Unusual ovarian leukemic relapse in a girl with history of B cell
lymphoblastic leukemia
ACH Fung, KKY Wong
To the editor:
Extramedullary leukemic recurrence mostly occurs in the central nervous
system and occasionally in the skin. [1] Here, we report an unusual
case of a girl with relapsed lymphoblastic leukemia involving the ovary,
presenting as a large pelvic mass.
A 10-year-old girl with history of B cell lymphoblastic leukaemia
treatment and in remission 1 year ago presented to oncology clinic with
an enlarging pelvic mass for 2 months. Magnetic resonance imaging
revealed a large lobulated solid heterogeneous pelvic mass (measuring
12cm in greatest dimension) with mass effect on pelvic organs (Figure
1a). Bone marrow aspirate at conventional site confirmed absence of
lymphoblastic cells, which would have suggested leukaemia relapse. In
view of the suspicion of a second primary tumour in the ovary with
complication, exploratory laparotomy and left salpingo-oophorectomy were
performed (Figure 1b). Histology showed diffuse infiltration of the
ovary by B cell lymphoblastic leukaemia. Hematoxylin and eosin staining
showed sheets of lymphoid cells with irregular nuclei. The tumour cells
are positive for TdT, CD19, CD79a and CD34. (Figure 1c) She was well
after operation. In view of extramedullary relapse, bone marrow aspirate
was repeated after operation at anterior iliac spine which confirmed
bone marrow relapse. She received treatment according to high risk
protocol of CCCG Relapsed Acute Lymphoblastic Leukemia 2017 Study in
Children [2].
Acute lymphoblastic leukaemia (ALL) is responsible for one-third of
childhood malignancy. Despite efforts in the treatment of ALL, 15-20%
of patients developed relapse with highest risk immediately after
cessation of treatment and diminishes with time. [3] Extramedullary
involvement constitutes 2-5% of all relapses with the majority in the
central nervous system. It seldom involves other sites, such as the eye,
kidney, and omentum. Ovarian leukemic relapse is rarely reported in
children with scarce case reports in the literature [4, 5] It is
insidious and not usually detected until symptoms arise. Patient most
commonly presents with lower abdominal pain or a palpable large
abdominal mass. [6] Timely detection is important as it often
coincides with marrow recurrence. Routine surveillance pelvic sonography
is recommended for timely detection of pelvic extramedullary
relapse.[7] In patients with background of leukaemia presenting with
an ovarian mass, a higher level of suspicion needs to be kept. In the
absence of evidence in bone marrow relapse at conventional site, repeat
marrow aspirate at another site would be warranted since this affect the
choice of management. Previous reports shown that mainly chemotherapy
had a beneficial effect, while neither local radiation nor extensive
surgical resection of the leukaemic mass had any obvious effect on
overall outcome [8]. Operation could have been avoided if marrow
relapse is confirmed at the time of ovarian mass detection. However,
surgical excision was reported to have role in situation when bone
marrow had good response but no signs of regression of ovarian mass.
[9]
Reference
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