Background
Methods
Pathological review
Antibody
|
Company and clone
|
Target retrieval solution (TRS)
|
Detection
|
Dilution
|
Link to spec sheet
|
---|---|---|---|---|---|
Beta catenin | Dako, B-catenin-1 | High | Flex | 1:200 | Monoclonal mouse anti-human beta-catenin, clone β-catenin-1 |
Inhibin | Dako, R1 | High | Flex | 1:5 | Monoclonal mouse anti-human inhibin α, clone R1 |
Ki67 | Dako, MIB1 | High | Flex | 1:50 | Monoclonal mouse anti-human Ki-67 antigen, clone MIB-1 |
Ki67 decal | Dako, MIB1 | High | Flex | 1:20 | Same as above |
p53 | Dako, D0-7 | High | Flex | 1:100 | Monoclonal mouse anti-human p53 protein, clone DO-7 |
SF-1 | Invitrogen | Dako3in1 high pH on Dako PT instrument | Dako-envision + mouse | 1:150 | Monoclonal mouse [Mm] IgG1 anti-human clone N1665 |
Literature review
Results
Pathological review
Case number
|
Age
|
Sex
|
Ki-67 (%)
|
Β-catenin intracellular accumulation
|
P53 (%)
|
Inhibin
|
SF-1
|
---|---|---|---|---|---|---|---|
1 | 59 | M | 80 | Present | 85 | Positive | Positive |
2 | 25 | F | 40 | Present | 30 | Negative | Positive |
3 | 65 | M | 20 | Absent | 30 | Negative | Negative |
4 | 66 | F | 50 | Absent | Focal | Positive | Tissue not available |
5 | 79 | M | 10 | Present | 10 | Positive | Positive |
6 | 34 | F | 85 | Present | 90 | Negative | Positive |
7 | 68 | F | 60 | Absent | 60 | Negative | Negative |
8 | 32 | F | 10 | Absent | Absent | Negative | Positive |
Clinical review
Case number
|
Age
|
Sex
|
Presentation
|
Time to recurrence (days)
|
Time to death (days)
|
---|---|---|---|---|---|
1 | 59 | M | Cushingoid | N/A | N/A |
2 | 25 | F | Incidentally | 119 | N/A |
3 | 65 | M | Incidentally | N/A | N/A |
4 | 66 | F | Metastatic | 0 | 25 |
5 | 79 | M | Incidentally | N/A | 353 |
6 | 34 | F | Incidentally | 195 | 254 |
7 | 68 | F | Incidentally | 68 | 223 |
8 | 32 | F | Incidentally | N/A | N/A |
Sarcomatoid cases
Discussion
Epidemiology and clinical presentation
Reference
|
Published year
|
Country
|
Database
|
Number of patients
|
Average Age (years)
|
Male-to-female ratio
|
Median tumor size (cm)
|
Proportion of tumors with distant metastasis at presentation (%)
|
---|---|---|---|---|---|---|---|---|
Kutikov et al. [4] | 2011 | United States | National Cancer Database | 4,275 | 54.5 | 41.9% to 58.1% | 11.5 | 34.4 |
Kebebew et al. [3] | 2006 | United States | Surveillance, Epidemiology, and End Results (SEER) Database | 725 | 51.2 | 45.9% to 54.1% | 12 | 34.8 |
Fassnacht et al. [8] | 2009 | Germany | The German ACC Registry | 416 | 46.7 | 37.3% to 62.7% | 11.3 | 29.3 |
Else et al. [9] | 2014 | United States | Michigan Endocrine Oncology Repository | 391 | 47.4 | 40% to 60% | 11.8 | 29 |
Kerkhofs et al. [2] | 2013 | Netherlands | Netherlands Cancer Registry | 359 | 56 | 45% to 55% | Not available | 35 |
Ayala-Ramirez et al. [10] | 2013 | United States | Tumor Registry Database at the University of Texas MD Anderson Cancer Center | 330 | 48.5 | 35.8% to 64.2% | 11 cm | 25.8 |
Diagnosis
Imaging
Histopathology
Criteria
|
Numeric value
|
---|---|
Histological | |
Diffuse growth pattern | 0.92 |
Vascular invasion | 0.92 |
Tumor cell necrosis | 0.69 |
Broad fibrous bands | 1.00 |
Capsular invasion | 0.37 |
Mitotic index (>1/10 HPFs) | 0.60 |
Pleomorphism | 0.39 |
Nonhistologic | |
Tumor mass (>100 g) | 0.60 |
Urinary 17-ketosteroids (10 mg/1 g creatinine 24 h) | 0.50 |
Response to ACTH (17-hydroxysteroids increased two times after 50 mcg of IV ACTH) | 0.42 |
Cushing syndrome with virilism, virilism alone, or no clinical manifestations | 0.42 |
Weight loss (>10 lb/3 months) | 2.00 |
Criteria
|
Numeric value
|
---|---|
Regressive changes such as necrosis, hemorrhage, fibrosis, or calcification | 5.7 |
Loss of normal structure | 1.6 |
Nuclear atypia | 2.1 |
Nuclear hyperchromasia | 2.6 |
Abnormal nucleoli structure | 4.1 |
Mitotic activity >2/10 HPFs | 9.0 |
Capsular or vascular invasion | 3.3 |
Immunohistochemistry
Pathogenesis
Staging and prognosis
-
Stage I - tumor size less than or equal to 5 cm; 82%.
