Presentation
Incidental.
Patient Data
A well-defined, circumscribed mass in the right lower quadrant, measuring approximately 40 x 33 mm. It appears hypoechoic and contains hyperechoic structures resembling fibers or debris. There is no association with the intestines or large blood vessels.
A mass in the right lower quadrant measures approximately 28 x 38 mm. It is well-defined and circumscribed, with homogenous, intense enhancement (50–60 HU) in both the arterial and portal phases, except for central hypoattenuation. There is no association with the surrounding intestines or signs of intestinal wall retraction or thickening.
Histopathology
The specimen appeared tough and firm, grey with pink and white areas, measuring 3.6 x 3.5 x 3 cm.
Microscopic description:
The cut pieces are from lymph node tissue, with the capsule intact. The lymph node parenchyma has lost its structure. The number of lymphoid follicles has decreased, and most of the germinal centers have shrunken. The centers have been hyalinized. There are no longer any macrophages, and there are areas with the image of twin follicle centers. The capsule area is thick, consisting of small nucleated cells with narrow lymphocyte-like cytoplasm. In some areas, the cells are arranged in many concentric layers resembling an onion shell, and they are scattered with Russell bodies. The intercystic area is enlarged and increased. There is hypervascularity, a thick-walled vascular ridge, hyalinization, and minimal eosinophilic infiltration. The jelly center shows a large area of complete fibrosis.
The five specimens stained immunohistochemically with markers DC3, CD20, CD79a, CD10, Bcl16, CD138, CD68, CD15, CD30, CD5, cyclin D1, CD21, lambda, kappa, and Ki67 did not show an immunophenotype, which is consistent with lymphoma.
Conclusion:
Histopathological images are consistent with hyaline vascular Castleman disease.
Case Discussion
This patient doesn’t have any signs or symptoms; the lesion was incidentally explored during a routine abdominal ultrasound.
In this case, the first initial diagnosis is extra-gastrointestinal GIST.
The patient underwent surgery to remove the lesion and the pathology confirms hyaline vascular Castleman disease.
Castleman disease is a rare, nonclonal lymphoproliferative disease being classified into subtypes based on its etiology, pathology, and clinical symptoms. It can affect lymph nodes of any body region including the neck, chest, abdomen, and pelvis, and presents on imaging similar to both benign and malignant lesions.