Primary gastric amyloidosis associated with linitis plastica and delayed progression to systemic amyloidosis and multiple myeloma

Abstract

We report the case of a woman in her 50s who underwent, 5 years prior, a total gastrectomy after neoadjuvant chemotherapy for diffuse-type gastric cancer diagnosed during a workup for isolated gastric primary light chain (AL) amyloidosis. At the time of diagnosis, immunoglobulins light chain measurements and bone marrow biopsy were performed to rule out multiple myeloma and came back normal. Three years later, the patient developed systemic amyloidosis involving the heart and the lungs, after which she developed multiple myeloma. Isolated amyloid deposits in the stomach are a rare finding. While AL amyloidosis is frequently found in concomitance with multiple myeloma, late progression of primary AL amyloidosis to systemic amyloidosis and multiple myeloma is uncommon.

Keywords: Cancer - see Oncology; Gastric cancer; Pathology.

Figure 1

(A) Atypical plasmacytoid cells with an eccentric nucleus (H&E stain) ×10. (B) Multinucleated foreign body giant cell containing amorphous eosinophilic material (H&E stain) ×20. (C) Diffuse-type gastric adenocarcinoma; the cells do not reveal classic intracytoplasmic mucin with the alcian blue stain ×40. (D) Amorphous eosinophilic material Congo red stain and appearing orange-red by conventional light microscopy. Under polarised light microscopy, amyloid exhibits apple-green birefringence ×20.

Figure 2

(A) Immunostaining of atypical cells with cytokeratin cocktail (CKC) reveals their epithelial nature ×40. (B) Positive immunostaining of plasmacytoid cells with CD138 ×40. (C–D) Reactional plasma cells are polytypic for light chains immunostaining ×40.

Figure 3

(A) Massive pan-gastric pseudotumoral amyloidosis with amyloid deposits in the gastric wall (H&E stain) ×20. (B) Congo red stain of amyloid deposits in the gastric wall ×20.