Castleman disease: unicentric - mandible

Case contributed by Qutaiba Jaf'ar Mahmoud
Diagnosis certain

Presentation

Left neck painless swelling.

Patient Data

Age: 15 years
Gender: Male

There is a well-defined homogeneously relatively hyper enhancing lesion measuring 3 x 2.5 cm seen in the left submandibular area, without internal calcification, fat or necrosis, causing mass-effect on the left submandibular gland, left sternocleidomastoid muscle and mild mass-effect on the left internal jugular vein.

No other masses or enlarged cervical lymph nodes.

Case Discussion

Chest and abdomen CT scan with contrast was done (not shown) and revealed that no other lesions or enlarged lymph nodes are noted.

Localized hyper-enhancing enlarged lymph nodes in comparison with muscles are suggestive of unicentric Castleman disease.

PATHOLOGY REPORT

GROSS:
Specimen fixed labeled with patient's name, consists of 15 gm, multiple fragmented brownish soft tissue pieces in aggregate measuring 5x5x2 cm. Submitted all in six cassettes.

MICROSCOPIC:
Fragmented lymph node, showing areas with reactive lymphoid follicular hyperplasia, with germinal centers of variable size and shape, with tingible body macrophages. Other areas show follicles having markedly- expanded mantle zone, with concentric layering, and small, inconspicuous germinal centers, some with hyalinization and minimal vascular proliferation. The interfollicular stroma is prominent with hyperplastic post-capillary venules, and areas with thick hyaline bands, negative for amyloid by Congo red stain. No granulomas were seen. No malignancy was seen in the levels examined.

DIAGNOSIS:
Left cervical lymph node, excisional biopsy:
Features most in keeping with reactive lymph node with partial involvement by Castleman disease, hyaline vascular type, lymphoid subtype.

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