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Does tumor heterogeneity limit the use of the Weiss criteria in the evaluation of adrenocortical tumors?

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Abstract

Adrenal incidentalomas are detected more frequently with high-resolution imaging modalities. It is difficult to distinguish between benign and malignant lesions despite the so-called histologic Weiss criteria, imaging features, and molecular studies. We here present a 52 yr-old man who was found to have an adrenal incidentaloma during an annual check-up at his urologist. An 8 cm large adrenal lesion was detected on ultrasound, computed tomography, and magnetic resonance imaging with imaging features suggestive of malignancy. The lesion was hormonally inactive. A left-sided adrenalectomy was performed and histologic grading revealed a Weiss score of 2, suggesting a benign tumor. However, on further follow-up, the patient developed a local recurrence and pulmonary metastases diagnosed 6 yr after initial presentation. After repeat surgery in the left adrenal bed adrenocortical tumor tissue had a Weiss score of 8, clearly suggesting histologic malignancy. The patient received adjuvant mitotane therapy. Under this therapy, he developed a right-sided adrenal mass (contralateral from the primary tumor) of 2 cm size which disappeared during the following 9 months, whereas the pulmonary metastases remained unchanged, suggesting tumor clones with a variable response to treatment or spontaneous apoptosis. This case suggests that adrenal inciden-talomas larger than 6 cm with imaging features such as intratumoral necrosis suggestive of malignancy, should be managed as potential cancers independent of the so-called Weiss criteria. In such patients, close follow-up examinations including high-resolution imaging (preferably 3 monthly) are needed and should be carried out by a physician familiar/specialized in endocrine oncology.

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References

  1. Russi S, Blumenthal HT, Gray SH. Small adenomas of the adrenal cortex in hypertension and diabetes. Arch Int Med 1945, 76: 284–96.

    Article  CAS  Google Scholar 

  2. Hedeland H, Ostberg G, Hokfelt B. On the prevalence of adrenolcortical adenomas in an autopsy material in relation to hypertension and diabetes. Act Med Scand 1968, 194: 211–4.

    Google Scholar 

  3. Kloos RT, Gross MD, Francis IR, et al. Incidentally discovered adrenal masses. End Rev 1995, 16: 460–84.

    CAS  Google Scholar 

  4. National Cancer Institute, Biometrics branch. In: Cutler SJ, Young JL eds. Third national cancer survey: incidence data. National Cancer Institute Monograph. Washington: Government Printing Office. 1975, 41: 75–787.

    Google Scholar 

  5. Wooten MD, King DK. Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer 1993, 72: 3145–55.

    Article  CAS  PubMed  Google Scholar 

  6. Weiss LM. Comparative histologic study of 43 metastasiz-ing and nonmetastasizing adrenocortical tumors. Am J Surg Path 1984, 8: 163–9.

    Article  CAS  PubMed  Google Scholar 

  7. Giquel C, Bertagna X, Gaston V, et al. Molecular marker and long-term recurrences in a large cohort of patients with sporadic adrenocortical tumors. Canc Res 2001, 61: 6762–7.

    Google Scholar 

  8. Koch CA, Pacak K, Chrousos GP. The molecular patho-genesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab 2002, 87: 5367–84.

    Article  CAS  PubMed  Google Scholar 

  9. Abraham J, Bakke S, Rutt A, et al. A phase II trial of combination chemotherapy and surgical resection for the treatment of metastatic adrenocortical carcinoma: continuous infusion doxorubicin, vincristine, and etoposide with daily mitotane as a P-glycoprotein antagonist. Cancer 2002, 94: 2333–43.

    Article  CAS  PubMed  Google Scholar 

  10. Korobkin M, Brodeur FJ, Francis IR, et al. Delayed enhanced CT for differentiation of benign from malignant adrenal masses. Radiology 1996, 200: 737–42.

    CAS  PubMed  Google Scholar 

  11. National Institutes of Health. State-of-the-science-conference-statement. Managment of the Clinically Inapparent Adrenal Mass (“Incidentaloma”). Final statement 8/2002. http://consensus.nih.gov.

    Google Scholar 

  12. Kopf D, Goretzki PE, Lehnert H. Clinical management of malignant adrenal tumors. J Canc Res Clin Oncol 2001, 127: 143–55.

    Article  CAS  Google Scholar 

  13. Terzolo M, Boccuzzi A, Bovio S, Cappia S, et al. Immunohis-tochemical assessment of Ki-67 in the differential diagnosis of adrenocortical tumors. Urology 2001, 57: 176–82.

    Article  CAS  PubMed  Google Scholar 

  14. Dackiw A P, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg 2001, 25: 914–26.

    Article  CAS  PubMed  Google Scholar 

  15. Mantero F, Terzolo M, Arnaldi G, et al. A survey on adrenal incidentaloma in Italy. Study group on adrenal tumors of the Italian society of endocrinology. J Clin Endocrinol Metab 2000, 85: 637–44.

