Adrenal carcinoma: Difference between revisions
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*Cushing's syndrome (most common) | *Cushing's syndrome (most common) | ||
*Mixed Cushing's and virilization ([[glucocorticoid]] and [[androgen]] overproduction), with virilization presenting most obviously in women as: | *Mixed Cushing's and virilization ([[glucocorticoid]] and [[androgen]] overproduction), with virilization presenting most obviously in women as: | ||
*[[Hirsutism|Excess facial and body hair]] (hirsutism) | **[[Hirsutism|Excess facial and body hair]] (hirsutism) | ||
*[[Acne]] | **[[Acne]] | ||
*Enlargement of the [[clitoris]] | **Enlargement of the [[clitoris]] | ||
*Deepening of the voice | **Deepening of the voice | ||
*Coarsening of facial features | **Coarsening of facial features | ||
*[[Amenorrhea|Cessation of menstruation]] (amenorrhea) | **[[Amenorrhea|Cessation of menstruation]] (amenorrhea) | ||
*[[Feminization]] (i.e. estrogen excress, most readil<nowiki/>y seen in men) presents as: | *[[Feminization]] (i.e. estrogen excress, most readil<nowiki/>y seen in men) presents as: | ||
**[[Gynecomastia|Breast enlargement]] (gynecomasytia) | **[[Gynecomastia|Breast enlargement]] (gynecomasytia) | ||
| Line 112: | Line 112: | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
*[[Radiation therapy]] and [[radiofrequency ablation]] may be used for [[Palliative care|palliation]] in patients who are not surgical candidates | *[[Radiation therapy]] and [[radiofrequency ablation]] may be used for [[Palliative care|palliation]] in patients who are not surgical candidates | ||
*[[Chemotherapy]] regimens typically include the drug [[mitotane]], an inhibitor of [[steroid]] synthesis which is toxic to cells of the [[adrenal cortex]], as well as standard cytotoxic drugs. One widely used regimen consists of [[cisplatin]], [[doxorubicin]], [[etoposide]]) and mitotane. The endocrine cell toxin [[streptozotocin]] has also been included in some treatment protocols. Chemotherapy may be given to patients with unresectable disease, to shrink the tumor prior to surgery ([[neoadjuvant chemotherapy]]), or in an attempt to eliminate microscopic residual disease after surgery ([[adjuvant chemotherapy]]). | *[[Chemotherapy]] regimens typically include the drug [[mitotane]], an inhibitor of [[steroid]] synthesis which is toxic to cells of the [[adrenal cortex]], as well as standard cytotoxic drugs. One widely used regimen consists of [[cisplatin]], [[doxorubicin]], [[etoposide]]) and mitotane. The endocrine cell toxin [[streptozotocin]] has also been included in some treatment protocols. Chemotherapy may be given to patients with unresectable disease, to shrink the tumor prior to surgery ([[neoadjuvant chemotherapy]]), or in an attempt to eliminate microscopic residual disease after surgery ([[adjuvant chemotherapy]]). | ||
*[[Hormonal therapy (oncology)|Hormonal therapy]] with steroid synthesis inhibitors such as [[aminoglutethimide]] may be used in a palliative manner to reduce the symptoms of hormonal syndromes | *[[Hormonal therapy (oncology)|Hormonal therapy]] with steroid synthesis inhibitors such as [[aminoglutethimide]] may be used in a palliative manner to reduce the symptoms of hormonal syndromes | ||
===Surgery=== | ===Surgery=== | ||
The only curative treatment is complete [[Surgery|surgical]] excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the [[renal vein]] or [[inferior vena cava]] | |||
<br /> | * The only curative treatment is complete [[Surgery|surgical]] excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the [[renal vein]] or [[inferior vena cava]] | ||
* The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates<br /> | |||
==References== | ==References== | ||
Revision as of 14:23, 5 August 2019
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American Roentgen Ray Society Images of Adrenal carcinoma |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Overview
Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an aggressive cancer originating in the cortex (steroid hormone-producing tissue) of the adrenal gland. Adenocortical carcinoma is remarkable for the many hormonal syndromes which can occur in patients with steroid hormone-producing ("functional") tumors, including Cushing's syndrome, Conn syndrome, virilization, and feminization.
