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Neonatal Hypoglycemia
Joseph Junewick, MD FACR
over 5 years ago
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Macrocystic Lymphovascular Malformation

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: US CT
Activities:
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History

11 month old with soft left-sided neck mass.


Case Images


Diagnosis

Macrocystic Lymphovascular Malformation

Clinical Notes

Immunohistochemical stain positive for D2-40 (indicating lymphothelium).

Discussion

Lymphangioma is a common benign tumor, thought to arise from sequestered embryonic lymph channels. Most lymphangiomas are detected by the time the patient is 2 years old, the age of greatest lymphatic growth. Lymphangiomas are found equally in females and males. The jugular lymphatic sac is most commonly involved and accounts for the predisposition of the neck and face. In children, the most common location is the posterior cervical space, followed by the oral cavity, the axilla, the mediastinum, the abdominal cavity (colon, spleen, and liver), the retroperitoneum (kidneys), the scrotum, and even the skeleton. These lesions are characteristically infiltrative in nature and do not respect fascial planes.

The majority of cystic hygromas are clinically asymptomatic and manifest as painless, soft or semifirm masses in the neck. Size is extremely variable. Although these masses usually grow slowly, they may suddenly increase in size secondary to hemorrhage or trauma or because of a viral infection when large amounts of lymphatic fluid are produced from the lymphoid follicles in the cyst wall. Other reported clinical manifestations include facial nerve paralysis, dysphagia, or other feeding problems. Chylothorax and chylopericardium may occur as complications of mediastinal involvement.

On CT, lymphangiomas tend to appear as poorly circumscribed, unilocular or multilocular, hypodense masses; traumatized or inflamed lesions commonly show higher attenuation than that seen in simple fluid. Usually, the mass is centered in the posterior triangle or in the submandibular space. On MR, lymphangiomas show low or intermediate signal intensity on T1-weighted images and high signal on T2-weighted images. Infrequently, this lesion may be hyperintense on T1-weighted images, related to blood or chyle.

Reference

Koeller KK, Alamo L, Adair CF, Smirniotoupolos JG. From the Archives of the AFIP: Congenital Cystic Masses of the Neck: Radiologic-Pathologic Correlation. RadioGraphics (1999); 19: 121-146.



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