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==Surgery==
==Surgery==
*Surgery is the mainstay of treatment for adrenocortical carcinoma.
*Surgery is the mainstay of treatment for adrenocortical carcinoma.
*Surgery can also be performed even in the case of the invasion into large blood vessels, such as the [[renal vein]] or [[inferior vena cava]].
*[[Adrenalectomy]] can also be performed even in the case of the invasion into large [[blood vessels]], such as the [[renal vein]] or [[inferior vena cava]].
*A large percentage of patients are not surgical candidates.
*A large percentage of patients are not surgical candidates.
Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome.  
*Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome.
*Preoperative imaging should be obtained to evaluate the extent of the [[tumor]].
*Imaging should be done just before the surgery because ACC grow quickly and involvement of adjacent structures may change.
*Optimization of [[hormone]] excess in patients with functional [[Mass|masses]] especially those with [[Cushing's syndrome|Cushing’s syndrome]] should be done.
*Patients widespread distant [[Metastasis|metastatic]] disease in multiple organs or those with multiple metastatic deposits in one organ system unable to be completely resected should not undergo [[adrenalectomy]]. The primary [[tumor]] can instead be treated with [[External beam radiotherapy|external beam radiation]] for [[Palliative care|palliation]] along with other adjuncts to improve local symptoms and better control [[hormone]] excess if present.<ref name="pmid20675074">{{cite journal| author=Sabolch A, Feng M, Griffith K, Hammer G, Doherty G, Ben-Josef E| title=Adjuvant and definitive radiotherapy for adrenocortical carcinoma. | journal=Int J Radiat Oncol Biol Phys | year= 2011 | volume= 80 | issue= 5 | pages= 1477-84 | pmid=20675074 | doi=10.1016/j.ijrobp.2010.04.030 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20675074  }}</ref>
*Obstruction of the [[Inferior vena cava|vena cava]] by [[tumor]] [[thrombus]] can lead to significant lower body [[edema]]. If [[tumor]] resection is not technically operable, [[Vena cavae|vena cava]] [[stents]] can be placed, leading to temporary prevention of occlusion.
*[[Debulking]] for control of [[hormone]] excess in the setting of known [[Metastasis|metastatic]] disease is also performed in some situations. The benefits of [[debulking]] must outweigh the risks of surgery in these patients who have [[Wound healing phases|poor wound healing]].


Preoperative imaging should be obtained to evaluate the extent of the tumor.
* [[Lymph nodes]] should be removed as part of the en bloc [[resection]]. The [[lymphadenectomy]] is performed based on the [[arterial]] supply of [[Adrenal mass causes|adrenal tumors]].<ref name="pmid22306837">{{cite journal| author=Gaujoux S, Brennan MF| title=Recommendation for standardized surgical management of primary adrenocortical carcinoma. | journal=Surgery | year= 2012 | volume= 152 | issue= 1 | pages= 123-32 | pmid=22306837 | doi=10.1016/j.surg.2011.09.030 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22306837  }}</ref>
 
* Risk of severe [[bleeding]] and benefits of massive removal of [[Lymph node|lymph nodes]] should be balanced by surgeons in every case.<ref name="pmid22143204">{{cite journal| author=Reibetanz J, Jurowich C, Erdogan I, Nies C, Rayes N, Dralle H et al.| title=Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. | journal=Ann Surg | year= 2012 | volume= 255 | issue= 2 | pages= 363-9 | pmid=22143204 | doi=10.1097/SLA.0b013e3182367ac3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22143204  }}</ref>
Imaging should be done just before the surgery because ACC grow quickly and involvement of adjacent structures may change
* Published data comparing the efficacy of LA vs OA for ACC are limited.
 
Optimization of hormone excess in patients with functional masses especially those with Cushing’s syndrome
 
Patients widespread distant metastatic disease in multiple organs or those with multiple metastatic deposits in one organ system unable to be completely resected should not undergo adrenalectomy.
 
The primary tumor can instead be treated with external beam radiation for palliation along with other adjuncts to improve local symptoms and better control hormone excess,
 
if present (247).
 
Obstruction of the vena cava by tumor thrombus can lead to significant lower body edema, which leads to significant patient suffering. If tumor resection is not technically operable, vena cava stents can be placed, leading to temporary prevention of occlusion.
 