Classification
Adrenal cancer is subclassified according to its activity into:[1]
- Functional
- Non-functional
Pathophysiology
- Adrenal tumors are often not biopsied prior to surgery, so diagnosis is confirmed on examination of the surgical specimen by a pathologist.[2][3][4][5][6]
- Pathologic criteria of Wieneke et al[7]
Risk factors
- Risk factors for adrenal carcinoma include:[8]
- Genetic mutations
- Family history of adrenal carcinoma
- Having some genetic diseases increase the chance of getting adrenal carcinoma such as:
Prognosis
- ACC, generally, carries a poor prognosis and is unlike most tumours of the adrenal cortex, which are benign (adenomas) and only occasionally cause Cushing's syndrome. Five-year disease-free survival for a complete resection of a stage I-III ACC is approximately 30%.[9][10][11][12]
- Weiss criteria has a really good prognostic value for adrenocortical tumors[13]
Diagnosis
History and Symptoms
- Adrenocortical carcinoma may present differently in children and adults[14][15][16][17]
- Most tumors in children are functional, and present with:
- Virilization (most common presenting symptom)
- Cushing's syndrome (glucocorticoid excess):
- Weight gain
- Muscle wasting
- Purple striae/lines on the abdomen
- Fatty "buffalo hump" on the neck
- "Moonlike" face
- Thinning of skin
- Fragile skin
- Precocious puberty[18]
- Adults present with hormonal syndromes such as:
- Cushing's syndrome (most common)
- Mixed Cushing's and virilization (glucocorticoid and androgen overproduction), with virilization presenting most obviously in women as:
- Excess facial and body hair (hirsutism)
- Acne
- Enlargement of the clitoris
- Deepening of the voice
- Coarsening of facial features
- Cessation of menstruation (amenorrhea)
- Feminization (i.e. estrogen excress, most readily seen in men) presents as:
- Breast enlargement (gynecomasytia)
- Decreased libido
- Impotence
- Conn syndrome (mineralcorticoid excess, <10% cases) with low plasma renin activity, and high serum aldosterone presents with:[19]
- High blood pressure causing:
- Hypokalemia causing:
- Pheochromocytoma-like hypersecretion of catecholamines (rarely)
- Non-functional tumors (40%) usually present with:
- Abdominal or flank pain
- Asymptomatic
- Detected incidentally
Physical Examination
- All patients with suspected adrenocortical carcinoma should be carefully examined for the signs and symptoms of following as mentioned above:
- Cushing's syndrome
- Virilization
- Conn syndrome
- Feminization
Laboratory findings
- Hormonal syndromes should be confirmed with laboratory testing
- Laboratory findings in Cushing syndrome include:
- Increased serum glucose
- Increased urinary cortisol levels
- Adrenal virilization is confirmed by the finding of:
- Increased serum androstenedione
- Increased serum dehydroepiandrosterone
- Laboratory findings in Conn syndrome include:
- Low serum potassium (hypokalemia)
- Low plasma renin activity
- High serum aldosterone
- Feminization is confirmed with the finding of:
- Increased serum estrogen
Imaging studies
CT
- Radiological studies of the abdomen, such as CT scans and magnetic resonance imaging are useful for identifying the site of the tumor, differentiating it from other diseases, such as adrenocortical adenoma, and determining the extent of invasion of the tumor into surrounding organs and tissues.[20]
- CT scans of the chest and bone scans are routinely performed to look for metastases to the lungs and bones respectively. These studies are critical in determining whether or not the tumor can be surgically removed, the only potential cure at this time.
PET scan
Molecular imaging
- Iodometomidate (IMTO) as tracer for molecular imaging of cytochrome P450 family 11B (Cyp11B) enzymes[23]
Treatment
Medical Therapy
- Radiation therapy and radiofrequency ablation may be used for palliation in patients who are not surgical candidates
- Chemotherapy regimens typically include the drug mitotane, an inhibitor of steroid synthesis which is toxic to cells of the adrenal cortex, as well as standard cytotoxic drugs. One widely used regimen consists of cisplatin, doxorubicin, etoposide) and mitotane. The endocrine cell toxin streptozotocin has also been included in some treatment protocols. Chemotherapy may be given to patients with unresectable disease, to shrink the tumor prior to surgery (neoadjuvant chemotherapy), or in an attempt to eliminate microscopic residual disease after surgery (adjuvant chemotherapy).
- Hormonal therapy with steroid synthesis inhibitors such as aminoglutethimide may be used in a palliative manner to reduce the symptoms of hormonal syndromes
Surgery
- The only curative treatment is complete surgical excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the renal vein or inferior vena cava
- The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates
References
- ↑ "Adrenocortical Carcinoma Treatment - National Cancer Institute".
- ↑ Weiss LM (1984). "Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors". Am J Surg Pathol. 8 (3): 163–9. PMID 6703192.
- ↑ "Mechanism of abnormal production of adrenal androgens in patients with adrenocortical adenomas and carcinomas | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
- ↑ "Variable Expression of the Transcription Factors cAMP Response Element-Binding Protein and Inducible cAMP Early Repressor in the Normal Adrenal Cortex and in Adrenocortical Adenomas and Carcinomas | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
- ↑ Fassnacht M, Libé R, Kroiss M, Allolio B (2011). "Adrenocortical carcinoma: a clinician's update". Nat Rev Endocrinol. 7 (6): 323–35. doi:10.1038/nrendo.2010.235. PMID 21386792.
