J Korean Soc Radiol.  2014 Jul;71(1):20-25. 10.3348/jksr.2014.71.1.20.

Malignant Extrarenal Rhabdoid Tumor in Adults: Three Case Reports Originating from the Ileum, Adrenal Gland and Uterus

• Shim E
• Yeom SK
• Um JW
• Kim JH
• Lee SH
• Cha SH
• Choi JW

• KMID: 1709016
• DOI: http://doi.org/10.3348/jksr.2014.71.1.20

Abstract

Malignant extrarenal rhabdoid tumor (MERT) is a very aggressive tumor that is extremely rare in adults. Herein, we introduce three cases of MERT in adults that originated in the ileum, adrenal gland, and uterine endometrium. The MERTs in the ileum and adrenal gland showed non-aggressive features and a good prognosis, while the MERT in the uterine presented with aggressive features and distant metastasis.

MeSH Terms

Adrenal Glands*
Adult*
Endometrium
Female
Fluoroscopy
Humans
Ileum*
Neoplasm Metastasis
Prognosis
Rhabdoid Tumor*
Uterus*

Figure

• 

  Fig. 1 A 52-year-old woman suffered from cramping abdominal pain. A. A 70-second-delayed contrast-enhanced coronal CT scan shows a well-enhanced mass (arrowheads) in the distal ileum. The mass has an intraluminal protruding appearance and no luminal obstruction. B. On 5 days from initial study, contrast enema with water-soluble contrast medium (Gastrografin) is performed due to recurred pain. It shows an ileocolic type intussusception (arrowheads). C. The intussusception (arrowheads) is moved proximally to the distal ileum with the pressure of the enema, but not completely resolved. D. The mass (M) is protruding at the mucosal surface, and the cut surface of the tumor is pale yellow, invading the muscular layer (arrow) and sparing the adventitia (arrowhead) macroscopically. E. In a low-power field-microscopic photograph (H&E stain, × 40), the tumor penetrates the entire proper muscle (PM) layer (arrowhead), and lymphatic tumor emboli are noted. F. In a high-power field-microscopic photograph (H&E stain, × 400), the tumor is composed of polygonal and loosely cohesive cells. The cells show large, eccentric nuclei, homogenous eosinophilic cytoplasm, and intra-cytoplasmic inclusions (arrows), concordant with rhabdoid cells.

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  Fig. 2 A 63-year-old man presenting a right adrenal gland mass. A. A pre-enhanced CT scan shows well-defined, round-shaped, low attenuatting mass in the right upper abdominal cavity. B. An 120-second-delayed contrast-enhanced CT scan shows poor enhancement of the mass. The mass is positioned at a blunt angle to the right adrenal gland (arrowheads). C. The cut surface of the tumor is pinkish tan and soft with hemorrhagic and myxoid foci.

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  Fig. 3 A 58-year-old woman complaining vaginal spotting. A, B. The uterine endometrial mass presents with heterogeneous high-signal intensity on T2-weighted image (A) and heterogeneous low-signal intensity on T1-weighted image (B). Multiple T2 low-signal and T1 high-signal intensity foci in the mass, representing hemorrhage (arrows). C. Contrast-enhanced T1-weighted MR image shows poor enhancement of the mass. D, E. Contrast-enhanced CT scan images performed 3 months after the operation show multiple pelvic tumors and retroperitoneal lymphadenopathy.

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