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April 2018

A 77-year-old female with a history of DM type-2, hypertension and nephrolithiasis presented with flank pain

Contributed by:

Khaleel I. Al-Obaidy

Resident Physician PGY-3, Anatomic Pathology and Clinical Pathology

Indiana University School of Medicine


David J. Grignon, M.D., FRCP(C)

Centennial Professor, Department of Pathology and Laboratory Medicine

Indiana University School of Medicine

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Clinical history

The patient is a 77-year-old female with a history of DM type-2, hypertension and nephrolithiasis who presented to the urology clinic with flank pain. A CT scan was done and showed bilateral hydronephrosis. On cystoscopy, significant narrowing of both ureteric lumens with complete occlusion of the right ureter was noted. Bilateral double-J ureteral stents were inserted after multiple unsuccessful attempts on the right side. A few months later, the patient developed septicemia secondary to candidal pyelonephritis and right distal ureterectomy was done.

Gross description

The specimen consisted of a segment of ureter with a bladder cuff. It had dimensions of 2.5 x 1.5 x 1.2 cm. Sectioning revealed a pink-tan to red-brown, heterogeneous cut surface with thickened wall and narrowed lumen. No obvious mass lesion was identified.

Microscopic description

On microscopic examination, the ureteric lumen was circumferentially narrowed by an abundant acellular hyaline material and numerous variable-sized tubules within the lamina propria. The tubules were lined by flattened to simple cuboidal cells with occasional hobnail appearance surrounded by a mildly thickened basement membrane. The nuclei were round with inconspicuous nucleoli and minimal cytologic atypia. No mitotic activity was identified. A background of abundant lymphoplasmacytic inflammation within the lamina propria and muscularis propria was also present. The tubules and the abundant inflammatory infiltrates formed a polypoid mass which further obstructed the ureteral lumen (Figure 1A-D).

Figure 1: A. Ureteric wall with amorphus hyaline material deposited circumferentially, along with tubular and cystic proliferation in the lamina propria in a background of inflammation. B. Extracellular amorphus hyaline material, underneath the mucosa, expanding the lamina propria. C and D. Proliferation of cysts and tubules with focal thickening of basement membrane further expanded the lamina propria. The cells are mostly flattened with hobnailed nuclei (H&E. A-B. 100X, C. 200X, D. 400X, respectively).

Ancillary studies

Immunohistochemical staining for PAX-8 showed diffuse nuclear staining of the cells lining the tubules and cysts (figure 2). The acellular hyaline material had a salmon-pink color on Congo red stain and showed apple-green birefringence on polarization (figure 3A-B).

Figure 2: PAX-8 Immunostain showing diffuse nuclear staining of the cells lining the tubules and cysts.

Figure 3-A and B: The hyaline material with circumferential expansion of lamina propria showing salmon-pink color on Congo red stain and apple-green birefringence on polarization, respectively.

April 2018 Final Diagnosis and Discussion

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