    CAS  PubMed  Google Scholar 

  16. Barnett CC Jr, Varma DG, El-Naggar AK, et al. Limitations of size as a criterion in the evaluation of adrenal tumors. Surgery 2000, 128: 973–82.

    Article  PubMed  Google Scholar 

  17. Tang CK, Gray GF. Adrenocortical neoplasms. Prognosis and morphology. Urology 1977, 5: 691–5.

    Article  Google Scholar 

  18. Falke THM, te Strake L, Shaff MJ, et al. MR imaging of the adrenals: correlation with computed tomography. J Comput Assist Tomogr 1986, 10: 242–53.

    Article  CAS  PubMed  Google Scholar 

  19. Bernardino ME. Managment of the asymptomatic patient with unilateral adrenal mass. Radiology 1988, 166: 121–3.

    CAS  PubMed  Google Scholar 

  20. Davis PL, Hricak H, Bradley WG Jr. Magnetic resonance imaging of the adrenal glands. Radiol Clin North Am 1984, 22: 891–5.

    CAS  PubMed  Google Scholar 

  21. Van Erkel AR, van Gils, APG, Lequin M, et al. CT and MR distinction of adenomas and nonadenomas of the adrenal glands. J Comput Assist Tomogr 1994, 18: 432–8.

    Article  PubMed  Google Scholar 

  22. McLoughlin RF, Bilbey JH. Tumors of the adrenal gland: findings on CT and MR imaging. Am J Roentgenol 1994, 163: 1413–8.

    Article  CAS  Google Scholar 

  23. Reining JW, Doppman JL, Dwyer AJ, et al. MRI of indeterminate adrenal masses. AJR 1986, 147: 493–6.

    Article  Google Scholar 

  24. Khafagi FA, Gross MD, Shapiro B, et al. Clinical significance of the large adrenal mass. Br J Surg 1991, 78: 828–33.

    Article  CAS  PubMed  Google Scholar 

  25. Siren JE, Haapiainen RK, Huikuri KT, et al. Incidentalomas of the adrenal gland: 36 operated patients and review of literature 1993, 17: 634–9.

    CAS  Google Scholar 

  26. Hussein S, Belldegrun A, Seltzer SE, et al. Differentiation of malignant from benign adrenal masses: predictive indices on computed tomography. AJR Am J Roentgenol 1985, 144: 61–5.

    Article  Google Scholar 

  27. Paivansalo M, Lahde S, Merikanto J, et al. Computed tomography in primary and secondary adrenal tumours. Acta Radiol 1988, 29: 519–22.

    Article  CAS  PubMed  Google Scholar 

  28. Dominguez-Gadea L, Diez L, Bas C, et al. Differntial diagnosis of solid adrenal masses using adrenocortical szintigraphy. Clin Radiol 1994, 49: 796–9.

    Article  CAS  PubMed  Google Scholar 

  29. Gross MD, Shapiro B, Francis IR, et al. Scintigraphic evaluation of clinically silent adrenal masses, J Nucl Med 1994, 35: 1145–52.

    CAS  PubMed  Google Scholar 

  30. Lee MJ, Hahn PF, Papanicolaou N, et al. Benign and malignant adrenal masses: CT distinction with attenuation coefficients, size, and observer analysis. Radiology 1991, 179: 415–8.

    CAS  PubMed  Google Scholar 

  31. Gross MD, Shapiro B, Francis IR, et al. Incidentally discoverd bilateral adrenal masses. Eur J Nucl Med 1995, 22: 315–21.

    Article  CAS  PubMed  Google Scholar 

  32. Lucon AM, Pereira MA, Mendonca BB, et al. Adrenocortical tumors: results of treatment and study of the Weiss score as a prognostic factor. Rev Hosp Clin Fac Med Sao Paulo 2002, 57: 251–6.

    Article  PubMed  Google Scholar 

  33. Terzolo M, Ali A, Osella G, Mazza E. Prevalence of adrenal carcinoma among incidentally discovered adrenal masses. A retrospective study from 1989 to 1994. Gruppo Piemon-tese Incidentalomi Surrenalici. Arch Surg 1997, 132: 914–9.

    CAS  Google Scholar 

  34. Stojadinovic A, Ghossein RA, Hoos A, et al. Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol 2002, 20: 941–50.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to C. A. Koch MD, FACP, FACE.

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Pohlink, C., Tannapfel, A., Eichfeld, U. et al. Does tumor heterogeneity limit the use of the Weiss criteria in the evaluation of adrenocortical tumors?. J Endocrinol Invest 27, 565–569 (2004). https://doi.org/10.1007/BF03347480

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