We present the case of a 34-year-old African woman, to our observation as outpatient, for a soft and palpable neck mass to be investigated with MR in view of an eventual surgical removal. Her clinical past history was irrelevant.
A previous neck ultrasound revealed a large fluid mass on left supraclavicular region, and its cytologic fluid analysis resulted in some white blood cells and chyle.
MR imaging of the neck confirmed large multicystic fluid-filled masses, presenting as multiseptated fluid collections without diffusion restriction, on both sides (Fig. 1, 2).
Besides, MR demonstrated multiple and focal bone lesions, in particular on several ribs, sternum and several vertebral bodies. These lesions presented variable signal intensity, appearing mainly hypo- to isointense on T1-weighted and hyperintense on T2-weighted images (Fig. 3).
On the suspicion of systemic lymphangiomatosis, an abdominal and lung ultrasound (US) was performed some days after the MR examination.
US examination revealed multiple cysts of the spleen, the largest about 25 mm, in addition to this no further changes in abdominal organs nor pleural effusion were found.
Lymphangiomatosis is a rare condition characterised by a diffuse proliferation of lymphatic vessels. It is most common in children and young adults. Symptoms and prognosis vary greatly between individuals and according to the organs involved [1, 2].
The majority of patients have both visceral and skeletal involvement, most commonly in the spleen, liver, lung, long bones, pelvis, skull, and vertebrae. Although this condition is not generally thought to metastasize, the lesions may be extremely destructive locally.
General radiologic findings may include massive chylothorax, pulmonary interstitial infiltrates, pleural effusion, splenomegaly, splenic cysts, and lytic bone lesions [1, 2].
The skeletal changes frequently consist of multiple, rounded, cyst-like lesions, found particularly in the pelvis and upper femur, however, lesions in the spine are not uncommon [3]. In this context, MR can be helpful in suggesting the correct diagnosis, as in our case.
The diagnosis of lymphangiomatosis is made through clinical suspicion combined with radiologic findings. The coexistence of lytic bone lesions and chylothorax supports the diagnosis of lymphangiomatosis.
Systemic lymphangiomatosis
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Based on the provided MRI images, there is a relatively large cystic lesion in the cervical and upper thoracic region with fairly clear borders. On T2-weighted sequences, it appears hyperintense, and on some T1-weighted images, it is hypointense or isointense, suggesting that the contents may be lymphatic fluid or fluid with a high protein content.
In the corresponding segments of the cervical and thoracic spine, there are varying degrees of vertebral or paravertebral lesions, presenting as multiple small cystic changes. Some areas exhibit internal septations or multi-chambered structures without obvious signs of soft tissue destruction, vertebral collapse, or aggressive tumor invasion.
In the soft tissue layers of the neck, the palpable mass corresponds to the cystic lesion seen on MRI. The lesion has a relatively regular shape, and currently there is no obvious evidence of local lymph node enlargement. Overall, this is consistent with findings suggestive of abnormal lymphatic proliferation.
Considering the patient’s profile as a young female with a soft cervical mass, the presence of multiple cystic lesions on MRI, and literature descriptions of lymphatic malformations (lymphangiomatosis or disseminated lymphatic proliferation), the most likely diagnosis is: Lymphangiomatosis.
For definitive confirmation, surgical pathology or ultrasound/CT-guided biopsy may be considered to characterize the histological features and confirm the expression of lymphatic markers. Additionally, a comprehensive evaluation is needed to determine whether the patient has associated pleural effusion (such as chylothorax) or involvement of other organs.
1. Treatment Strategies:
- Surgical Treatment: For localized, symptomatic, or large cervical lesions, surgical excision or decompressive surgery can be considered once the lesion is clearly identified. This can alleviate local compressive symptoms and obtain tissue for pathological diagnosis.
- Interventional or Sclerotherapy: Certain lymphatic malformations may be amenable to sclerotherapy agents, such as OK-432 or bleomycin, to reduce the size of the lesion.
- Medication and Supportive Care: If there is chylothorax or other complications, nutritional support, diuretics, and immunomodulatory treatments may be required as part of a comprehensive management plan.
- Follow-Up Observation: For multiple bony and visceral lesions with mild symptoms, regular imaging and clinical evaluations are recommended to detect lesion progression or complications in a timely manner.
2. Rehabilitation/Exercise Prescription Recommendations (FITT-VP Principle):
- Frequency: 3-5 times per week.
- Intensity: Focus on low to moderate intensity, especially when bone or organ involvement is present. Avoid high-impact activities or those that place excessive stress on bones. Start with light aerobic exercises (e.g., brisk walking, stationary cycling).
- Time: 20-30 minutes per session, initially shorter sessions multiple times a day if necessary, gradually increasing as tolerated.
- Type: Emphasize low-impact aerobic activities that minimize stress on joints and bones, supplemented with low-intensity resistance training (e.g., resistance bands, light dumbbells) to maintain muscle strength and cardiovascular function.
- Progression: In the absence of pain or discomfort, gradually increase the duration or slightly elevate the intensity of exercise. If there is neck or chest pressure, adjust movement range carefully and seek medical evaluation if needed.
- Precautions: In cases of bone lesions, avoid strenuous exercises and reduce the risk of falls. If pleural effusion or impaired lung function is present, closely monitor respiratory status and increase exercise load gradually.
This report provides a reference analysis based on the current imaging and clinical data and does not replace an in-person diagnosis or medical professional’s opinion. Actual treatment plans require a complete history, laboratory tests, and pathological findings to be evaluated and formulated by a qualified medical team.
If you have further questions or experience any changes in your condition, please seek medical attention or consult your specialist promptly.
Systemic lymphangiomatosis
