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| CASE REPORT |
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| Year : 2019 | Volume
: 10
| Issue : 1 | Page : 39-41 |
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Intra parotid cystic lymphangioma masquerading as a neoplasm, a rare entity at unusual site
Nibedita Sahoo, Debahuti Mohapatra, Pranita Mohanty, Prateek Das, Debasmita Das
Department of Pathology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
| Date of Web Publication | 29-May-2019 |
Correspondence Address:
Dr. Debahuti Mohapatra
Department of Pathology, IMS and SUM Hospital, Bhubaneswar - 751 003, Odisha
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/mjmsr.mjmsr_39_18
Cystic hygroma is an uncommon congenital malformation and usually present as an asymptomatic, painless, soft, fluctuant mass in infancy, and early childhood. The neck is the most common site followed by the face, tongue, and floor of the mouth. The primary site at parotid gland is extremely unusual, though it can secondarily involves parotid gland. Cystic lesion in parotid brings the other differentials of Warthin tumor, low-grade mucoepidermoid carcinomas, and lymphoepithelial cyst. Fine-needle aspiration cytology is not always diagnostic, and histopathology is the confirmatory. Primary parotid cystic lymphangioma is rare and even rarer in the adult age group. Herein, we report a case of primary parotid cystic hygroma in a 24-year-old male, which was misdiagnosed in cytology and later confirmed by histopathology.
Keywords: Cystic lymphangioma, histopathology, parotid
How to cite this article:
Sahoo N, Mohapatra D, Mohanty P, Das P, Das D. Intra parotid cystic lymphangioma masquerading as a neoplasm, a rare entity at unusual site. Muller J Med Sci Res 2019;10:39-41 |
How to cite this URL:
Sahoo N, Mohapatra D, Mohanty P, Das P, Das D. Intra parotid cystic lymphangioma masquerading as a neoplasm, a rare entity at unusual site. Muller J Med Sci Res [serial online] 2019 [cited 2023 Jun 2];10:39-41. Available from: https://www.mjmsr.net/text.asp?2019/10/1/39/259253 |
| Introduction | |  |
Lymphangiomas are uncommon developmental malformations that arise from sequestrations of lymphatic tissue that fail to communicate normally with the lymphatic system. Although any anatomic site can be affected, the most common site is the head-and-neck region, due to the rich lymphatics at that site. The rest other anatomic sites affected are axilla, mediastinum, groin, retroperitoneal space, and pelvis.[1] Cystic lymphangioma (CL) is common in children of <2 years of age with no gender predilection.[2] Presentation in adult life is rare and even less commonly arises within the salivary gland. Till date, few cases of CL of the parotid have been described in the English literature. Herein, we report a rare occurrence of CL within the parotid gland of an adult male which caused a diagnostic dilemma in cytology and later confirmed by histopathology.
| Case Report | | |
A 24-year-old male presented with swelling near the right parotid region for 20 days, following trauma to that site. The lesion was of size 7 cm × 5 cm × 2 cm and was firm, nontender with restricted mobility. With this clinical background, he was advised for ultrasonography and fine-needle aspiration cytology of the lesion. Ultrasonogram of the neck revealed a well-defined heterogenous hypoechoic space occupying lesion with internal cystic spaces and low vascularity suggesting hemangioma. On aspiration, 5 mL of hemorrhagic fluid came out, and the swelling reduced in size after aspiration. Cytosmears revealed occasional salivary gland acini and few naked nuclei over the hemorrhagic background. No duct epithelial cells were seen [Figure 1]. In correlation with the clinico-radiological findings and the presence of a hemorrhagic background in cytosmears, a diagnosis of the benign vascular lesion was suggested. | Figure 1: Cytosmear showing occasional salivary gland acini over a hemorrhagic background containing occasional mature lymphocytes
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With this superficial parotidectomy was done, and the gross specimen measured of a size 4.7 cm × 4 cm × 1 cm which on cut section revealed a circumscribed solid cystic lesion of size 2.3 cm × 2 cm × 2 cm. The cyst was multiloculated with intervening septa showed the focal area of golden brown discoloration. The cysts varied of sizes ranging from 0.2 cm to 1.2 cm in diameter containing thick mucoid material to blood clot in some [Figure 2]. Microscopy showed a circumscribed lesion comprising cystic spaces of varying caliber lined by attenuated endothelium and filled with eosinophilic proteinaceous fluid-containing lymphocytes [Figure 3]. Few cysts contained old hemorrhagic fluid with hemosiderin-laden macrophages and few lymphocytes. Small lymphoid aggregates, few plasma cells, and fibroblasts were noted in the intervening stroma with unremarkable salivary gland acini and ducts. With these findings, a final diagnosis of intraparotid CL was made. | Figure 2: Superficial parotidectomy specimen showing a circumscribed solid and cystic lesion (arrow)
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 | Figure 3: (a) Lower magnification revealing dilated lymphatics and surrounding normal salivary gland acini (asterix), (b) Lower magnification showing intervening fibrous septa with lymphoid aggregates, (c) Higher magnification depicting lymphatic lined by flattened epithelium, (d) Lymphatics filled with hemorrhagic fluid with few hemosiderin-laden macrophages (inset)
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| Discussion | | |
Lymphangiomas are developmental malformation rather than true neoplasm. The lymphatic system arises from six primitive sacs that develop at the 6th week of intrauterine life. The first pair is jugular sac, the second pair is cisterna chyli located at retroperitoneal tissues, and the third pair is posterior lymph sacs develop at inguinal region. Aberrant proliferation of lymphatic vessels leads to these malformations with a common presentation as large soft-tissue mass. Depending on the histomorphology, lymphangiomas are classified as lymphangioma simplex, cavernous lymphangioma, and CL or cystic hygroma.[1] Though this system of classification do not have any prognostic significance.
