J Korean Med Sci.  2017 May;32(5):764-771. 10.3346/jkms.2017.32.5.764.

Myxoid and Sarcomatoid Variants of Adrenocortical Carcinoma: Analysis of Rare Variants in Single Tertiary Care Center

Affiliations
  • 1Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
  • 2Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, Korea.
  • 3Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
  • 4Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. hipuha@hanmail.net

Abstract

The aim of this study is to describe rare variants of adrenocortical carcinoma (ACC) and to compare the prognosis with that of conventional ACC. We retrospectively reviewed 8 cases of myxoid variant, 1 sarcomatoid variant, and 14 cases of conventional ACC, who underwent surgical resection at the Asan Medical Center between 1996 and 2014. An analysis of the clinicopathological characteristics, including the Weiss score, Ki-67 labeling index, and reticulin framework assessment is presented. The mean age of patients with myxoid/sarcomatoid ACC was 45 years; 4 out of 9 patients were women. Mean primary tumor size was 12.9 cm and the mean weight was 702.4 g. Seven patients presented in an advanced stage (stage III/IV); 8 of these eventually developed distant metastasis. The mean Weiss score was 5.0 points and the Ki-67 labeling index was 15.6%. The extent of myxoid or sarcomatoid change on histological examination ranged from 10% to 75% of the examined tumor areas; reticulin framework alteration was observed in all cases. Four patients showed venous tumor thrombus. Most of the clinicopathological parameters were not significantly different from those of conventional ACC. However, myxoid or sarcomatoid variant (hazard ratios [HR], 3.59; 95% confidence intervals [CI], 1.13-11.38; P = 0.030) and Ki-67 labeling index (HR, 3.97; 95% CI, 1.18-13.41; P = 0.030) were independent predictors of overall survival after adjusting for age and sex. Myxoid or sarcomatoid histological features or an increased Ki-67 labeling index may be associated with poor overall survival in patients with ACC.

Keyword

Adrenocortical Carcinoma; Myxoid; Sarcomatoid; Ki-67; Survival

MeSH Terms

Adrenocortical Carcinoma*
Chungcheongnam-do
Female
Humans
Neoplasm Metastasis
Prognosis
Reticulin
Retrospective Studies
Tertiary Care Centers*
Tertiary Healthcare*
Thrombosis
Reticulin

Figure

  • Fig. 1 Histological features of myxoid (A-D) and sarcomatoid (E, F) variants of ACC. Grossly, the myxoid variant ACC shows a variegated cut surface with yellowish tan, necrotic, focally gelatinous and hemorrhagic foci (A). Tumor cells of myxoid ACC show various growth patterns, including inter-anastomosing cords, small clusters, and microcystic patterns (B) in an Alcian-Blue positive myxoid stroma background (C) and also show reticulin network alterations (D). Grossly, the sarcomatoid variant of ACC shows a variegated cut surface with yellowish tan, necrotic, and partially white fleshy foci (E). Tumor cells of the sarcomatoid variant ACC show a diffuse growth pattern with mainly spindle cells (F). ACC = adrenocortical carcinoma.

  • Fig. 2 Overall survival of patients with adrenocortical carcinoma disaggregated by tumor subtype (A) and Ki-67 labeling index (B) by the log-rank test. Presence of myxoid or sarcomatoid histological features (A) and an increased Ki-67 labeling index (B) show a similar marginally significant association with overall survival on the log-rank test.


