PILOMATRIXOMA OF FACE PRESENTING AS PAROTID SWELLING

Pilomatrixoma, also known as pilomatrixoma or calcifying epithelioma of Malherbe, is a benign tumor of skin appendages. Although it is an uncommon and benign neoplasm of the head and neck region, it may sometimes become malignant. It arises from the cells of the outer sheath of the hair follicle root. Owing to its rarity, pilomatrixoma can be missed during differential diagnosis, and it can be misdiagnosed as malignancy. Therefore, for definitive and confirmatory diagnosis, histopathological analysis is imperative. We report a case of a 26-year-old female who presented with hard swelling on the right side of her face near the angle of the mandible. We have discussed its clinical, radiological, and histological findings and reflected light on the treatment of choice for pilomatrixoma.

Case report of malignant peripheral nerve sheath tumor of parotid in a patient with neurofibromatosis type 1

<p>Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, accounts for &lt;5% of all soft tissue sarcomas, and carries a poor prognosis. Diagnosis of MPNSTs is usually based on histopathology aided by IHC, which reflects the Schwann cell differentiation in this neoplasm. Approximately 50-90% of MPNSTs are positive for S-100 protein. A 56-year-old male presented with right parotid swelling for 3 months and incomplete right eye closure. The patient was diagnosed with neurofibromatosis 1 (NF-1) and pulmonary tuberculosis in the past. On Examination, a hard, tender swelling measuring 5×4 cm involving the right parotid gland and right grade 2 LMN facial palsy was noted. Histopathology was suggestive of a MPNST.</p><p><strong> </strong></p>

P046 A review and retrospective case series of paediatric Sjögren’s syndrome from Southern Africa

Background Paediatric Sjögren’s syndrome (pSS) is an uncommon autoimmune paediatric disease, rarely reported in children in Africa. It remains an important consideration in a child with parotid swelling. Here, we present a retrospective case-series of four patients from a paediatric rheumatology clinic in South Africa and discuss some of the difficulties of diagnosis in our context. Methods We performed a retrospective analysis of patients who attend the Red Cross War Memorial Hospital Paediatric Rheumatology clinic between 2010 and 2019. Result The four patients diagnosed with Sjögren’s all had varied presentations and disease courses. There were 3 females and 1 male and the ages at diagnosis ranged from 6 to 19 years old. The time to diagnosis was prolonged and ranged from 1 month to 10 years. Two patients with primary pSS presented with extra-articular manifestations of arthritis, abdominal pain and fatigue, followed by dry mouth, dry eyes, parotid swelling and and suggestive histopathology. The remaining 2 patients had a secondary pSS due to juvenile onset SLE and tuberculosis respectively. Conclusion In less resourced settings the diagnosis of pSS is often delayed. Both patients with primary pSS had preceding extra-glandular manifestations. In less resourced settings, infectious diseases may present as pSS and associated rheumatic diseases causing secondary pSS must be considered.

