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Keywords:

  • Focal mucinosis;
  • oral pathology

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References

Oral focal mucinosis (OFM) is an uncommon soft tissue lesion of unknown aetiology. Clinically, it is most commonly found on the gingiva and presents as a painless, sessile or pedunculated mass that is the same colour as surrounding mucosa. Histologically, it is characterized by focal myxoid degeneration of connective tissue. OFM occurs predominantly in adults during the fourth and fifth decade of life, although it has been reported infrequently in children and adolescents. Diagnosis relies on histological analysis and treatment involves complete surgical excision. Recurrence is unreported. This case report describes the clinical and histological presentation and subsequent management of OFM in an adolescent female patient.


Abbreviations and acronyms:
CTM

cutaneous focal mucinosis

OFM

oral focal mucinosis

Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References

Oral focal mucinosis was first described by Tomich in 1974.1 Fifty-one cases of human OFM have since been reported, affecting children and adults ranging from 4 to 74 years.2,3 It most commonly occurs during the fourth to fifth decade of life and females are more frequently affected than males with a ratio of almost 2:1.2

The occurrence of OFM in young patients is rare. Four cases of OFM affecting individuals aged less than 18 years exist in the literature and are listed as follows: a 4-year-old girl with OFM of the hard palate4; a 16-year-old female with OFM of the anterior maxillary gingiva;5 a 16-year-old female with OFM of the anterior mandibular gingiva;6 and a 16-year-old male with OFM of the gingiva at an unknown location.1,2 A further four cases have been reported in females aged 18–22 years of age.2

The mucosa overlying bone appears to be the most affected.3 The gingiva is the most commonly reported site, most frequently the mandibular attached gingivae,2 followed by the hard palate.3 Other oral sites which have been reported include the buccal mucosa, tongue and lips. Histologically, OFM is characterized by a well-defined area of myxoid tissue containing an accumulation of hyaluronic acid and elongated, stellate fusiform or ovoid fibroblast cells in the subepithelium.3,6,7 There are few capillaries within the lesion.3 The overlying mucosa and surrounding connective tissue is normal.6 However, there have been reports of diffuse infiltration of mixed inflammatory cells and flattening of rete pegs.3 The objective of this paper was to report the occurrence and management of OFM in a 17-year-old female.

Case Description

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References

A 17-year-old female presented to the Paediatric Dentistry Department, Women’s and Children’s Hospital, Adelaide, South Australia, by referral from the Emergency Department of the same hospital. The presenting complaint was an asymptomatic lump on the gingival papilla lingual to the lower left central and lateral incisors. The history of the presenting complaint was unknown. The soft tissue lesion was approximately 6 mm in diameter and round in shape. The lesion was firm, sessile and non-tender to palpation. The overlying mucosal surface was smooth, not ulcerated and appeared the same colour as the surrounding mucosa (Fig. 1).

image

Figure 1.  Clinical appearance at the time of presentation.

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Systems review of the patient’s medical history included the following conditions: acute lymphoblastic leukaemia from age two and a half to four years of age, which was managed with chemotherapy without relapse; epilepsy diagnosed at age 11 years which was controlled with lamotrigine, clobazam and sodium valproate; and mild intellectual disability. Dental history revealed routine reviews, preventive treatments, and orthodontic extraction of the upper second premolars under local anaesthesia.

On clinical examination, the patient was in the permanent dentition. She demonstrated generalized late effects from oncology treatment including enamel hypoplasia and hypomineralization and microdontia. The lower left central and lateral incisors were positive to pulp sensibility tests, non-tender and demonstrated normal mobility. No radiographic changes were noted in the area of the soft tissue lesion.

The lesion was managed with excisional biopsy under nitrous oxide sedation and local anaesthesia. Electrocautery was performed to achieve haemostasis. Histopathologic examination was performed; essential with all soft tissue biopsies. However, histopathological assessment was of particular importance in this case due to the history of malignancy. It revealed myxomatous connective tissue with a plasma cell infiltrate on haematoxylin and eosin stained sections. Alcian Blue-PAS pH 2.5 showed strong blue staining in keeping with the presence of hyaluronic acid (Fig. 2).

image

Figure 2.  Histopathology slides stained with alcian blue-PAS.

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Based on the clinical and histologic findings, a diagnosis of OFM was established. At one-week postoperative review, the biopsied area had healed without complication. The patient is currently under routine periodic dental review.