-
Stage II - tumor size greater than 5 cm; 58%.
-
Stage III - any tumor size with at least one positive lymph node or tumor infiltrating into surrounding adipose tissue or adjacent organs including the presence of venous tumor thrombus in the inferior vena cava or renal vein; 55%.
-
Stage IV - any metastatic disease; 18%.
Conclusions
Reference
|
Age
|
Sex
|
Clinical presentation
|
Laterality
|
Size
|
Treatment
|
Time to recurrence
|
Time to death
|
---|---|---|---|---|---|---|---|---|
Okazumi et al. [74] | 46 | M | Abdominal distention + back pain | Right | 14 cm | Right adrenalectomy and nephrectomy followed by removal of the tumor thrombus | 5 months | 206 days |
Collina et al. [75] | 68 | F | Abdominal discomfort | Right | 11 cm | Surgical resection followed by radiotherapy after tumor recurrence | 2 months | 6 months |
Decorato et al. [76] | 42 | F | Abdominal pain | Left | 19 cm | Surgical resection | 3 months | 7 months |
Fischler et al. [77] | 29 | F | Virilization | Left | 12.5 cm | Nephroadrenalectomy and splenectomy followed by systemic chemotherapy (cisplatin and etoposide) after recurrence | 4 months | 8 months |
Barksdale et al. [78] | 79 | F | Severe hypertension | Right | 9 cm | Right adrenalectomy and cavotomy | 4 months | Not reported |
Lee et al. [79] | 61 | M | Flank pain + hypertension | Right | 12 cm | Radical nephrectomy and right hepatic lobectomy | No recurrence noted | 2 days |
Sturm et al. [7] | 31 | M | Abdominal pain | Left | 12 cm | Adrenalectomy followed by systemic chemotherapy (VP16-cisplatinum) after recurrence | 2 months | 3 months |
Coli et al. (2009) [80] | 75 | F | Abdominal pain | Left | 15 cm | Adrenalectomy and splenectomy | 3 months | 12 months |
Feng et al. [81] | 72 | M | Left lumbar pain | Left | 7.1 cm on CT scan | Surgical resection | Not reported | Not reported |
Sasaki et al. [82] | 45 | M | Abdominal pain, fever, nausea, vomiting, anorexia, hypertension | Left | 17 cm | Radical nephrectomy, splenectomy, distal pancreatectomy, left partial colectomy, and wedge biopsy of one hepatic lesion | Hepatic metastasis at presentation. Locoregional recurrence at 3 months | 3 months |
Bertolini et al. [83] | 23 | F | Incidentally during work-up of metastatic rectal mass | Left | 14 cm | Left adrenalectomy with systemic chemotherapy for metastatic rectal cancer | Not reported, however patient had metastatic lesions on presentation which were presumed to be rectal cancer based on the co-existence of a metastatic rectal cancer lesion in the adrenal gland | 14 months |
Thway et al. [84] | 45 | M | Abdominal bloating + back pain | Left | 24 cm | Left radical nephrectomy and splenectomy followed by palliative chemotherapy (vincristine, ifosfamide, carboplatin, doxorubicin, and etoposide) | Metastatic at presentation | 11 months |
Yan et al. [85] | 72 | M | Flank pain | Right | 13 cm | Adrenalectomy | 2 years | 2.5 years |
Kao et al. [86] | 48 | F | Abdominal pain + hypokalemia + weight loss | Right | 15 cm | Adrenalectomy, partial nephrectomy, and partial hepatectomy followed by systemic chemotherapy (cisplatin and ifosfamide) after distant metastasis | 2 months | Alive with disease at 7 month follow-up |
Mark et al. [87] | 58 | M | Flank pain | Right | 12 cm | Radial nephrectomy followed by eternal beam radiotherapy to the tumor site | Not reported after 16 month follow-up | Not reported after 16 month follow-up |
Shaikh et al. [88] | 62 | F | Abdominal pain | Right | 6.5 cm | Adrenalectomy | 3 months | 4 months |