A clinical case of manifestations of dysgenetic polycystosis of the parotid salivary glands

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Introduction

Dysgenetic polycystosis, an anomaly of the salivary gland system development, is a unique and poorly understood pathology of the salivary glands. There is a lack of a clear understanding of the etiology and pathogenesis of the disease, the clinical picture can be largely similar to other salivary gland disorders, and there is no consensus on treatment strategies. Only 21 cases of dysgenetic polycystosis have been described in the international literature [1]. There is no data on this nosology in Russian-language sources. A rare clinical case of dysgenetic polycystic parotid salivary gland disease is described in a patient treated in the Department of Maxillofacial Surgery of the Clinical Dental Hospital of the Perm State Medical University named after E.A. Wagner of the Russian Ministry of Health.

Dysgenetic polycystic disease was first described in 1962 [2]. Most often, the pathological process occurs in the parotid salivary gland, however, isolated cases of damage to the submandibular salivary glands and minor salivary glands of the tongue have been described [1; 3]. In 1981, the cause of polycystic disease was established, associated with anomalies in the development of the terminal duct system of the gland during the embryonic period of development [4]. This nosology has a hereditary nature [1]. There is a hypothesis about an autosomal dominant type of inheritance. Hormonal changes, including pregnancy, can be a trigger for the appearance of clinical symptoms of polycystosis [5]. The clinical presentation of dysgenetic polycystosis is subtle. It is characterized by recurrent, painless enlargement of the affected salivary glands in the absence of significant somatic pathology. The disease most often manifests in young adults and can remain undetected for a long time, with pain appearing only when infection occurs [5]. To diagnose polycystic parotid salivary gland disease, E. Brown et al. identified the following features: a) bilateral enlargement of the parotid salivary glands, more often in females; b) absence of autoimmune and other systemic diseases; c) presence of characteristic multiple areas of reduced signal in the parenchyma of the glands on MRI [5]. Dysgenetic polycystosis should be differentiated from Warthin's tumor, pleomorphic adenoma, mucoepidermoid carcinoma, and adenoid cystic carcinoma [6]. The diagnosis is confirmed by intravital pathological examination of biopsy material. The first histological description of polycystosis was made in 1981 [6; 7]. The microscopic picture of the disease is in many ways reminiscent of polycystic pancreas and kidney disease [8]. The entire salivary gland can be filled with cysts of varying sizes, with normal intersepta, residual serous acini, and excretory ducts between them. The architecture of the salivary gland remains unchanged. Cysts are lined with single-layered cuboidal, squamous, or columnar epithelium, with eosinophilic cytoplasm. The cells may also acquire a pseudopapillary shape with a large amount of stainable lipid. Duct dilation is noted in the remaining intact acini [5; 9]. Sialography is not used in the diagnosis of dysgenetic polycystic disease, as there are no pathognomonic radiographic signs of the disease. Furthermore, this type of examination may provoke further dilation and rupture of terminal ducts and cysts, followed by the development of acute inflammation [10]. There is no single treatment strategy for patients with dysgenetic polycystic salivary glands. A number of authors describe successful outcomes of surgical treatment in cases where the pathological process is localized superficially or in one of the lobes of the parotid gland (lobectomy) [4]. In cases of unilateral cyst localization, parotidectomy is recommended [8]. At the same time, most authors adhere to conservative treatment tactics, since the disease does not belong to the group of neoplastic diseases, has a benign course, and the number and size of cystic cavities most often remain unchanged. In this case, dynamic observation is recommended [5]. Currently, the problem of studying dysgenetic polycystosis remains relevant.

The aim of the study is to analyze a clinical case of treatment of a patient with a rare disease – dysgenetic polycystosis of parotid glands, who was treated in the maxillofacial surgery department of the clinical dental hospital of the Federal State Budgetary Educational Institution of Higher Education “Perm State Medical University named after Academician E.A. Wagner” of the Ministry of Health of the Russian Federation.

