Extramedullary breast relapse of acute
lymphoblastic leukemia after stem cell
transplantation in a child
Dr. Gülsün Karasu
Bahçeşehir University Schoool of Medicine
Pediatric Stem Cell Transplantation Unit
1
Case presentation
• July 2010
• 9-year-old girl presented with a 1-month history of fatigue,
weakness and low grade intermittent fever since 2 days.
• Past history was unremarkable.
• Family history was unremarkable.
2
Physical examination
• Mild pallor
• Hepatosplenomegaly
Hematological investigation
• Hb: 7.9 g/dL
• WBC: 38.000/mm3
• Platelet: 40.000/mm3
3
Bone marrow aspiration
CALLA (+)
4
Conventional cytogenetic: 46XX
FISH
t(12:21): (-)
t(4:11): (-)
t(9:22) (-)
5
She received ALLIC 2009 protocol
She received prophylactic cranial radiotherapy
July 2010 – August 2012
6
March 2015:
Hb: 11.2 gr/dL
Isolated BM relapse
WBC: 12900/mm3
PLT: 67.000/mm3
REZ-ALL BFM protocol
Donor search
7
• October 2015 : Referral for transplantation
• Bone Marrow Aspiration: Not in remission
IDA-FLAG: 7-11.10.2015
8
Transplantation: 11.30.2015
• Donor: MSD (sister)
• Conditioning regimen: TBI +Etoposide
• GVHD prophylaxis: CsA+ Mtx
• Stem cell source: PBSC
• TNC: 9.1 X 108/kg-CD34: 12X106kg
9
Transplantation: 11.30.2015
• Neutrophil engraftment: +12
• Platelet engraftment: +17
• Day +18: CsA
MMF (nephrotoxicity)
10
Posttransplant day
+14
+28
+60
+100
Chimerism
91%
90%
93%
100% (BM)
Bone marrow morphological evaluation at day 100 indicated CR
The posttransplant course was uneventful without GVHD.
11
Five months after HSCT,
• She presented with a breast swelling and tenderness
without a history of trauma or constitutional symptoms.
12
Breast USG demonstrated masses in her both breasts
and diffuse edeama.
13
PET-CT demonstrated a moderately hypermetabolic
soft tissue lesions more pronounced on left side
14
The biopsy from breast
indicated the presence
of small round cell
tumor with an
immunohistochemistry
profile similar to that of
CALLA (+)
Bone marrow aspiration and CSF were free of leukemic cells.
Chimerism evaluation of bone marrow sample showed full donor
chimerism.
15
Isolated extramedullary breast relapse
Systemic chemotherapy?
Radiotherapy?
Metil prednisolone
Cytarabine 100 mg/m2/day
6.6.2016- 15.6.2016
10 day
16
August 2016 (3 months later)
• Normal hematological evaluation
Hb: 12.2 gr/dl WBC: 9800/mm3 Plt: 330.000/mm3
• Full donor chimerism
• PET-CT: Regression of measurable disease
17
Radiotherapy
She underwent intensity-modulated radiotherapy
Left breast 45 Gy/180 cGy/25 fraction
(17.10.2016-16.11.2016)
Right breast + lymph nodes 46 Gy/200 Gy/ 23 fraction
(16.12.2016-18.01.2017)
18
November 2016
• Bone marrow biopsy: Remission
• Chimerism: Full donor chimerism
• CNS evaluation: Free of leukemic cells
19
March 2017
Headache and blurred vision
CSF evaluation
Bone marrow aspirate: 40%
Chimerism: 48% of donor cells
20
7-11 March 2017 RICE treatment
Intrathecal triple treatment
21
4.4.2017
• Bone marrow: Remission
• CSF: Free of cells
• Chimerism: full donor chimerism
22
23
What should be the next treatment?
Chemotherapy?
Chemotherapy+DLI?
Chemotherapy+Transplantation ?
Same donor?
Different donor?
24
Because
• Isolated extramedulary relapse of the breast in ALL
after allgeneic HSCT is extremely rare.
• The optimal treatment remains uncertain owing to
rarity
25