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| | At diagnosis | After COJEC | After DB |
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| Patient 1 | (i) Primary tumor in left adrenal gland with metastases in multiple abdominal and pelvic lymph nodes
(ii) Intra- and retroperitoneal masses ≤5 cm on MRI
(iii) CNS and BM involvement
(iv) Very high tumor marker levels (NSE, HVA, VMA) | (i) Shrinkage of tumors to ≤2 cm on MRI
(ii) Total response according to BM involvement
(iii) PR according to CNS involvement | After 1 cycle of DB and 2 cycles of TVD:
(i) Further shrinkage of tumors on MRI
(ii) NSE, HVA, and VMA levels normalized
(iii) No evidence of leptomeningeal thickening and solid CNS metastases on CNS MRI
(iv) No BM involvement
After surgery:
(i) 100% tumor necrosis
After 1 cycle of TVD, 1 cycle of DB, BuMel + ASCT, and 4 cycles of DB:
(i) No residual tumors in the abdomen and no signs of leptomeningeal thickening on MRI
(ii) No BM involvement
(iii) Patient in full remission |
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| Patient 2 | (i) Primary tumor in left adrenal gland with enlarged para-aortic and paracaval lymph nodes
(ii) Total BM involvement
(iii) High tumor marker levels (NSE, HVA, VMA) | (i) PR in primary tumor and local lymph nodes on MRI
(ii) No BM involvement | After 1 cycle of DB and surgery:
(i) 98% tumor necrosis
After another cycle of DB:
(i) NSE, HVA, and VMA levels normalized
After BuMel + ASCT and 3 cycles of DB:
(i) No residual tumors on MRI
(ii) No BM involvement
(iii) Patient in full remission |
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| Patient 3 | (i) Primary tumor in left adrenal gland with bone metastasis on mandibula and local cervical lymphadenitis
(ii) Total BM involvement
(iii) High tumor marker levels (NSE, HVA, VMA) | (i) Patient developed severe pancytopenia due to BM progression accompanied by secondary paraneoplastic membraneous glomerulopathy
(ii) Higher HVA and VMA than those at diagnosis
(iii) Shrinkage of primary tumor and metastasis on mandibula and full regression in cervical lymph nodes on MRI
(iv) Both kidneys were 2 cm larger than on the initial MRI, without focal lesions and with a normal ratio
(v) Nearly total BM involvement | After 3 cycles of DB and I/T:
(i) Size of kidneys and renal function normalized
(ii) No BM involvement
(iii) Tumor markers normalized
After surgery and radiotherapy:
(i) No residual tumors on MRI
After BuMel + ASCT and another 3 cycles of DB:
(i) Patient in full remission according to tumor markers, trephine biopsy, CNS and abdominal MRI, and DOTA |
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