Reference

1. Baudin E; Endocrine Tumor Board of Gustave Roussy. Adrenocortical carcinoma. Endocrinol Metab Clin North Am. 2015; 44:411–434.
2. Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoili EM, Jolly S, Miller BS, Giordano TJ, Hammer GD. Adrenocortical carcinoma. Endocr Rev. 2014; 35:282–326.
3. de Krijger RR, Papathomas TG. Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants. Virchows Arch. 2012; 460:9–18.
4. Erickson LA, Rivera M, Zhang J. Adrenocortical carcinoma: review and update. Adv Anat Pathol. 2014; 21:151–159.
5. Phan AT. Adrenal cortical carcinoma--review of current knowledge and treatment practices. [viii-ix.]. Hematol Oncol Clin North Am. 2007; 21:489–507.
6. Tang CK, Harriman BB, Toker C. Myxoid adrenal cortical carcinoma: a light and electron microscopic study. Arch Pathol Lab Med. 1979; 103:635–638.
7. Forsthoefel KF. Myxoid adrenal cortical carcinoma. A case report with differential diagnostic considerations. Arch Pathol Lab Med. 1994; 118:1151–1153.
8. Brown FM, Gaffey TA, Wold LE, Lloyd RV. Myxoid neoplasms of the adrenal cortex: a rare histologic variant. Am J Surg Pathol. 2000; 24:396–401.
9. Izumi M, Serizawa H, Iwaya K, Takeda K, Sasano H, Mukai K. A case of myxoid adrenocortical carcinoma with extensive lipomatous metaplasia. Arch Pathol Lab Med. 2003; 127:227–230.
10. Suresh B, Kishore TA, Albert AS, Joy A. Myxoid adrenal cortical carcinoma--a rare variant of adrenocortical carcinoma. Indian J Med Sci. 2005; 59:505–507.
11. Karim RZ, Wills EJ, McCarthy SW, Scolyer RA. Myxoid variant of adrenocortical carcinoma: report of a unique case. Pathol Int. 2006; 56:89–94.
12. Raparia K, Ayala AG, Sienko A, Zhai QJ, Ro JY. Myxoid adrenal cortical neoplasms. Ann Diagn Pathol. 2008; 12:344–348.
13. Papotti M, Volante M, Duregon E, Delsedime L, Terzolo M, Berruti A, Rosai J. Adrenocortical tumors with myxoid features: a distinct morphologic and phenotypical variant exhibiting malignant behavior. Am J Surg Pathol. 2010; 34:973–983.
14. Zhang J, Sun J, Liang Z, Gao J, Zeng X, Liu T. Myxoid adrenocortical neoplasms: a study of the clinicopathologic features and EGFR gene status of ten Chinese cases. Am J Clin Pathol. 2011; 136:783–792.
15. Hsieh MS, Chen JH, Lin LW. Myxoid adrenal cortical carcinoma presenting as primary hyperaldosteronism: case report and review of the literature. Int J Surg Pathol. 2011; 19:803–807.
16. Weissferdt A, Phan A, Suster S, Moran CA. Myxoid adrenocortical carcinoma: a clinicopathologic and immunohistochemical study of 7 cases, including 1 case with lipomatous metaplasia. Am J Clin Pathol. 2013; 139:780–786.
17. Gurzu S, Szentirmay Z, Bara T, Bara T Jr, Jung I. Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature. Pathology. 2014; 46:83–85.
18. Okazumi S, Asano T, Ryu M, Nagashima T, Odaka M, Isono K, Nishizawa T. Surgical resection of adrenal carcinoma extending into the vena cava, right atrium and ventricle: case report and review of the literature. Nippon Geka Gakkai Zasshi. 1987; 88:231–238.
19. Collina G, Maldarizzi F, Betts CM, Eusebi V. Primary sarcomatoid carcinoma of the adrenal gland. First case report. Virchows Arch A Pathol Anat Histopathol. 1989; 415:161–167.
20. Decorato JW, Gruber H, Petti M, Levowitz BS. Adrenal carcinosarcoma. J Surg Oncol. 1990; 45:134–136.
21. Fischler DF, Nunez C, Levin HS, McMahon JT, Sheeler LR, Adelstein DJ. Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings. Am J Surg Pathol. 1992; 16:626–631.
22. Barksdale SK, Marincola FM, Jaffe G. Carcinosarcoma of the adrenal cortex presenting with mineralocorticoid excess. Am J Surg Pathol. 1993; 17:941–945.
23. Lee MS, Park IA, Chi JG, Ham EK, Lee KC, Lee CW. Adrenal carcinosarcoma--a case report. J Korean Med Sci. 1997; 12:374–377.
24. Sturm N, Moulai N, Laverrière MH, Chabre O, Descotes JL, Brambilla E. Primary adrenocortical sarcomatoid carcinoma: case report and review of literature. Virchows Arch. 2008; 452:215–219.
25. Coli A, Di Giorgio A, Castri F, Destito C, Marin AW, Bigotti G. Sarcomatoid carcinoma of the adrenal gland: a case report and review of literature. Pathol Res Pract. 2010; 206:59–65.
26. Feng YC, Yang ZG, Chen TW, Su XY, Deng W, Wang QL. Adrenal sarcomatoid carcinoma: a rare case depicted on multi-detector row computed tomography. Indian J Med Sci. 2010; 64:37–40.
27. Sasaki K, Desimone M, Rao HR, Huang GJ, Seethala RR. Adrenocortical carcinosarcoma: a case report and review of the literature. Diagn Pathol. 2010; 5:51.
28. Thway K, Olmos D, Shah C, Flora R, Shipley J, Fisher C. Oncocytic adrenal cortical carcinosarcoma with pleomorphic rhabdomyosarcomatous metastases. Am J Surg Pathol. 2012; 36:470–477.
29. Mark D, Boyd C, Eatock F. Adrenal sarcomatoid carcinoma: a case report and review of the literature. Ulster Med J. 2014; 83:89–92.
30. Yan JJ, Sun AJ, Ren Y, Hou C. Primary adrenocortical sarcomatoid carcinoma: report of a case. Can Urol Assoc J. 2012; 6:E189–91.
31. Papotti M, Libè R, Duregon E, Volante M, Bertherat J, Tissier F. The Weiss score and beyond--histopathology for adrenocortical carcinoma. Horm Cancer. 2011; 2:333–340.
32. Duregon E, Fassina A, Volante M, Nesi G, Santi R, Gatti G, Cappellesso R, Dalino Ciaramella P, Ventura L, Gambacorta M, et al. The reticulin algorithm for adrenocortical tumor diagnosis: a multicentric validation study on 245 unpublished cases. Am J Surg Pathol. 2013; 37:1433–1440.
33. Lughezzani G, Sun M, Perrotte P, Jeldres C, Alasker A, Isbarn H, Budäus L, Shariat SF, Guazzoni G, Montorsi F, et al. The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the international union against cancer-staging system: a North American validation. Eur J Cancer. 2010; 46:713–719.
34. Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol. 1984; 8:163–169.
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