Debulking for control of hormone excess in the setting of known metastatic disease is also performed in some situations. The benefits of debulking must outweigh the risks of surgery in these patients who have poor wound healing and lengthy recovery periods due to preexisting debilitation
* Lymph nodes ideally should be removed as part of the en bloc resection. The lymphadenectomy is performed based on the arterial supply of adrenal tumors. 248)
* Risk of severe bleeding and benefits of massive removal of lymph nodes should be balanced by surgeons in every case. (249)
Published data comparing the efficacy of LA vs OA for ACC are limited.
{| class="wikitable"
{| class="wikitable"
!Laparoscopic adrenalectomy (LA)
![[Laparoscopic surgery|Laparoscopic]] adrenalectomy (LA)
!Open adrenalectomy (OA)  
!Open adrenalectomy (OA)  
|-
|-
|
|
* LA has shown lower morbidity, less pain, shorter hospital stays, and decreased overall time to recovery when compared with open adrenalectomy  
* LA has shown lower morbidity, less pain, shorter hospital stays, and decreased recovery time if compared with open adrenalectomy  
* Application of laparoscopic instruments can result in the shedding of malignant cells. 246, 251
* Application of laparoscopic instruments can result in the shedding of malignant cells.<ref name="pmid23158185">{{cite journal| author=Miller BS, Gauger PG, Hammer GD, Doherty GM| title=Resection of adrenocortical carcinoma is less complete and local recurrence occurs sooner and more often after laparoscopic adrenalectomy than after open adrenalectomy. | journal=Surgery | year= 2012 | volume= 152 | issue= 6 | pages= 1150-7 | pmid=23158185 | doi=10.1016/j.surg.2012.08.024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23158185  }}</ref>
* Local recurrence and peritoneal carcinomatosis were more common in the LA group.  
* Recurrence and [[Peritoneal|peritonea]]<nowiki/>l spread were more common in the LA group.<ref name="pmid16360394">{{cite journal| author=Gonzalez RJ, Shapiro S, Sarlis N, Vassilopoulou-Sellin R, Perrier ND, Evans DB et al.| title=Laparoscopic resection of adrenal cortical carcinoma: a cautionary note. | journal=Surgery | year= 2005 | volume= 138 | issue= 6 | pages= 1078-85; discussion 1085-6 | pmid=16360394 | doi=10.1016/j.surg.2005.09.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16360394  }}</ref>
* Shorter disease-free interval and but with a higher incidence of incomplete resections.
* Higher [[incidence]] of incomplete [[Resection|resections]] is found in LA.
* Peritoneal carcinomatosis occurred in only 25%  
* [[Peritoneal carcinomatosis]] ([[Spread of the cancer|spread]]) occurred in only 25% of cases.
* Recurrence rate of 86% in the OA group
* Recurrence rate is 100%.<ref name="pmid19851811">{{cite journal| author=Grubbs EG, Callender GG, Xing Y, Perrier ND, Evans DB, Phan AT et al.| title=Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane. | journal=Ann Surg Oncol | year= 2010 | volume= 17 | issue= 1 | pages= 263-70 | pmid=19851811 | doi=10.1245/s10434-009-0716-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19851811  }}</ref>
* 16% of patients had positive margins.
* 16% of patients had positive margins after the surgery.
|
|
* American Association of Clinical Endocrinologists and the American Association of Endocrine Surgeons recommended OA as the procedure of choice (254).  
* American Association of Clinical Endocrinologists and the American Association of Endocrine Surgeons recommended OA as the procedure of choice.<ref name="pmid19632968">{{cite journal| author=Zeiger MA, Thompson GB, Duh QY, Hamrahian AH, Angelos P, Elaraj D et al.| title=American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas: executive summary of recommendations. | journal=Endocr Pract | year= 2009 | volume= 15 | issue= 5 | pages= 450-3 | pmid=19632968 | doi=10.4158/EP.15.5.450 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19632968  }}</ref>
* Peritoneal spread is 60%with significantly earlier recurrence.
* the [[Peritoneal carcinomatosis|peritoneal spread]] is 60% with significantly earlier recurrence.


* 30% of patients had positive margins.
* 30% of patients had positive margins.
 
* Recurrence rate of 86% in the OA group
* Recurrence rate is 100%. (251, 257).
* Survival for patients with stage 2 was longer in those undergoing OA.
* Survival for patients with stage 2 was longer in those undergoing OA
|}
|}


==== '''''Surgical approach''''' ====
==== '''''Surgical approach''''' ====
1. Incision and exploration of the peritoneal cavity.
1. [[Incision]] and [[exploration]] of the [[peritoneal cavity]].
 