Clinical Case

Patient S., 46 years old, came to the clinical dental hospital of PGMU with complaints of painless enlargement of the parotid salivary glands. According to the medical history, there were no previous cases of salivary gland disease in the family. About five years ago, the patient's relatives began to notice changes in his facial features; his face became rounder, and the lower third of his face became wider, although the patient himself did not notice any changes in his facial features. In 2021, against the background of acute respiratory viral infection, swelling appeared in the left parotid-masticatory region. There was no pain syndrome throughout the entire period of the disease. In the spring of 2024, during a medical examination, the patient underwent an ultrasound scan of the salivary glands, which revealed fluid formations in the parotid salivary glands on the right and left sides. No evidence of a neoplastic process was found after undergoing an MRI, cytological examination, and consultation with an oncologist. Laboratory test results (complete blood count, urinalysis, coagulogram, blood chemistry panel) were within normal limits, and a physical examination by a physician revealed no somatic pathology. He was admitted to the maxillofacial surgery department for incisional biopsy and diagnosis verification. External examination revealed no deterioration in general condition and no clinical changes in the body's organ systems at the time of his hospitalization. There was a symmetrical enlargement of the soft tissues in the parotid and masticatory regions and in the upper lateral parts of the neck on the right and left sides (Fig. 1).

 

Fig. 1. Appearance of patient C. upon admission

 

The skin on his face is unchanged in color, with no tissue infiltration. The parotid glands on the right and left sides are enlarged: on the left to 6.0 × 8.0 cm, and on the right to 7.0 × 6.0 cm. They are not visually contoured, are elastic in consistency on palpation, painless, and have a bumpy surface. An area of induration measuring 2.0 × 2.0 cm is detected in the posterior-inferior part of the parotid-masticatory region on the left. Mouth opening is not impaired. Oral cavity organs show no anatomical or functional abnormalities. No signs of salivation impairment were detected. The results of the instrumental examination were evaluated. Ultrasound conclusion dated June 24, 2024: multiple fluid formations in the projection of the parotid salivary glands. Cytological examination dated August 8, 2024: unstructured masses, no cellular elements detected. MRI dated July 23, 2024: enlargement of the parotid salivary glands with multiple cystic inclusions (Fig. 2).

 

Fig. 2. Axial MRI slice of patient C. The parotid glands on the right and left are enlarged in volume, with multiple cystic inclusions visible in their structure

 

Based on clinical data and additional examination methods, a preliminary diagnosis was established:

K11.6 – dysgenetic polycystosis of the parotid salivary glands. On November 26, 2024, surgery was performed: incisional biopsy of the left parotid salivary gland. The biopsy site was determined based on the presence of a hardened area in the posterior-inferior part of the parotid-masticatory region on the left side, as well as taking into account the lowest risk of complications (trauma to the facial nerve branches, formation of salivary fistulas) in the lower pole of the parotid gland. A 3.5 cm incision was made under endotracheal anesthesia in the projection of the lower pole of the parotid gland, bordering the angle of the lower jaw. The lower pole of the parotid gland was found in a capsule with multiple round cystic formations filled with viscous saliva (Fig. 3).

 

Fig. 3. Cystic formation of the lower pole of the parotid gland

 

Fig. 4. Biopsy material – the capsule of a cystic formation with contents and adjacent parenchyma of the parotid gland

 

The capsule is incised, revealing a cystic formation with a fragment of the surrounding gland parenchyma,measuring 2 × 2 × 2 cm. The specimen is sent for ante mortem pathological examination (Fig. 4).

Macroscopically, the specimen is a cyst measuring 3.0 × 2.5 × 2.2 cm. On sectioning, mucus is visible in the lumen, and the inner surface is grayish and smooth.

The surgical wound was sutured layer by layer with the creation of a duplicate parotid-masticatory fascia to prevent the formation of a salivary fistula.