Cystic hygroma usually present as slow growing, soft, fluctuant mass in infancy, and childhood, but occurrence in adulthood is rarely seen.[3] Acute-onset rapid enlargement of the mass is usually associated with trauma, infection, or hemorrhage into the cyst. Trauma is the probable cause of acute-onset swelling in our case.
Although posterior triangle of the neck is the most common location, it can occur in the tongue, face, and floor of the mouth and rarely involve the parotid gland. However, origin from parotid gland is extremely unusual and causes a diagnostic dilemma.
There are few case reports of parotid gland lymphangioma describing in cytology.[4],[5] The classical cytology smears of CL show predominantly mature lymphocytes admixed with cytologically bland spindle-shaped cells against a proteinaceous background. In this case, the hemorrhagic background along with few bare nuclei, misleaded to a diagnosis of vascular malformation in correlation with the radiological findings. All these changes are probably due to trauma-induced hemorrhage into the cyst.
Cystic lesions within the parotid are uncommon, and clinically, they are misdiagnosed as tumors. During dealing with cystic lesions of the parotid gland, the various differentials considered are neoplastic lesions such as Warthin tumor, cystic pleomorphic adenoma, cystic low-grade mucoepidermoid carcinoma, acinic cell carcinoma, and nonneoplastic lesions such as retention cyst, branchial cleft cyst, benign lymphoepithelial lesion, and rarely congenital malformation like polycystic disease of the parotid gland. Proper history, clinical examination, and radiological findings can help to rule out the other differentials and the final diagnosis will be reached after surgical excision and histopathological examination.
Sometimes, secondary hemorrhage into the cyst confuses with cavernous hemangioma as in our case, but the presence of lymphoid aggregates in the stroma with more irregular lumen of the cyst favors the diagnosis of CL. In addition, the immunohistochemical markers for lymphatic lineage such as vascular endothelial growth factor receptor 3, D2-40 add to the diagnosis.[6]
Complete excision of the cyst is the mainstay of treatment; however, if inadequate excision occurs the rate of recurrence ranges from 10% to 38%.[7] In some cases, treatment with sclerosing agents such as OK-432 and bleomycin is also used with varying results.[8],[9] Good response to sclerosants is seen in the head-and-neck location, size <5 cm and macrocystic architecture. In our case, the patient had undergone superficial parotidectomy and doing well in his 3 months of follow-up visit with no sign of recurrence.
| Conclusion | | |
Although rare, CL should be considered as a differential in dealing with cystic lesions of the salivary gland. Although trauma alters the classic morphology of lymphangioma in cytology, biopsy and histopathology confirm the diagnosis.
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 initials 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 | | |
| 1. | Kandakure VT, Thakur GV, Thote AR, Kausar AJ. Cervical lymphangioma in adult. Int J Otorhinolaryngol Clin 2012,4:147-50.  |
| 2. | Guruprasad Y, Chauhan DS. Cervical cystic hygroma. J Maxillofac Oral Surg 2012;11:333-6. |
| 3. | Naidu SI, McCalla MR. Lymphatic malformations of the head and neck in adults: A case report and review of the literature. Ann Otol Rhinol Laryngol 2004;113:218-22. |
| 4. | Henke AC, Cooley ML, Hughes JH, Timmerman TG. Fine-needle aspiration cytology of lymphangioma of the parotid gland in an adult. Diagn Cytopathol 2001;24:126-8. |
| 5. | Gutmann EJ. Lymphangioma presenting as a primary parotid neoplasm in an adult. Report of a case with the diagnosis suggested by fine needle aspiration biopsy. Acta Cytol 1994;38:747-50. |
| 6. | Fukunaga M. Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours. Histopathology 2005;46:396-402. |
| 7. | Shah GH, Deshpande MD. Lymphatic malformation in adult patient: A rare case. J Maxillofac Oral Surg 2010;9:284-8. |
| 8. | Sichel JY, Udassin R, Gozal D, Koplewitz BZ, Dano I, Eliashar R, et al. OK-432 therapy for cervical lymphangioma. Laryngoscope 2004;114:1805-9. |
| 9. | Banieghbal B, Davies MR. Guidelines for the successful treatment of lymphangioma with OK-432. Eur J Pediatr Surg 2003;13:103-7. |
[Figure 1], [Figure 2], [Figure 3]
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