Haemangioendotheliomas (HAE), although rare but are the most common parotid gland tumours in children. We report a 4-month-old girl who presented with a progressively enlarging right sided facial swelling overlying the angle of the mandible. An Ultrasound of the lesion and a computed tomography (CT) scan of the head and neck was carried out which revealed a large lesion within the right parotid gland. CT scan further demonstrated a direct communication with the right external carotid artery and external jugular vein. Considering the clinical course and radiological findings, there was sufficient evidence to avoid any invasive testing. Due to the self-limiting nature of the disease, patient was managed expectantly

Ansari, Mohammad Ahmed

Aziz, Abeer

Baig, Mirza Zain

Khan, Muhammad Arif Mateen

Saleem, Areej

English

eCommons@AKU

eCommons@AKU Medical College Documents Medical College, Pakistan 4-2020 Infantile haemangioendothelioma of the parotid gland: Case report and review of literature Mirza Zain Baig Aga Khan University Mohammad Ahmed Ansari Aga Khan University Abeer Aziz Aga Khan University Areej Saleem Aga Khan University, areej.salim@aku.edu Muhammad Arif Mateen Khan Aga Khan University, arif.mateen@aku.edu Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_mc  Part of the Pediatrics Commons, and the Surgery Commons Recommended Citation Baig, M. Z., Ansari, M. A., Aziz, A., Saleem, A., Khan, M. (2020). Infantile haemangioendothelioma of the parotid gland: Case report and review of literature. JPMA. The Journal of the Pakistan Medical Association, 70(4), 776-779. Available at: https://ecommons.aku.edu/pakistan_fhs_mc_mc/123 Abstract Haemangioendotheliomas (HAE), although rare but are the most common parotid gland tumours in children. We report a 4-month-old girl who presented with a progressively enlarging right sided facial swelling overlying the angle of the mandible. An Ultrasound of the lesion and a computed tomography (CT) scan of the head and neck was carried out which revealed a large lesion within the right parotid gland. CT scan further demonstrated a direct communication with the right external carotid artery and external jugular vein. Considering the clinical course and radiological findings, there was sufficient evidence to avoid any invasive testing. Due to the self-limiting nature of the disease, patient was managed expectantly. Keywords: Parotid gland, Haemangioendothelioma, Haemangioma. https://doi.org/10.5455/JPMA.47901 Introduction Haemangioendotheliomas (HAE), although rare but are the most common parotid gland tumours in children.1-7 They constitute 1-5% of all salivary gland neoplasms.8 These present in infancy as a painless large rapidly growing mass.4 It is a benign and self-limiting condition.4,5 It is therefore of paramount importance that early and accurate diagnosis be made to avoid invasive procedures and alleviate undue anxiety among the caretakers. We report the case of a infantile haemangioendothelioma of the parotid gland that was managed conservatively at our centre. Although, there have been previous case reports on infantile haemangioendotheliomas,1-10 this is the first time such a case is coming to light from our part of the world. Prior permission from the patient's guardians was acquired before the preparation of this manuscript. Case Report A 4-month-old girl was brought to the paediatric surgery outpatient clinic on the 22nd of July 2018, with a progressively enlarging right sided facial swelling. Swelling was first noticed at one month age by her parents.  It was located at the right side of angle of the mandible and had no overlying erythema or discharge. There was no demonstrable tenderness on palpation. Parents denied any presence of fever, signs of infection or feeding issues and the patient was otherwise healthy with adequate growth and no developmental delay. Patient was born to non-consanguineous parents. She was delivered spontaneously at term without any natal or peri-natal complications. She had no relevant past medical, family or genetic history. Investigations ordered showed a haemoglobin (Hb) of 10.5 G/dl, (normal: 9 - 11G a total leukocyte count (TLC) of 14000x109 (N; 5-10,000) and platelets of Vol. 70, No. 4, April 2020776STUDENTS’ CORNER CASE REPORT Infantile haemangioendothelioma of the parotid gland: Case report and review of literature Mirza Zain Baig,1 Mohammad Ahmed Ansari,2 Abeer Aziz,3 Areej Salim,4 Muhammad Arif Mateen Khan51-35th Year MBBS Student, Medical College, Aga Khan University Hospital, Karachi, 4,5Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan. Correspondence: Mirza Zain Baig. Email: mzainbaig@gmail.comFigure-1: Ultrasound demonstrating internal vascularity in a large parotid swelling.307000x109 (normal: 150-250,000). Ultrasound demonstrated