- ↑ Menon V, Krishnamurthy SV (2006). "Adrenocortical carcinomas: a 12-year clinicopathologic study of 15 cases". Indian J Pathol Microbiol. 49 (1): 7–11. PMID 16625963.
- ↑ Magro G, Esposito G, Cecchetto G, Dall'Igna P, Marcato R, Gambini C; et al. (2012). "Pediatric adrenocortical tumors: morphological diagnostic criteria and immunohistochemical expression of matrix metalloproteinase type 2 and human leucocyte-associated antigen (HLA) class II antigens. Results from the Italian Pediatric Rare Tumor (TREP) Study project". Hum Pathol. 43 (1): 31–9. doi:10.1016/j.humpath.2011.04.016. PMID 21820153.
- ↑ Hsing AW, Nam JM, Co Chien HT, McLaughlin JK, Fraumeni JF (1996). "Risk factors for adrenal cancer: an exploratory study". Int J Cancer. 65 (4): 432–6. doi:10.1002/(SICI)1097-0215(19960208)65:4<432::AID-IJC6>3.0.CO;2-Y. PMID 8621222.
- ↑ Weiss LM, Medeiros LJ, Vickery AL (1989). "Pathologic features of prognostic significance in adrenocortical carcinoma". Am J Surg Pathol. 13 (3): 202–6. PMID 2919718.
- ↑ Moreno S, Montoya G, Armstrong J, Leteurtre E, Aubert S, Vantyghem MC; et al. (2004). "Profile and outcome of pure androgen-secreting adrenal tumors in women: experience of 21 cases". Surgery. 136 (6): 1192–8. doi:10.1016/j.surg.2004.06.046. PMID 15657575.
- ↑ Wagner M, Walter PR, Ghnassia JP, Gasser B (1993). "[Adrenocortical tumors. I. Prognostic evaluation of a series of 17 cases using the Weiss criteria]". Ann Pathol. 13 (5): 306–11. PMID 8311856.
- ↑ Gandour MJ, Grizzle WE (1986). "A small adrenocortical carcinoma with aggressive behavior. An evaluation of criteria for malignancy". Arch Pathol Lab Med. 110 (11): 1076–9. PMID 3778125.
- ↑ Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A (2010). "Weiss criteria in large adrenocortical tumors: a validation study". Indian J Pathol Microbiol. 53 (2): 222–6. doi:10.4103/0377-4929.64325. PMID 20551521.
- ↑ "Hypoaldosteronism accompanied by normal or elevated mineralocorticosteroid pathway steroid: a marker of adrenal carcinoma. | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
- ↑ "ADRENAL CORTICAL CARCINOMA IN A MALE WITH EXCESS GONADOTROPIN IN THE URINE | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
- ↑ "UpToDate".
- ↑ Allolio B, Fassnacht M (2006). "Clinical review: Adrenocortical carcinoma: clinical update". J Clin Endocrinol Metab. 91 (6): 2027–37. doi:10.1210/jc.2005-2639. PMID 16551738.
- ↑ Sandrini R, Ribeiro RC, DeLacerda L (1997). "Childhood adrenocortical tumors". J Clin Endocrinol Metab. 82 (7): 2027–31. doi:10.1210/jcem.82.7.4057. PMID 9215267.
- ↑ Moreno S, Guillermo M, Decoulx M, Dewailly D, Bresson R, Proye Ch (2006). "Feminizing adreno-cortical carcinomas in male adults. A dire prognosis. Three cases in a series of 801 adrenalectomies and review of the literature". Ann Endocrinol (Paris). 67 (1): 32–8. PMID 16596055.
- ↑ Szolar DH, Korobkin M, Reittner P, Berghold A, Bauernhofer T, Trummer H; et al. (2005). "Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT". Radiology. 234 (2): 479–85. doi:10.1148/radiol.2342031876. PMID 15671003.
- ↑ Groussin L, Bonardel G, Silvéra S, Tissier F, Coste J, Abiven G; et al. (2009). "18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients". J Clin Endocrinol Metab. 94 (5): 1713–22. doi:10.1210/jc.2008-2302. PMID 19190108.
- ↑ Khan TS, Sundin A, Juhlin C, Långström B, Bergström M, Eriksson B (2003). "11C-metomidate PET imaging of adrenocortical cancer". Eur J Nucl Med Mol Imaging. 30 (3): 403–10. doi:10.1007/s00259-002-1025-9. PMID 12634969.
- ↑ Hahner S, Stuermer A, Kreissl M, Reiners C, Fassnacht M, Haenscheid H; et al. (2008). "[123 I]Iodometomidate for molecular imaging of adrenocortical cytochrome P450 family 11B enzymes". J Clin Endocrinol Metab. 93 (6): 2358–65. doi:10.1210/jc.2008-0050. PMID 18397978.