J Korean Med Sci.  2006 Jun;21(3):572-576. 10.3346/jkms.2006.21.3.572.

A Case of Non-Functioning Huge Adrenocortical Carcinoma Extending Into Inferior Vena Cava and Right Atrium

• Kim KH
• Park JC
• Lim SY
• Sohn IS
• Yun KH
• Cho SH
• Hong YJ
• Park HW
• Kim JH
• Kim W
• Ahn YK
• Chung IJ
• Jeong MH
• Cho JG
• Kang JC

• KMID: 1778448
• DOI: http://doi.org/10.3346/jkms.2006.21.3.572

Abstract

Primary adrenocortical carcinoma (ACC) is a rare tumor and its usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%). However, intracaval invasion extending into the right atrium is very rare and spontaneous regression of tumor burden in adrenal carcinoma is also rare. We report a case of ACC with direct invasion of the inferior vena cava and right atrium. A 34-yr-old male patient presented with progressive dyspnea, weight loss, and poor oral intake over 3 months. Non-functioning ACC with direct invasion of the inferior vena cava and right atrium was confirmed by imaging, pathologic, and hormonal study. Chemo-radiotherapy was attempted. However, tumor burden was not changed, but rather toxic hepatitis and thrombocytopenia were developed. His subjective symptoms and general conditions were improved after 1 month of conservative management and the patient was discharged. During clinical follow-up, this tumor showed spontaneous regression.

Keyword

Adrenocortical Carcinoma; Neoplasm Metastasis; Vena Cava, Inferior

MeSH Terms

Vena Cava, Inferior/*pathology
Tomography, X-Ray Computed
Remission Induction
Neoplasm Metastasis
Male
Humans
Heart Neoplasms/pathology/*secondary
Heart Atria/*pathology
Follow-Up Studies
Echocardiography
Biopsy
Adult
Adrenocortical Carcinoma/*diagnosis/*pathology
Adrenal Cortex Neoplasms/*diagnosis/*pathology

Figure

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  Fig. 1 Computed tomography (CT) at initial diagnosis (upper panel) reveals 17.5×10 cm sized huge lobulated mass arising from the right adrenal gland (A), heterogeneously enhanced tumor thrombi in inferior vena cava (IVC) (B), and filling defect caused by tumor which occupied nearly entire space of right atrium (RA) (C). Follow-up CT scan (lower panel) reveals marked reduction of tumor size (about 9.5×5.5 cm in size) in the right adrenal gland (D), decreased filling defect in IVC, and markely reduced filling defect in RA (F).

• 

  Fig. 2 Trans-thoracic echocardiography (TTE) at the time of initial diagnosis (upper panel) shows large right atrial mass which occupied nearly entire space of the right atrium (RA) on parasternal short axis view (A) and apical four chamber view (B). On subcostal view, RA mass connected continuously into the inferior vena cava (IVC) (C). Follow-up TTE (lower panel) reveals marked reduction in the size of right atrial mass on parasternal short axis view (D) and apical four chamber view (E). Tumor thrombi in IVC also shows reduction in size on subcostal view (F).

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  Fig. 3 Gun biopsy of the adrenal mass reveals cellular pleomorphism with large nuclei, abundant cytoplasm, and numerous small vascular channels which was compatible findings of endocrine tumor by Hematoxylin-Eosin stain (A: ×40, B: ×200).

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  Fig. 4 Hematoxylin-Eosin stain of bone marrow biopsy reveals increased number of megakaryocytes without abnormal cells suggesting immune thrombocytopenic purpura (ITP) after the chemotherapy (A: ×40, B: ×100).

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