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
Age/sex | Primary site | Remission site | Proposed mechanisms | Follow-up | |
|---|---|---|---|---|---|
Pasqualini & Gurevich, 19565 | 21/F | Adrenal cortex | Endocrine glands | Not reported | Not reported |
Newborn / Not reported | Adrenal gland | Skin, CNS | DNA ploidy pattern | 1 year | |
20/F | Adrenal gland | Adrenal gland | Not reported | Not reported | |
72/M | Adrenal gland | Adrenal gland | Apoptotic mechanism | 26 months | |
45/M | Adrenal gland | Adrenal gland | Not reported | 190 days | |
72/F | Adrenal gland | Adrenal gland | Drug withdrawal | 6 months | |
86 -year-old female | Left side nose | Clinical remission at 8 weeks | IHC T/B cell infiltration. Tcell mediated immune reaction | Clinical remission at 8 weeks | |
38 years old woman. Operated of appendicitis at the age of 5. At 26 right oophorectomy. At 37 ovarian cystectomy. Patient had smoked one pack of cigarettes daily for 20 years. | Kidney/Adrenal glands bilateral | After 6 months from the first admission her abdominal CT and USG revealed total regression of surreal haematoma. | After 6 months from the first admission her abdominal CT and USG revealed total regression of surreal haematoma. | ||
42 -year-old male | right submandibular, right epitrochlear, left axillary, right inguinal and right femoral regions | 2 months following his lymphogram, peripheral lymphadenopathy was noted to regress 4 months following his lymphogram, he had no evidence of disease on physical exam and the previously abnormal lymph nodes observed on the abdominal lymphogram and chest roentgenogram had regressed showing equivocal abnormalities but no definite evidence of lymphoma he has continued asymptomatic and without evidence of disease 5 years since his initial diagnsis | host immunity, local host or environment factors, diagnostic tools may affect the course of the disease | 2 months following his lymphogram, peripheral lymphadenopathy was noted to regress 4 months following his lymphogram, he had no evidence of disease on physical exam and the previously abnormal lymph nodes observed on the abdominal lymphogram and chest roentgenogram had regressed showing equivocal abnormalities but no definite evidence of lymphoma he has continued asymptomatic and without evidence of disease 5 years since his initial diagnsis | |
68 -year-old female | right posterior cervical, right posterior auricular, right submandiibular, bilateral axillary | 3 months later, it was noted that her peripheral lymphadenopathy had regressed the left hilar mass on chest roentgenogram resolved and the abdominal lymphogram demonstrated marked reduction in the size of the involved lymph nodes she continues asymptomatic without therapy and without clinical evidence of disease 2 years after her visit | host immunity, local host or environment factors, diagnostic tools may affect the course of the disease | 3 months later, it was noted that her peripheral lymphadenopathy had regressed the left hilar mass on chest roentgenogram resolved and the abdominal lymphogram demonstrated marked reduction in the size of the involved lymph nodes she continues asymptomatic without therapy and without clinical evidence of disease 2 years after her visit | |
43 -year-old female | bilateral cervical, supraclavicular, axillary, and inguinal, and right iliac mass | 4 months later, her peripheral lymphadenopathy was noted to regress chest roentgenogram demonstrated reduction of the hiar and paratracheal lymphadenopathy and lymphogram showed reduction in the size of the opacified lymph nodes partial regression of her lymphadenopathy continued for 14 months subsequently, her generalized lymphadenopathy began to enlarge slowly 32 months following her initial visit, she has not yet required therapy | host immunity, local host or environment factors, diagnostic tools may affect the course of the disease | 4 months later, her peripheral lymphadenopathy was noted to regress chest roentgenogram demonstrated reduction of the hiar and paratracheal lymphadenopathy and lymphogram showed reduction in the size of the opacified lymph nodes partial regression of her lymphadenopathy continued for 14 months subsequently, her generalized lymphadenopathy began to enlarge slowly 32 months following her initial visit, she has not yet required therapy | |
18-year-old man. Smoker. History of lichen planus | muscle | While further investigations were in progress, the patient recovered spontaneously without treatment. | None reported | While further investigations were in progress, the patient recovered spontaneously without treatment. | |
72-year-old woman, T.T.G., general symptoms of discomfort, anorexia, asthenia and loss of weight, UCL and diabetes, a large decubitis ulcer of 6 centimeters in diameter is in the sacral region | Notable decrease of the large pulmonary metastasis, most of the pulmonary metastases were diminished in size and others had disappeared | Spontaneous reversion | Notable decrease of the large pulmonary metastasis, most of the pulmonary metastases were diminished in size and others had disappeared | ||
A forty-two-year-old black woman | A repeat IVP showed findings of papillary necrosis but no evidence of a cystic lesion. A selective right renal arteriogram proved that the lesion was no longer present | A repeat IVP showed findings of papillary necrosis but no evidence of a cystic lesion. A selective right renal arteriogram proved that the lesion was no longer present | |||
A sixty-four-year-old white woman | a repeat IVP with nephrotomograms showed bilateral irregular renal outlines but failed to demonstrate the large mass. Repeat renal angiography showed only bilateral multiple small cysts | a repeat IVP with nephrotomograms showed bilateral irregular renal outlines but failed to demonstrate the large mass. Repeat renal angiography showed only bilateral multiple small cysts | |||
References:
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- Yoneda, T., Demura, M., Takata, H., Kometani, M., Karashima, S., Yamagishi, M., & Takeda, Y. (2012). Unilateral primary aldosteronism with spontaneous remission after long-term spironolactone therapy. The Journal of Clinical Endocrinology and Metabolism, 97(4), 1109-1113. doi:10.1210/jc.2011-2563
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- 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.
- Bittencourt J., Averbeck M., & Schmitz H. Hemorrhagic shock due to spontaneous rupture of adrenal pheochromocytoma. Int. braz j urol.. 2003;29(5):428-430
- 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 Medici