Adrenal-Cortical Carcinoma

Adrenal-Cortical Carcinoma (ACC) : is an extremely rare form of cancer, actually 125 times more rare than breast or lung cancer (0.7-2 people per million every year are diagnosed - compared to 125 people per million year for either breast or lung cancer). ACC is defined by unique malignant tumor cells which form on the outer layer of ones adrenal gland known as the adrenal - "cortex " of the adrenal gland. There are two adrenal glands in the average person and sit on top the kidneys like little triangle or pyramid like structures. The adrenal glands (think 'adrenaline') produce cortisol and other vital hormones throughout your day - it makes sure your bodies stress levels are regulated, not too much stress, not too little, just right. The adrenal glands act as little sentinals detecting hormonal changes the require it to release stop releasing the. Another important role it plays, is a part in circadian rhythms as cortisol for most people is suppose to slowly increase in blood concentration until it hits a point to help you wake up, with the right amount of stress in your body - at night your adrenal glands slowly begin to circuit less cortisol to help you fall asleep without being stressed.

References - ACC Scientific Research & Reviews

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Ilanchezhian, M., Varghese, D. G., Glod, J. W., Reilly, K. M., Widemann, B. C., Pommier, Y., Kaplan, R. N., & Del Rivero, J. (2022). Pediatric adrenocortical carcinoma. Frontiers in Endocrinology, 13, 961650. https://doi.org/10.3389/fendo.2022.961650

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Ahmed, A. A., Thomas, A. J., Ganeshan, D. M., Blair, K. J., Lall, C., Lee, J. T., Morshid, A. I., Habra, M. A., & Elsayes, K. M. (2020). Adrenal cortical carcinoma: Pathology, genomics, prognosis, imaging features, and mimics with impact on management. Abdominal Radiology, 45(4), 945–963. https://doi.org/10.1007/s00261-019-02371-y

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de Krijger, R. R., & Papathomas, T. G. (2012). Adrenocortical neoplasia: Evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants. Virchows Archiv, 460(1), 9–18. https://doi.org/10.1007/s00428-011-1166-y

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Duan, K., Gucer, H., Kefeli, M., Asa, S. L., Winer, D. A., & Mete, O. (2020). Immunohistochemical analysis of the metabolic phenotype of adrenal cortical carcinoma. Endocrine Pathology, 31(3), 231–238. https://doi.org/10.1007/s12022-020-09624-3

Luconi, M., Cantini, G., van Leeuwaarde, R. S., Roebaar, R., Fei, L., Propato, A. P., Santi, R., Ercolino, T., Mannelli, M., Canu, L., de Krijger, R. R., & Nesi, G. (2023). Prognostic value of microscopic tumor necrosis in adrenal cortical carcinoma. Endocrine Pathology, 34(2), 224–233. https://doi.org/10.1007/s12022-023-09760-6

Mills, J. K., Khalil, M., Pasieka, J., Kong, S., Xu, Y., & Harvey, A. (2019). Oncocytic subtypes of adrenal cortical carcinoma: Aggressive in appearance yet more indolent in behavior? Surgery, 166(4), 524–533. https://doi.org/10.1016/j.surg.2019.05.049

Wang, X., Zhou, N., Xiao, Y., Zhu, W., Bai, C., & Zhao, L. (2019). Metastatic adrenal cortical carcinoma responding to octreotide: A case report. The Oncologist, 24(8), e793–e797. https://doi.org/10.1634/theonc

Biology Behind ACC' Emotional Toll

ACC disrupts the adrenal cortex—the source of cortisol, the hormone that regulates stress, energy, immunity, and emotional balance. When ACC invades, cortisol often surges uncontrollably. The result? A body locked in permanent “fight-or-flight,” causing:

Rapid weight gain
Muscle weakness
Blood pressure spikes
Anxiety and depression
Intense mood swings

For Pamela, this hormonal storm has been as painful as the cancer itself. It’s reshaped her daily life, amplified fear, and tested her mental health. Yet she continues to fight with courage and grit. Pamela’s Treatment Journey Despite the rupture setback, Pamela pursued every medical path available:

Chemotherapy
Proton therapy
Removal of the adrenal gland (triggering severe anxiety until her remaining gland “woke up”)
A second complex abdominal surgery
More chemo, then more radiation

Doctors often express shock at her resilience—she continues to outpace expectations. She declined a chemo drug that would destroy her remaining adrenal gland because living permanently steroid-dependent for only marginal benefit wasn’t worth the cost. Insurance has resisted covering promising immunotherapies due to the cancer’s rarity. Charities rarely include ACC. Research funding for rare cancers has been slashed. The safety net for patients like Pamela is disappearing.

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