multiple echogenic scattered nodules in the right parotid gland with markedly increased internal vascularity suggestive of a haemangioma. In the light of the above findings a computed tomography (CT) scan of the head and neck was ordered and it demonstrated a large lobulated and vividly enhancing lesion within the right parotid gland measuring 41x43x36mm having a direct communication with the right external carotid artery and external jugular vein. These findings were consistent with a parotid haemangioendothelioma. Parents were reassured regarding the benign nature of this condition. Patient underwent no further investigations and no therapeutic intervention was deemed necessary. Periodic surveillance for the patient was decided and upon her visit at the outpatient department a month later, no change was noted. Parents were advised to follow up if there were any further development or if any emergent findings were evident. Discussion Haemangioendotheliomas (HAE) are neoplastic growths of vascular endothelial cells.11 Juvenile haemangiomas may occur on any part of the body but most commonly occur in the head and neck region particularly, the parotid gland.6 These are benign in nature and most cases of HAE appear in the early months of the life.5 Median age of the presentation is around 4 months.1,3,4 There is a female predominance with a 3:1 ratio compared to males.3,4,8 Bluish/purplish discolouration of the overlying skin is a suggestive clinical sign.1,8 The diagnosis may also be supported by a cutaneous strawberry haemangioma either locally or elsewhere.5,8 Infantile parotid haemangiomas have a natural history that includes rapid growth during infancy followed by gradual involution at around 8-18 months.1,4 They are usually not noticeable in the neonatal period but become prominent in the first few months of life.1,5 Characteristic imaging findings aid diagnosis so as to avoid invasive biopsies in the infants. Sonographic findings include a homogenous mass arising in the parotid gland with a lobular structure, fine echogenic internal structure and a lobulated contour.7,8 The presence of large blood vessels within the tumour is highly suggestive.4 CT scan shows a soft tissue mass that enhances with contrast material. On MRI these lesions appear isointense when compared to muscle on T1-weighted MRI and they show hyperintense or intermediate signal on T2-weighted MRI. The lesions exhibit homogenous contrast enhancement on MRI.11 MRI is the best imaging modality.1,3 J Pak Med Assoc777 M. Z. Baig, M. A. Ansari, A. Aziz, et alFigure-2: Head CT showing a large right parotid swelling with intense contrast uptake demonstrating vascularity.Figure-3: Coronal CT Sequence demonstrating communication of Right Parotid mass with internal carotid artery.Red cell scintigraphy can diagnose head and neck haemangiomas with very high accuracy. It shows uniform, well defined uptake similar to the heart and great vessels.4 We came across three cases that reported an association between infantile parotid haemangioendothelioma and a cytomegalovirus (CMV) infection.2,6,10 This suggests that there might be some aetiological role of CMV in this pathology. However, it is equally possible that these findings may have been purely incidental.  Since, many tumours regress on their own, no active management is required. Surgery is not recommended due to the risk of damage to the underlying facial nerve.4,8 There is reported success with oral propranolol, it has been proven an effective, safe and well tolerated treatment option.1,9,12 Propranolol facilitates vasoconstriction, decreases expression of vascular growth factors and activates apoptosis of capillary endothelium cells.1 Minor side effects include agitated sleep, diarrhoea and hypoglycaemia.12 These are all easily controlled.12 Rare but significant systemic complications of parotid HAE include cardiac failure and Kasabach Merrit syndrome.4,5 Kasabach Merrit syndrome involves a vascular tumour and developing subsequent thrombocytopenia. Treatment of choice in such cases are oral steroids and interferon alpha.4,5 Our patient had normal platelet levels, as previously mentioned and so was not currently at risk of developing such a condition. The Table, summarises case reports published in the last 10 years that were accessible to us as full text English articles. Conclusion Suggestive clinical course and characteristic findings on radiology provide sufficient evidence for confident diagnosis of infantile haemangioendotheliomas of the parotid gland. There is no need for invasive biopsy procedures. Prognostically the disease shows resolution with no medical or surgical intervention. Disclaimer: None to declare. Conflict of Interest: None to declare. Funding Disclosure: None to declare. References 1. Chaubal R, Tavri O, Sawant A, Singh C. Infantile Vol. 70, No. 4, April 2020Infantile haemangioendothelioma of the parotid gland: Case report and review of literature 778Table: Case reports on infantile haemangioendothelioma of the parotid gland, published in the last 10 years that were accessible as full text, English articles.  