2. Evaluation of liver for metastasis
 
3. Containment A self-retaining retractor system with towels or laparotomy pads should be placed in such a way as


to exclude the rest of the peritoneal cavity from the area of the tumor and other organs requiring
2. Evaluation of [[liver]] for [[metastasis]]


resection.4. Mobilization of organs
3. Containment A self-retaining retractor system with towels or [[laparotomy]] pads should be placed in such a way as to exclude the rest of the [[peritoneal cavity]] from the area of the [[tumor]] and other organs requiring [[resection]].


adjacent to tumor
4. Mobilization of organs adjacent to [[tumor]]


5. En bloc resection. Preserve any tissue overlying the tumor.
5. En bloc [[resection]]. Preserve any tissue overlying the [[tumor]]


6. Regional lymphadenectomy
6. Regional [[lymphadenectomy]]


7. Provide intact en bloc specimen for pathologic review
7. Provide intact en bloc specimen for [[Pathology|pathologic]] review


8. Mark field to facilitate postsurgical external beam radiation therapy
8. Mark field to facilitate postsurgical [[External beam radiotherapy|external beam radiation therapy]]


9. Dictate operative report
9. Dictate operative report


==== '''''Recurrence of ACC''''' ====
==== '''''Recurrence of ACC''''' ====
* The number of organs involved by tumor at the time of the first metastasis is a predictor of survival (243, 265).
* The number of organs involved by tumor at the time of the first metastasis is a predictor of survival.<ref name="pmid23150691">{{cite journal| author=Erdogan I, Deutschbein T, Jurowich C, Kroiss M, Ronchi C, Quinkler M et al.| title=The role of surgery in the management of recurrent adrenocortical carcinoma. | journal=J Clin Endocrinol Metab | year= 2013 | volume= 98 | issue= 1 | pages= 181-91 | pmid=23150691 | doi=10.1210/jc.2012-2559 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23150691  }}</ref>
* University of Michigan data show the site of first metastasis can also be used to predict survival
* University of Michigan data show the site of first [[metastasis]] can also be used to predict survival.
* Recurrence in the peritoneum outside the tumor bed having the worst survival.
* Recurrence in the [[peritoneum]] outside the tumor bed having the worst survival.
* Surgery is indicated in those patients with disease confined to 1 site or organ.
* Surgery is indicated in those patients with disease confined to 1 site or organ.
* waiting 3 months while treating with chemotherapy to assess for tumor responsiveness of progression. If progression is not rapid, surgery may proceed with greater benefit.
* Waiting 3 months while treating with [[chemotherapy]] to assess for [[tumor]] responsiveness of progression. If progression is not rapid, surgery may proceed with greater benefit.<ref name="pmid20694732">{{cite journal| author=Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM| title=Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade. | journal=Langenbecks Arch Surg | year= 2010 | volume= 395 | issue= 7 | pages= 955-61 | pmid=20694732 | doi=10.1007/s00423-010-0698-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20694732  }}</ref>
* Median survival of 74 months in those undergoing complete second resections vs a median survival of 16 months in those undergoing incomplete second resection. (243, 245).
* Median survival of 74 months in those undergoing complete second resections and a median survival of 16 months in those undergoing incomplete second resection.<ref name="pmid17062775">{{cite journal| author=Assié G, Antoni G, Tissier F, Caillou B, Abiven G, Gicquel C et al.| title=Prognostic parameters of metastatic adrenocortical carcinoma. | journal=J Clin Endocrinol Metab | year= 2007 | volume= 92 | issue= 1 | pages= 148-54 | pmid=17062775 | doi=10.1210/jc.2006-0706 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17062775  }}</ref>


==References==
==References==

Revision as of 13:37, 25 September 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Surgery is the mainstay of treatment for adrenocortical carcinoma.

Surgery

  • Surgery is the mainstay of treatment for adrenocortical carcinoma.
  • Adrenalectomy can also be performed even in the case of the invasion into large blood vessels, such as the renal vein or inferior vena cava.
  • A large percentage of patients are not surgical candidates.
  • Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome.
  • Preoperative imaging should be obtained to evaluate the extent of the tumor.
  • Imaging should be done just before the surgery because ACC grow quickly and involvement of adjacent structures may change.
  • Optimization of hormone excess in patients with functional masses especially those with Cushing’s syndrome should be done.
  • Patients widespread distant metastatic disease in multiple organs or those with multiple metastatic deposits in one organ system unable to be completely resected should not undergo adrenalectomy. The primary tumor can instead be treated with external beam radiation for palliation along with other adjuncts to improve local symptoms and better control hormone excess if present.[1]
  • Obstruction of the vena cava by tumor thrombus can lead to significant lower body edema. If tumor resection is not technically operable, vena cava stents can be placed, leading to temporary prevention of occlusion.
  • Debulking for control of hormone excess in the setting of known metastatic disease is also performed in some situations. The benefits of debulking must outweigh the risks of surgery in these patients who have poor wound healing.
Laparoscopic adrenalectomy (LA) Open adrenalectomy (OA)
  • LA has shown lower morbidity, less pain, shorter hospital stays, and decreased recovery time if compared with open adrenalectomy
  • Application of laparoscopic instruments can result in the shedding of malignant cells.[4]
  • Recurrence and peritoneal spread were more common in the LA group.[5]
  • Higher incidence of incomplete resections is found in LA.
  • Peritoneal carcinomatosis (spread) occurred in only 25% of cases.
  • Recurrence rate is 100%.[6]
  • 16% of patients had positive margins after the surgery.
  • American Association of Clinical Endocrinologists and the American Association of Endocrine Surgeons recommended OA as the procedure of choice.[7]
  • the peritoneal spread is 60% with significantly earlier recurrence.
  • 30% of patients had positive margins.
  • Recurrence rate of 86% in the OA group
  • Survival for patients with stage 2 was longer in those undergoing OA.

