Journal of Oral and Maxillofacial Pathology

CASE REPORT
Year
: 2022  |  Volume : 26  |  Issue : 1  |  Page : 101--103

A novel mucocele: Myxoglobulosis


Rutuja Gajanan Vidhale, Subraj Shetty, Nikita Kamble, Treville Pereira 
 Department of Oral Pathology and Microbiology, D.Y. Patil Deemed to be University, School of Dentistry, Navi Mumbai, Maharashtra, India

Correspondence Address:
Treville Pereira
Department of Oral Pathology, D.Y. Patil Dental College, D.Y. Patil University, Mumbai, Maharashtra
India

Abstract

Oral extravasation mucoceles are among the most widely recognized submucosal lesions which are usually a result of trauma involving salivary glands and their ducts. This paper reports a case of a 40-year-old female patient with a chief complaint of a painless swelling on the lower lip since 6 months. Upon a provisional diagnosis of mucocele, surgical excision was carried out. Histopathological examination revealed a mucus extravasation cyst having lumen filled with unique mucinous globular structures similar to a rare variant of appendiceal mucocele, myxoglobulosis.



How to cite this article:
Vidhale RG, Shetty S, Kamble N, Pereira T. A novel mucocele: Myxoglobulosis.J Oral Maxillofac Pathol 2022;26:101-103


How to cite this URL:
Vidhale RG, Shetty S, Kamble N, Pereira T. A novel mucocele: Myxoglobulosis. J Oral Maxillofac Pathol [serial online] 2022 [cited 2022 Jul 1 ];26:101-103
Available from: https://www.jomfp.in/text.asp?2022/26/1/101/341393


Full Text



 Introduction



Mucoceles are one of the most common benign soft tissue lesions of the oral cavity which often results due to local trauma and consequent rupture of salivary gland ducts, especially in the lower lip.[1] They represent a localized accumulation of saliva that is classified into two types based on its pathogenesis. The term mucus extravasation phenomenon is used when there is mucin leakage into the connective tissue from a severed minor salivary duct. Whereas, mucus retention cyst is accumulation of mucin within the lumen of a distally obstructed salivary duct.[2] Apart from the conventional classification, other variants are also seen: (1) superficial mucoceles, (2) mucoceles with myxoglobulosis and (3) mucoceles exhibiting papillary synovial metaplasia like change.[3] The case reported in this article, reveals an extravasation mucocele showing unique globular organization of mucous content representing myxoglobulosis.

 Case Report



A 40-year-old female patient was referred for evaluation of an asymptomatic swelling on the lower lip of 6 months duration with a history of local trauma. Clinical examination revealed a well-defined, nontender, smooth-surfaced, roughly oval, fluctuant swelling [Figure 1]. No relevant medical history was elicited. Oral hygiene was fair. A provisional diagnosis of mucocele was made; the lesion was excised under local anesthesia. Histopathological examination of the excised tissue revealed cystic lumen devoid of lining epithelium and surrounded by compressed granulation tissue and peripherally located mixed salivary glands. The lumen was filled with numerous mucinous globular structures which were oval or round in shape and of varying sizes [Figure 2]. Most of the globules present in the cystic lumen were attached to the surrounding cystic capsule and seemed dissociated from one another because of their globular organization. Some of the globules were suspended freely within the lumen [Figure 3]. Individual globules exhibited a mildly cellular core with peripheral laminations of dystrophic calcifications [Figure 4]. The granulation tissue forming the cystic wall was highly cellular, consisting of chronic inflammatory cells. The mucinous globules showed positive results for PAS and Alcian blue [Figure 3] and [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}

 Discussion



Mucus extravasation phenomenon most commonly affects children or young adults, aged between 10 and 30 years. About 80%–90% cases are observed in the lower lip as an asymptomatic swelling with translucent or bluish hue.[2] Histologically, the presence of a well-demarcated interstitial mucin surrounded by a granulation tissue containing neutrophils and multinucleated giant cells in the submucosa serves as a peculiar feature to diagnose mucus extravasation phenomenon.[2] Myxoglobulosis or caviar appendix is a variant of mucocele characterized by the presence of opaque pearl-like globules composed of mucoid material. They are generally 2–3 mm in diameter and form clusters that appear like “fish eggs” or frogspawns.” The first case of myxoglobulosis was accidentally discovered during a postmortem examination by Latham in the year 1897.[4]

In the present case, overall clinical and histopathological features were suggestive of an extravasation mucocele. Albeit, the diagnosis of extravasation mucocele is usually straightforward, the presence of globular structures within the lumen is unique and this condition is referred to as myxoglobulosis. The globules present in this case showed mildly cellular core with peripheral laminations of dystrophic calcifications. This might be a result of a long-standing lesion which has become less cellular over a period of time as stated by KA Shah.[5]

The precise etiology and pathogenesis of myxoglobulosis remain unknown. However, the etiological factors for myxoglobulosis of the intestine or appendix, as suggested by Probstein and Lassar include bacteria and necrotic epithelial debris which represent the focus of infection for mucin pooling.[4] An uncommon histologic feature was observed in the lower lip mucocele which bore a resemblance to globules observed in a variant of mucocele of the vermiform appendix by Li et al. in 1997 and thus was described as “myxoglobulosis.” The globules represent an attempt to organize the mucin by the granulation tissue capsule, which is then expelled into the lumen because of persistent mechanical stresses.[6] Ide and Kusama proposed that the formation of these pearl-like globules of salivary mucocele develop as a result of a rich reparative interaction of the capsular granulation tissue in response to the intraluminal pooling of mucin.[6] Despite the etiology, the significance of myxoglobulosis is exclusively academic. Mucus extravasation phenomenon associated with myxoglobulosis does not reveal distinguishing clinical features, nor is the prognosis any different from conventional types. Treatment includes excision of salivary gland and associated duct along with the pool of extravasated mucin to avoid recurrence. Regardless of not being clinically significant, myxoglobulosis continues to be a remarkable phenomenon for its relative rarity.[2]

Acknowledgement

I am sincerely thankful to D.Y. Patil Dental College for providing me the opportunity to write a case report on the above topic.

I am also thankful to Dr. Treville Pereira for guiding me in every stage of this paper. Without his support, it would've been extremely difficult for me to prepare the paper so meaningful and interesting.

I am also thankful to Dr. Subraj Shetty who has helped me during this course of paper in several ways.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Conceição JG, Gurgel CA, Ramos EA, De Aquino Xavier FC, Schlaepfer-Sales CB, Cangussu MC, et al. Oral mucoceles: A clinical, histopathological and immunohistochemical study. Acta Histochem 2014;116:40-7.
2Schulman JM, Jordan RC. Oral mucus extravasation phenomenon with myxoglobulosis. J Cutan Pathol 2014;41:767-70.
3Chi AC, Lambert PR 3rd, Richardson MS, Neville BW. Oral mucoceles: A clinicopathologic review of 1,824 cases, including unusual variants. J Oral Maxillofac Surg 2011;69:1086-93.
4Probstein JG, Lassar GN. Mucocele of the appendix, with myxoglobulosis. Ann Surg 1948;127:171-6.
5Shah KA. Myxoglobulosis in oral extravasation mucocele: An entity? Histopathology 2003;43:291-6.
6Li TJ, Kitano M, Yoshida A, Iwashige Y, Yamashita S. Myxoglobulosis in an extravasation mucocele of the lower lip. J Oral Pathol Med 1997;26:342-s4.