O20 Approach to parotid swelling

Case report - Introduction The rarity of paediatric salivary gland disease and the lack of pathognomonic signs are likely to contribute to delay in diagnosis and make a structured approach to parotid swelling particularly important. Here we discuss a case of a 4-year-old girl with recurrent parotid swelling and some features to suggest multisystem involvement. Case report - Case description A partially immunised, 4-year-old girl with a history of eczema and vitiligo presented with a 3-month history of intermittent painless pre-auricular swelling. She had 2 days of fever at onset but was otherwise afebrile. She reported intermittent joint pain without swelling. There was no cough, coryza, sore throat, dryness of the mouth or eyes, rash, systemic upset, and no other evidence of multisystem involvement on systems review. She had no unwell contacts and no family history of autoimmune disease. On examination she had bilateral pre-auricular swelling which was non-tender, not fluctuant, and had no overlying skin changes. No calculi were identified on bimanual palpation of the parotid ducts and no pus was visible at the opening. Respiratory, cardiovascular, abdominal, musculoskeletal, and skin examination were unremarkable. Her bloods showed microcytic anaemia, raised ESR (peak 97mm/hr), CRP (peak 30mg/l), CK (peak 628 IU/l), and LDH (peak 512 IU/l), but normal ferritin and ACE. Serology showed ANA 1/10240, RNP Ab positive, negative myositis specific ENA, dsDNA, and rheumatoid factor, and normal complement. Infection screen, including TSpot, and screening for immunodeficiency were negative. Her urine dipstick was normal. Interferon signature was abnormal with very high levels. Parotid ultrasound scan showed heterogenous enlargement of all major salivary glands and lacrimal glands with reactive cervical lymph nodes. CT chest demonstrated basal ground-glass change and hilar, mediastinal, and axillary lymphadenopathy. Parotid biopsy was normal on two occasions, with no evidence of lymphoma or granulomatous disease. At this stage she was treated for undifferentiated autoimmune connective tissue disease with steroids and mycophenolate mofetil. Due to the lack of improvement and persistently mildly elevated CK and LDH, MRI thighs was subsequently performed. This demonstrated mild myositis prompting a muscle biopsy which showed typical features of juvenile dermatomyositis. She was therefore commenced on intravenous immunoglobulin and subcutaneous methotrexate. Case report - Discussion This is an unusual presentation of juvenile dermatomyositis with no typical clinical features of skin or muscle involvement, negative myositis ENA, and only mildly elevated CK, where parotitis was the main presenting feature. Despite a dramatic reduction in mumps following routine immunisation, viral adenitis remains the most common cause of parotid swelling. The most prominent features are unilateral or bilateral parotid swelling with fever and headache and, unlike this case, typically resolve in 1—2 weeks. Bacterial adenitis may be suggested by erythema and purulent secretions and may be precipitated by preceding viral sialadenitis, dehydration, or damage by calculi. Obstruction and inflammation caused by calculi, sialolithiasis, are suggested by pain with meals and almost complete resolution in between. A single short episode of parotid swelling without multisystem involvement will most commonly be caused by one of the above and would not require extensive investigation. Management consists of one or more of: ensuring adequate hydration, warm compresses, analgesia, salivary gland massage, sialagogues, antibiotics, and safety netting advice. More prolonged painful swelling despite sialagogues and antibiotics would warrant sialography to look for calculi, and prolonged painless swelling, particularly with the presence of any red flags, should prompt investigation for tumours or haematological malignancy. Only in recurrent, treatment refractory episodes, or, in cases such as this, where a thorough history and examination suggest multisystem involvement, would more extensive investigation be warranted to look for inflammatory causes of parotitis. Case report - Key learning points The differential diagnosis for parotid swelling is broad so a structured approach is essential. Extent of investigation should be guided by the history, particularly the clinical course and the evidence of multisystem involvement. It may be reasonable not to investigate at all in cases of isolated resolved or resolving parotid gland swelling without systemic upset and normal systems review. Where there are features to suggest multisystem disease, early referral to rheumatology for further investigation and treatment is needed.

Profile of parotid cancers at the ENT clinic Lamine Sine Diop of Fann teaching hospital

INTRODUCTION: Parotid cancers are characterized by a great histological diversity and they pose diagnostic, therapeutic and evolutionary problems. We deliver through this study our experience on the management of malignant parotid tumors. MATERIALS AND METHODS: This is a retrospective, descriptive and analytical study carried out over a period of 12 years in the ENT department of Fann teaching hospital. RESULTS: The mean age at diagnosis was 48 years with a sex-ratio of 1.6. The time to symptom progression was approximately 47 months. Parotid swelling was present in all patients and peripheral facial palsy was found in 31% of patients. Ultrasound of the parotid region was performed in 11 patients, i.e. 34%, and computed tomography in 18 patients, i.e. 56%. Fine needle aspiration was performed in 11 patients. Parotid cancers accounted for 44% of all parotid tumors. Sixty-eight percent of patients consulted at the T4 stage. Seventy-one percent of patients received surgical treatment. The most common histologic type was muco-epidermoid carcinoma. Five patients received radiotherapy. The postoperative effects were dominated by PFP (18%). Seven cases of death were recorded. CONCLUSION: The management of parotid cancer still poses diagnostic and above all therapeutic problems. The combination of radiotherapy surgery, very rarely encountered in our study, provides a better prognosis and better survival.

Transoral per punctum excision of an impacted parotid duct stone of unfavorable direction: a case report

Background In the literature, the reported parotid duct stones that did not respond to the conservative measures are few. The present work reported and described the diagnosis and treatment of a case of impacted parotid duct stone that was surgically removed through minimally invasive transoral per punctum approach. Case presentation On examination of a 27-year-old male with left intermittent parotid swelling and pain for 1 year, a stone was palpated in the left buccal mucosa region near the orifice of the parotid duct. Ultrasonography (US) showed left Stensen’s duct distal stone. Under general anesthesia and oral intubation, the stone measuring about 1 cm in length was felt transversely located (directed at a right angle with the orifice of the duct). A small mucosal incision was done at the upper lateral part of the punctum; pus came out, and then the stone bulged from the orifice and was pushed outside. Recovery was event-less; postoperative paracetamol was sufficient to relieve pain, and the patient was discharged a few hours after the surgery. Conclusion The transoral per punctum excision of an impacted parotid duct stone appears to be a reliable minimally invasive effective and safe procedure. The unfavorable direction of the parotid duct stone might be a cause of failure of the conservative treatment for this stone.