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Adrenal tumor

Adrenal tumor

Epidemiology:

Adrenal tumors represent a heterogeneous group of diseases characterized by varying clinical behaviors, with their incidence reported at approximately 3-4 cases per 100,000 population annually. Among these tumors, spontaneous remission (SR) is exceptionally rare, particularly in malignant forms such as adrenocortical carcinoma and pheochromocytoma, wherein documented cases suggest that SR constitutes less than 1% of related incidences 1,2. While sporadic cases have been reported concerning spontaneous remission associated with adverse events like hemorrhage or pharmacological interventions, substantial clinical research is needed to elucidate the mechanisms underlying such occurrences 3,4.

Clinical Characteristics:

To date, there have been eight reported cases of SR of either primary or metastatic adrenal tumors. There are several clinical trends that may be observed among these cases. The patients’ age at the time of remission ranges from newborn to 72 years. Females showed slightly higher rates of SR of adrenal tumors. See table 1 below for further information.

Histological Characteristics:

Of the cases analyzed, the histology of the tumors was primarily reported as originating from the adrenal gland, with most identified as adrenal cortical or glandular lesions. Instances of non-adrenal malignancies, such as renal or other extraglandular tumor types, were excluded from this data collection to maintain diagnostic consistency.

Proposed Contributing Mechanisms:

Among the reported cases, proposed mechanisms varied considerably. In two cases, tumor remission was associated with drug withdrawal, while one report proposed an apoptotic mechanism as a possible cause. Another case suggested alterations in DNA ploidy pattern as a contributing factor to remission. The remaining cases did not report any specific therapeutic or mechanistic associations. Collectively, these findings highlight that drug withdrawal, cellular apoptosis, and genetic alterations may play contributory roles in the spontaneous remission of adrenal tumors.

Site and Extent of Remission:

Among the reported cases, most showed complete remission of the primary adrenal tumor, while a few demonstrated remissions at metastatic sites such as the skin and CNS. One patient maintained remission for over two years, indicating durable disease control. Although follow-up data are limited, these findings suggest that spontaneous remission in adrenal tumors can occasionally result in sustained remission.

Table 1: Adrenal Tumor SR Cases and Clinical Characteristics

Author–year

Age/sex

Primary site

Remission site

Proposed mechanisms

Follow-up

Pasqualini & Gurevich, 19565

21/F

Adrenal cortex

Endocrine glands

Not reported

Not reported

Saracco et al., 19886

Newborn / Not reported

Adrenal gland

Skin, CNS

DNA ploidy pattern

1 year

Huston, 19887

20/F

Adrenal gland

Adrenal gland

Not reported

Not reported

Fujiwara et al., 20018

72/M

Adrenal gland

Adrenal gland

Apoptotic mechanism

26 months

Takeshita et al., 20189

45/M

Adrenal gland

Adrenal gland

Not reported

190 days

Hanai et al., 202110

72/F

Adrenal gland

Adrenal gland

Drug withdrawal

6 months

Uslu, 201911

18/M

Muscle

None reported

Recovered spontaneously

References:

  1. Loh K., Gupta R., & Shlossberg A. Spontaneous remission of ectopic cushing's syndrome due to pheochromocytoma: a case report. Acta Endocrinologica. 1996;135(4):440-443. doi:10.1530/eje.0.1350440
  2. Marti J., Millet J., Sosa J., Roman S., Carling T., & Udelsman R. Spontaneous adrenal hemorrhage with associated masses: etiology and management in 6 cases and a review of 133 reported cases. World j. surg.. 2011;36(1):75-82. doi:10.1007/s00268-011-1338-6
  3. Bittencourt J., Averbeck M., & Schmitz H. Hemorrhagic shock due to spontaneous rupture of adrenal pheochromocytoma. Int. braz j urol.. 2003;29(5):428-430. doi:10.1590/s1677-55382003000500007
  4. Motayagheni N., Azhough R., Barband A., Niafar M., & Pourfathi H. Spontaneous rupture of adrenal pheochromocytoma in a patient with von recklinghausen′s disease. Indian Journal of Critical Care Medicine. 2009;13(2):94-95. doi:10.4103/0972-5229.56056
  5. Pasqualini RQ, Gurevich N. Spontaneous remission in a case of Cushing’s syndrome. J Clin Endocrinol Metab. 1956;16:406–410.
  6. Saracco S, Abramowsky C, Taylor S, Silverman RA, Berman BW. Spontaneously regressing adrenocortical carcinoma in a newborn: a case report with DNA ploidy analysis. Cancer. 1988;62(3):507–511. doi:10.1002/1097-Q
  7. Huston N. Spontaneous remission of phaeochromocytoma. N Z Med J. 1988;101(845):248.
  8. Fujiwara T, Kawamura M, Sasaki A, Asahi H, Sasou S, Itoh S, Hiramori K. Transient spontaneous regression of aggressive non-Hodgkin’s lymphoma confined to the adrenal glands. Ann Hematol. 2001;80(9):561–564. doi:10.1007/s002770100335
  9. Takeshita Y, Teramura C, Takamura T. Vanishing of ruptured adrenal mass with takotsubo cardiomyopathy. Endocr J. 2018;65(12):1155–1159. doi:10.1507/endocrj.EJ18-0119
  10. Hanai S, Kobayashi K, Kawashima I, Ichijo M, Nakagomi D. Disappearance of bilateral adrenal tumours: immunodeficiency-associated lymphoproliferative disorder in a patient with rheumatoid arthritis. Rheumatology (Oxford). 2021;60(7):e255–e257. doi:10.1093/rheumatology/keab138
  11. Uslu, S., Yüce İnel, T., Karakaş, A., & Önen, F. (2019). Spontaneous Remission of Isaacs’ Syndrome. Balkan medical journal, 36(4), 251–252. https://doi.org/10.4274/balkanmedj.galenos.2019.2019.2.53
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