 

Fig. 5. Microstructure of biopsy material, stained with hematoxylin and eosin, 40x magnification

 

In the postoperative period, the patient underwent a course of antibacterial therapy (ceftriaxone 1.0 once daily intramuscularly for 7 days). No purulent-inflammatory complications were observed, and the wound healed by primary tension.

The results: we got the conclusion of the ante-mortem pathological examination No. 1116 dated November 29, 2024 (Fig. 5).

Microscopic description of the preparation: the walls of the multicameral cyst are formed by connective tissue with vessels, moderately expressed lymphoplasmacytic infiltration, lined with cylindrical epithelium, in the adjacent lobules of the gland there is moderate fibrosis, pronounced lipomatosis, and focal lymphoid infiltration in the stroma. Pathological diagnosis: polycystosis of the salivary gland.

Thus, the preliminary diagnosis was confirmed histologically. The decision to proceed with conservative treatment was made based on the absence of complaints from patient C., the preservation of full salivary function, bilateral involvement of all sections of the parotid salivary glands, and the exclusion of a neoplastic nature of the disease according to biopsy data. It was recommended that the patient undergo annual check-ups with a dental surgeon, including MRI monitoring. In order to minimize the risk of developing inflammatory processes in the parotid salivary glands, a therapeutic and protective regimen is prescribed (adhering to a diet that excludes foods that increase salivation, as well as taking measures to strengthen the immune system).

The following recommendations regarding the treatment of dysgenetic polycystic ovary syndrome were developed based on the analysis of a clinical case:

1. If this disease is detected, the scope of surgical interventions should be limited to incisional biopsy for the purpose of verifying the diagnosis. Dynamic observation and prevention of purulent-inflammatory complications are preferred.
2. If there are a small number of cysts and they are large enough to be detected by palpation, it is possible to excise them along with the adjacent gland parenchyma.
3. The optimal site for obtaining material during incisional biopsy is the lower pole of the parotid gland, as this carries the lowest risk of trauma to the facial nerve branches and the formation of salivary fistulas in this area.

Conclusions

1. Dysgenetic polycystosis of the parotid salivary glands is a rare and poorly studied disease. The available information on the etiology, pathogenesis, and clinical picture of this pathology is scattered, and treatment tactics have not been standardized. Therefore, further research into the nosology is needed in order to improve differential diagnosis and enhance the quality of treatment.
2. The clinical case of polycystic parotid salivary glands described by us was confirmed histologically. The absence of functional disorders and the exclusion of a neoplastic process served as the basis for choosing conservative treatment.
3. The study may be useful for maxillofacial surgeons, as well as for dentists and therapists, for the purpose of earlier detection of patients with this disease, determination of rational treatment tactics, and timely registration for dispensary observation.

About the authors

Gleb I. Antakov

Ye.A. Vagner Perm State Medical University

Email: glebreanimator@inbox.ru
ORCID iD: 0000-0002-4949-3294

PhD (Medicine), Head of the Operating Unit of Clinical Dental Hospital, Maxillofacial Surgeon

Russian Federation, Perm

Danil A. Zotov

Ye.A. Vagner Perm State Medical University

Author for correspondence.
Email: dzotov.01@mail.ru
ORCID iD: 0009-0007-7622-0762

Resident of the Department of Surgical Dentistry and Maxillofacial Surgery

Russian Federation, Perm

Saida А. Sultanova

Academician E.A. Wagner Perm State Medical University

Email: saidasultanova1991@yandex.ru
ORCID iD: 0009-0009-4415-3694

Head of the Department of Maxillofacial Surgery, Maxillofacial Surgeon

Russian Federation, Perm

Svetlana I. Rapekta

Ye.A. Vagner Perm State Medical University

Email: rapsvi@mail.ru
ORCID iD: 0009-0005-9643-8473

PhD (Medicine), Associate Professor of the Department of Surgical Dentistry and Maxillofacial Surgery, Head of the Department of Surgical Dentistry and Maxillofacial Surgery

Russian Federation, Perm

References

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