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References

The aetiology of OFM is unknown.6 Tomich, who described OFM as a counterpart to cutaneous focal mucinosis (CFM), suggested the pathogenesis involved ‘overproduction of hyaluronic acid by the fibroblasts at the expense of collagen production’.1 However, the cause of this overproduction is not known.1 There is controversy as to whether local trauma plays a role in the aetiology of OFM. Tomich said it does not play a role1 whilst others feel that trauma may be a contributing factor.4

The differential diagnosis of OFM based on clinical appearance varies upon location but includes neoplastic and inflammatory lesions such as fibrous hyperplasia, squamous papilloma, giant cell fibroma, peripheral giant cell granuloma, peripheral ossifying fibroma, peripheral odontogenic fibroma and pyogenic granuloma.2,3,6,8,9 Preoperative diagnosis of OFM is difficult due to infrequent occurrence and lack of distinctive clinical features.1,3 Despite being rare, malignant and metastatic lesions cannot be ruled out on clinical examination alone.9 Diagnosis is made by histologic analysis.3

Histologic differential diagnosis includes neural tumours with myxoid degeneration, neurofibroma, nerve sheath myxoma, mucocele, odontogenic myxoma and soft tissue myxoma.1,3,10 The histologic features of OFM are well recognized.6 The OFM lesion contains a well-circumscribed, localized area of myxoid connective tissue comprising of ovoid and stellate fibroblasts in an alcianophillic stroma.1 Fibroblasts contain a small amount of cytoplasm and may have delicate fibrillate processes extending into the surrounding matrix.1

The current management of OFM comprises of surgical excision and is considered curative.1,2 Recurrence of OFM lesions has not been reported.1 The period of follow-up varies among the cases reported with an example of no evidence of recurrence after 19 years.6

The clinical appearance of this OFM case is similar to that described in the literature. Aldred et al. reported a series of 15 cases of OFM which were mainly less than 10 mm in diameter, firm on palpation, and covered with normal colour, non-ulcerated mucosa.6

This report brings the total number of OFM cases in the literature to 52, and is the fifth case of OFM in a patient younger than 18 years. Despite infrequent occurrence, it is recommended that OFM is considered as a differential diagnosis of oral soft tissue lesions in adolescent patients.

Conclusions

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References

OFM occurs infrequently and histological assessment of features is necessary for diagnosis. However, OFM should be considered in the differential diagnosis of soft tissue lesions in the adult, adolescent and the paediatric population.

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case Description
  5. Discussion
  6. Conclusions
  7. References
  • 1
    Tomich CE. Oral focal mucinosis: a clinicopathologic and histochemical study of eight cases. Oral Surg Oral Med Oral Pathol 1974;38:714724.
  • 2
    Soares de Lima AA, Naval Machado MA, Martins WD, et al. Oral focal mucinosis. Quintessence Int 2008;39:611615.
  • 3
    Madhusudhan AS, Nagarajappa D, Manjunatha BS, Saawarn Swati, Charan Babu HS. Oral focal mucinosis: report of two cases. Rev Odonto Cienc 2010;25:310313.
  • 4
    Gnepp G, Vogler C, Sotelo-Avila C, Kielmovich IH. Focal mucinosis of the upper aerodigestive tract in children. Human Pathol 1990;21:85658.
  • 5
    Buchner A, Meerrell PW, Leider AS, Hansen LS. Oral focal mucinosis. Int J Oral Maxillofac Surg 1990;19:337340.
  • 6
    Aldred MJ, Talacko AA, Ruljancich K, et al. Oral focal mucinosis; report of 15 cases and review of the literature. Pathology 2003;35:393396.
  • 7
    Soda G, Baiocchini A, Bosco D, Nardoni S, Melis M. Oral focal mucinosis of the tongue. Pathol Oncol Res 1998;4:304307.
  • 8
    Iezzi G, Rubini C, Fioroni M, Paittelli A. Oral focal mucinosis of the gingiva: case report. J Periodontol 2001;72:11001102.
  • 9
    Gabay E, Sharon A, Machtei EE. Oral focal mucinosis associated with cervical external root resorption: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:e7578.
  • 10
    Saito I, Ide F, Enomoto T, Kudo I. Oral focal mucinosis. J Oral Maxillofac Surg 1985;43:372374.