Cervical cystic lymphangioma in an adult, diagnosed on FNAC

Sir,

A 30-year-old female presented in the Department of Surgery with a slowly enlarging mass on the left side of her neck of four-month duration. She noticed mild local discomfort and pain while moving her head and left arm. She did not have any obstructive symptoms like dysphagia, dyspnoea or any history of trauma or recent upper respiratory tract infection. Physical examination revealed a single, soft to cystic, fluctuant, mobile swelling measuring 6 × 4 cm in the left supraclavicular fossa, just above the medial one-third of the left clavicle.

Suspecting the swelling to be a lipoma, the patient was referred to the cytopathology department where fine-needle aspiration cytology (FNAC) of the swelling was performed. Thirty millilitres of clear, straw-colored fluid was aspirated, which reduced the swelling considerably. Smears prepared from the centrifuged deposits were both air-dried and alcohol-fixed. Air-dried smears were stained by May-Grünwald-Giemsa stain while alcohol-fixed smears were stained with Papanicolaou and hematoxylin and eosin stains. Smears showed only mature lymphocytes, few of which were degenerated, in a pink proteinaceous background [Figure 1, inset]. The swelling was reported as benign cystic lesion, most probably cystic lymphangioma (CL).

Figure 1.

Cystic spaces lined by endothelial cells with aggregates of mature lymphocytes (H and E, ×200). Inset: mature lymphocytes in an amorphous background (MGG, ×400)

Ultrasonography was performed and the findings supported the cytological diagnosis of CL. Excision biopsy was undertaken and a large, thick-walled cyst was removed. Microscopy demonstrated cystic spaces lined by endothelial cells with aggregates of mature lymphocytes [Figure 1].

The excision was reported as complete and there was no evidence of recurrence till the final review of the case.

Lymphangiomas are classified as lymphangioma simplex, cavernous lymphangioma and CL or cystic hygroma.[1] CL is defined as a benign proliferation of lymphoid tissue and is usually considered as a congenital lesion. It is well recognised in paediatric practice but seldom presents de novo in adulthood, with less than 100 adult cases of lymphangiomas having been reported in the English language literature.[2]

The most common sites are in the posterior triangle of the neck (75%), axilla (20%), mediastinum (5%), groin, retroperitoneal space and pelvis. They are most often seen in infancy and, in 80% of cases, within the first two years of life and rarely in adults.[3] In adults, lymphangiomas may occur either spontaneously or in response to infection or trauma.

Majority of cervical lymphangiomas in adulthood are asymptomatic, have no gender predilection and present as a painless mass that enlarges progressively. The lesion is soft, nontender, transilluminant, fluctuant and fixed to deep tissues, similar to pediatric presentation.[4,5]

There is no consensus in the literature concerning the use of FNAC to diagnose these lesions. However, in this case, FNAC proved to be an important diagnostic tool. FNAC shows small and round lymphocytes with intermingling histiocytes without mitoses or atypical cells. Histopathologically, endothelial-lined lymphatic spaces are seen with intervening fibrous tissue and lymphoid aggregates.[4] Various differential diagnoses of CL, viz. thymic cyst, pericardial cyst, bronchogenic cyst, cystic teratoma and cystic thymoma, should be kept in mind.[1]

The treatment of choice is surgical excision and there is a 15% recurrence rate if the lesion is not fully excised. A study however recommends a two-year postsurgical follow-up to detect any recurrence.[5]

The authors conclude that lymphangiomas in adults are uncommon lesions and a rare cause of cervical region masses. The case posed a diagnostic challenge as cervical CL presented de novo in an adult with no history of trauma or upper respiratory tract infection. The lesion was completely excised with no evidence of recurrence. Further, we would like to emphasise the importance of preoperative FNAC and imaging in the management of these lesions.