An 18-year-old girl presented with a painless swelling of the midline chest for 2 years, which gradually increased in size. Physical examination revealed a soft, fluctuant, non-pulsatile, non-tender swelling measuring approximately 9 x 8 cm over the manubrium sternum.
Contrast-enhanced CT of the chest showed a well-defined, non-enhancing, intramedullary, expansile lytic lesion with internal septations and a narrow zone of transition involving the manubrium sternum. Few areas of cortical break were seen with no soft tissue component. No matrix mineralisation/periosteal reaction was seen.
FDG PET CT showed no uptake within the lesion.
Background
Lymphangiomas, which are rare, benign malformations of the lymphatic system, are typically found in children [1,2]. Their occurrence in the chest wall is quite uncommon, as they typically occur in the cervicofacial and axillary regions [1,3].
Pathogenesis
The lymphatic system is vital for maintaining circulation and organ perfusion balance. During embryonic development, six primary lymph sacs are formed, which later connect with lymphatic channels from different parts of the body. The failure of the primary lymphatic sac to establish proper drainage into the venous system results in the enlargement of lymphatic vessels and the development of cystic structures known as lymphangiomas [3].
Lymphangiomas can manifest as various types, including macro cystic, microcystic and mixed types, and can morphologically be classified as cystic, simple (capillary) and cavernous [4,5]. They are benign swellings and do not undergo malignant change [5].
Clinical Perspective
Complete surgical resection is the preferred treatment, with prognosis depending upon the extent of resectability. Recurrence is unlikely with total removal of the lesion. Chemical drug sclerosis serves as an alternative only when surgery is not feasible, with full recovery achievable upon complete tumour removal.
Imaging Perspective
Ultrasound examination reveals cystic masses with smooth or irregular walls, appearing as uni- or multilocular structures [6]. Doppler ultrasonography should be done to detect any vascular component of the cystic lymphangioma [1]. CT and MR scans show variable enhancement of the cyst wall, with fluid density, ranging from -4 to 34 HU on CT depending on lipid content and haemorrhage presence. The cysts appear isointense to muscle on T1-weighted and hyperintense to fat on T2-weighted MR images. MR imaging provides a clearer delineation of tumour extension compared to ultrasound and CT. All three imaging modalities—ultrasound, CT, and MR—are valuable for lymphangioma evaluation, with MRI particularly useful for precise lesion extension assessment [3,6]. FDG PET CT usually do not show any uptake in lymphangioma, unless there is a superadded infection [7].
Histopathology Features
Typically, lymphangioma shows large, irregular, dilated cystic lymph spaces lined by single layers of endothelium within a fibrous or collagenous stroma [5]. Immunohistochemical markers such as D2‐40 and lymphatic vessel endothelial receptor‐1 (LYVE‐1) are diagnostic [8].
Our patient underwent wide local excision of the manubrial lesion. Histopathology showed the presence of bony trabeculae, thin-walled spaces filled with loose fibromyxoid tissue containing stellate cells and scattered endothelial-lined empty vascular spaces containing serous fluid, favouring a lymphangioma.
Lymphangioma
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Based on the provided chest CT and pathological image data, a relatively large low-density cystic lesion can be observed in the upper part of the sternum (sternal manubrium) region, with relatively well-defined boundaries. The specific findings are as follows:
Based on the patient’s age, clinical presentation (relatively slow growth, painless), and imaging characteristics (cystic lesion located in the sternal manubrium), the following differential diagnoses can be considered:
Considering the patient is an 18-year-old female with a slow-growing, painless cystic lesion in the region of the sternal manubrium, alongside postoperative pathology showing thin-walled vascular/lymphatic dilation and immunohistochemical findings consistent with a lymphatic origin, the most likely diagnosis is “Lymphangioma.”
After surgery or sclerotherapy, patients can begin gradual rehabilitation exercises to restore chest wall mobility and improve overall fitness. The following general recommendations are based on the FITT-VP principle:
During rehabilitation, special attention should be paid to the healing of the sternum and surgical incision to avoid excessive strain or external impact. If notable chest pain or discomfort occurs, discontinue the related exercise and consult a physician or rehabilitation therapist.
This report is a reference-based medical analysis derived from the currently provided clinical history and imaging data. It cannot fully replace in-person consultations or professional diagnoses and treatments by a physician. If you have any health concerns, please consult a qualified medical professional or visit a reputable medical institution.
Lymphangioma
