomorphism and mild inflammatory cells infiltrate giving an impression of Aggressive Fibromatosis

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Follow-up and outcomes

On follow-up visit after seven days, healing of site was evident. After a 4-month follow-up, there were no signs of recurrence [Figure 6]a and [Figure 6]b.
Figure 6: (a) Follow up picture after 4 months showing healed site. (b) Follow-up OPG taken after 4 months with no signs of recurrence

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   Discussion Top

The term fibromatosis shows a proliferation of well-differentiated fibroblasts and myofibroblasts.[2] Fibromatosis arises from the connective tissue of muscle, overlying fascia, periosteum, or Aponeurosis.[1] Enzinger and Weiss have classified fibromatosis into two types: Superficial and deep.[4] Superficial fibromatosis is slow-growing, small-sized, and encompasses palmar, plantar, penile, and knuckle pad lesions. Deep fibromatosis is also known as AF.[5] They are rapidly growing with a high recurrence rate and further divided into extra-abdominal (head and neck), abdominal, and intra-abdominal subtypes.[4] The extra-abdominal type is predominantly seen in the second and third decades[6] but the present case is of a 68-year-old patient. Only 5% of all cases affect the head and neck. AF was first identified by Stout in 1954[7] and it is a rare entity making up only 0.3% of all tumors with an annual incidence of around three to four per million of population.[5] Etiopathogenesis of AF is multifactorial, including local trauma, endocrine disturbances, and chromosome alterations.[8] Clinically, AF manifests as painless, firm, rapidly enlarging mass which is fixed to the underlying structure, Similar features were present in our case, though there was pain during the first visit, it can be attributed to surface ulceration.

Orthopantomogram of AF shows ill-defined osteolytic destructive lesions with the destruction of the cortical plate. Histopathologically, fibromatosis shows cellular proliferation of spindle-shaped cells arranged in streaming fascicles, associated with a variable amount of collagen.[9] Management of AF involves preoperative CT or MRI to identify tumor margins before surgical excision. Inoperable or unresectable tumors require a multidisciplinary treatment approach which includes chemotherapy, hyperthermy, therapy with hormonal agents (antiestrogen), and NSAID therapy.[1]

The recurrence rate is 46% to 62% in the head and neck region[10] thus a continuous follow-up protocol is maintained with the current case.

   Conclusion Top

This case report adds weight to the importance of considering AF, as it is rare in the maxillofacial region and if it occurs, it is more common in the mandible and in younger age but our case is of the maxilla in a 68-year-old patient. Although it has no metastatic potential, its treatment is challenging because of local infiltration and a high tendency for recurrence. Therefore, complete analysis in combination with advanced imaging is essential for early detection and better management. As it has a high recurrence rate, regular follow-up is imperative. Presently, the case is under follow-up for the past 5 months and there is no evidence of local 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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Seper L, Bürger H, Vormoor J, Joos U, Kleinheinz J. Agressive 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. 1
Angiero F, Benedicenti S, Stefan M. Fibromatosis of the head and neck: Morphological, immunohistochemical and clinical features. Anticancer Res 2008;28:1725-32.  Back to cited text no. 2
Fowler CB, Hartman KS, Brannon RB. Fibromatosis of the oral and paraoral region. Oral Surg Oral Med Oral Pathol 1994;77:373-86.  Back to cited text no. 3
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;14:333-4.  Back to cited text no. 4
Rao S, Dinesh BS. Aggressive fibromatosis of the oral cavity. Indian J Dent Res 2012;23:435.  Back to cited text no. 5
[PUBMED]  [Full text]  
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. 6
Schmoyer CJ, Brereton HD, Blomain EW. Contralateral recurrence of aggressive fibromatosis in a young woman: A case report and review of the literature. Oncol Lett 2015;10:325-8.  Back to cited text no. 7
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.  Back to cited text no. 8
  [Full text]  
Brasileiro Jr VL, Barboza de Carvalho LP, Vieira de Figueiredo CRL, Farias de Paiva MA. Pediatric fibromatosis involving mandible: Case report and a five-year post-operative follow-up. J Bras Patol Med Lab 2013;49:208-11.  Back to cited text no. 9
Garcia RG. Fibromatosis of the tongue: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:e31-4.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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