Author                                                                 Clinical features                                                                                                                   Imaging findings  Chaubal et al, 20174    John et al, 20168      Chatura et al, 20152      Kotrashetti et al, 20151   Tandon et al, 20117    Tuna et al, 20093 4 month old infant with painless swelling on left angle of mandible   3 month old female with rapidly enlarging left parotid swelling and cutaneous strawberry haemangioma on the face    5 month old male with 2 month history of right facial swelling. Parotid resected due to suspicion of cystic hygroma. Pathological findings suggested of arteriovenous haemangioma associated with CMV infection Two and a half month old baby boy with 15 days history of left parotid swelling that was gradually increasing in size 3 month old male with right sided swelling on the face. Swelling gradually increasing in size. Reddish patch on right side of face present since birth (cutaneous strawberry haemangioma). 4 month old infant in 2 month history of right neck swellingUSG: Homogenous, isoechoic lesion with lobulated margins replacing parotid gland. Few echogenic septae seen Colour Doppler showed multiple dilated vascular channels MRI: Homogenous contrast enhancement  USG: Enlarged parotid gland with hypoechoic and lobulated contours. Multiple large anechoic vascular channels noted within. Colour Doppler showed high density of blood vessels within. CT: Non contrast images showed diffuse enlargement of superficial and deep lobes of parotid with lobulated contour. Post contrast images showed intense enhancement of the entire left parotid gland. USG: Large lobulated tumour      USG: Enlarged parotid (3.1 x 2.2 x 2.5 cm) with increased vascularity MRI: well-defined, lobulated homogenously enhancing lesion diffusely involving the superficial and deep layers of the parotid gland USG: Large lobulated mass with echogenic septations replacing the right parotid gland Colour Doppler showed multiple intratumoral vessels with both arterial and venous components  MRI: lobulated mass with flow voids replacing superficial and deep lobes of right parotid gland. The lesion is isotense to muscle on T1 and hyperintense on T2. USG: Colour Doppler showed enlarged and heterogenous right parotid gland MRI: Hyperintense right parotid mass containing vascular flow voids on T2.Hemangioendothelioma of the Parotid Gland. J Clin Imaging Sci. 2017; 7:5 2. Chatura K, Iqbal M. Parotid gland hemangioma with cytomegalovirus infection. J Adv Clin Res Insights. 2015; 2:31-2. 3. Tuna İ, Doganay S, Yıkılmaz A, Coskun A. Hemangioma of the Parotid Gland in an Infant: MR and Doppler US Findings. Eurasian J Med. 2009; 41:141. 4. Chaubal R, Tavri O, Sawant A, Singh C. Infantile Hemangioendothelioma of the Parotid Gland. Am J Surg. 1958; 96:784-6. 5. Roebuck D, Ahuja A. Hemangioendothelioma of the Parotid Gland in Infants: Sonography and Correlative MR Imaging. AJNR Am J Neuroradiol. 2000; 21:219-23. 6. Horie Y, Kato M. Juvenile hemangioma (infantile hemangioendothelioma) of the parotid gland associated with cytomegalovirus infection. Pathol Int. 1999; 49:668-71. 7. Tandon A, Jain B, Tandon R, Grover C, Pandhi D, Bhatt S. Infantile Hemangioendothelioma of the Parotid Gland: A Sonographic Diagnosis. J Diagn Med Sonogr. 2011; 27:131-4. 8. John D, Shingade R, Patel J. A Case of Parotid Hemangioma of Infancy: Role of Ultrasound and Doppler in the Diagnosis. Ind J Neonatal Med Res. 2016; 4:10-2. 9. Morais P, Magina S, Mateus M, Trindade E, Jesus J, Azevedo F. Efficacy and safety of propranolol in the treatment of parotid hemangioma. Cutan Ocul Toxicol. 2011; 30:245-8. 10. Ayadi L, Khabir A, Boudawara T, Kharrat M, Makni S, Jlidi R. Parotid cell hemangioma associated with cytomegalovirus infection. Pathol Int. 1999; 49:668-71. 11. Guneyli S, Ceylan N, Bayraktaroglu S, Acar T, Savas R. Imaging findings of vascular lesions in the head and neck. Diagn Interv Radiol. 2014; 20: 432-7. 12. Zheng J. Comment on efficacy and safety of propranolol in the treatment of parotid hemangioma. Cutan Ocul Toxicol. 2011; 30:333-4. J Pak Med Assoc779 M. Z. Baig, M. A. Ansari, A. Aziz, et al

Infantile haemangioendothelioma of the parotid gland: Case report and review of literature

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Infantile haemangioendothelioma of the parotid gland: Case report and review of literature

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