Surgical approach

1. Incision and exploration of the peritoneal cavity.

2. Evaluation of liver for metastasis

3. Containment A self-retaining retractor system with towels or laparotomy pads should be placed in such a way as to exclude the rest of the peritoneal cavity from the area of the tumor and other organs requiring resection.

4. Mobilization of organs adjacent to tumor

5. En bloc resection. Preserve any tissue overlying the tumor

6. Regional lymphadenectomy

7. Provide intact en bloc specimen for pathologic review

8. Mark field to facilitate postsurgical external beam radiation therapy

9. Dictate operative report

Recurrence of ACC

  • The number of organs involved by tumor at the time of the first metastasis is a predictor of survival.[8]
  • University of Michigan data show the site of first metastasis can also be used to predict survival.
  • Recurrence in the peritoneum outside the tumor bed having the worst survival.
  • Surgery is indicated in those patients with disease confined to 1 site or organ.
  • Waiting 3 months while treating with chemotherapy to assess for tumor responsiveness of progression. If progression is not rapid, surgery may proceed with greater benefit.[9]
  • Median survival of 74 months in those undergoing complete second resections and a median survival of 16 months in those undergoing incomplete second resection.[10]

References

  1. Sabolch A, Feng M, Griffith K, Hammer G, Doherty G, Ben-Josef E (2011). "Adjuvant and definitive radiotherapy for adrenocortical carcinoma". Int J Radiat Oncol Biol Phys. 80 (5): 1477–84. doi:10.1016/j.ijrobp.2010.04.030. PMID 20675074.
  2. Gaujoux S, Brennan MF (2012). "Recommendation for standardized surgical management of primary adrenocortical carcinoma". Surgery. 152 (1): 123–32. doi:10.1016/j.surg.2011.09.030. PMID 22306837.
  3. Reibetanz J, Jurowich C, Erdogan I, Nies C, Rayes N, Dralle H; et al. (2012). "Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma". Ann Surg. 255 (2): 363–9. doi:10.1097/SLA.0b013e3182367ac3. PMID 22143204.
  4. Miller BS, Gauger PG, Hammer GD, Doherty GM (2012). "Resection of adrenocortical carcinoma is less complete and local recurrence occurs sooner and more often after laparoscopic adrenalectomy than after open adrenalectomy". Surgery. 152 (6): 1150–7. doi:10.1016/j.surg.2012.08.024. PMID 23158185.
  5. Gonzalez RJ, Shapiro S, Sarlis N, Vassilopoulou-Sellin R, Perrier ND, Evans DB; et al. (2005). "Laparoscopic resection of adrenal cortical carcinoma: a cautionary note". Surgery. 138 (6): 1078–85, discussion 1085-6. doi:10.1016/j.surg.2005.09.012. PMID 16360394.
  6. Grubbs EG, Callender GG, Xing Y, Perrier ND, Evans DB, Phan AT; et al. (2010). "Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane". Ann Surg Oncol. 17 (1): 263–70. doi:10.1245/s10434-009-0716-x. PMID 19851811.
  7. Zeiger MA, Thompson GB, Duh QY, Hamrahian AH, Angelos P, Elaraj D; et al. (2009). "American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas: executive summary of recommendations". Endocr Pract. 15 (5): 450–3. doi:10.4158/EP.15.5.450. PMID 19632968.
  8. Erdogan I, Deutschbein T, Jurowich C, Kroiss M, Ronchi C, Quinkler M; et al. (2013). "The role of surgery in the management of recurrent adrenocortical carcinoma". J Clin Endocrinol Metab. 98 (1): 181–91. doi:10.1210/jc.2012-2559. PMID 23150691.
  9. Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM (2010). "Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade". Langenbecks Arch Surg. 395 (7): 955–61. doi:10.1007/s00423-010-0698-y. PMID 20694732.
  10. Assié G, Antoni G, Tissier F, Caillou B, Abiven G, Gicquel C; et al. (2007). "Prognostic parameters of metastatic adrenocortical carcinoma". J Clin Endocrinol Metab. 92 (1): 148–54. doi:10.1210/jc.2006-0706. PMID 17062775.

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