Ga-67 Scintigraphy in a Child With Adrenocortical Carcinoma
ROBERT HOWMAN-GILES, M.B.B.S., F.R.A.C.P., D.D.U.,* LUCIANNO DALLA POZZA, M.B.B.S., F.R.A.C.P.,t AND ROGER UREN, M.B.B.S., F.R.A.C.P., D.D.U .*
Adrenocortical carcinoma is a rare malignancy in child- hood and often presents with a large abdominal mass and precocious puberty. The tumor is often advanced at the time of diagnosis. Radiological techniques are the main imaging modalities for assessment of the primary tumor. Ga-67 scintigraphy has been used in other pedi- atric solid tumors, however, no data were available for this tumor in childhood. A case of a 2.5-year-old male with a Ga-67 avid adrenocortical carcinoma is reported in which Ga-67 scintigraphy was very useful in detecting recurrence of the tumor and in assessing response to therapy.
A DRENOCORTICAL CARCINOMA is a rare malignancy in childhood (1). Ga-67 scintigraphy has been used extensively in the management of many solid tumors in childhood (2-4). There has, however, been little published data on the use of Ga-67 in adrenal carci- noma in adults (5-7), and no reports were found in the pediatric age group. The application of Ga-67 scintig- raphy in a 2-year-old child with adrenocortical carci- noma is described.
Case Report
A 2.5-year-old boy presented with a 3-week history of lethargy, fever, and episodes of pallor and cyanosis in January of 1986. Physical examination revealed a large, right-sided abdominal mass together with signs of precocious puberty. Serum cortisol, testosterone, and dehydroepiandrosterone sulfate levels all were markedly elevated. Abdominal ultrasonography con- firmed the presence of a multilobulated mass in the right upper quadrant of the abdomen, lying between
Reprint requests: Dr. R. Howman-Giles, Head, Department of Nuclear Medicine, The Childrens Hospital, Bridge Road, Camper- down, Sydney, New South Wales, Australia 2050.
From the Departments of Nuclear Medicine* and Oncology,t The Children’s Hospital, Sydney, Australia
the right lobe of the liver and the right kidney. Echo- cardiography revealed that the mass extended through the inferior vena cava into the right atrium and pro-
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truded through the tricuspid valve into the right ven- tricle. CT scans of the abdomen confirmed these find- ings. There was no evidence of pulmonary metastases. A radionuclide bone scan showed uptake of tracer into the mass but no evidence of skeletal metastases. A Ga-67 scan showed marked uptake of tracer into the mass with extension of tracer into the inferior vena cava, right atrium, and ventricle (Fig. 1). The patient underwent open heart surgery, at which time the caval and cardiac portions of the tumor were removed. A diagnosis of adrenocortical carcinoma was confirmed
histopathologically. Six days later, he had a laparot- omy with gross resection of the abdominal mass. After surgery, the patient was commenced on mitotane (o’p’DDD). Therapy was stopped in October of 1987, at which time it was considered he was in complete clinical remission.
The patient remained well with no evidence of dis- ease until July of 1988, at which time a routine Ga-67 scan revealed a focus of activity in the upper abdomen suggesting local relapse (Fig. 2). CT scans confirmed a mass and a second laparotomy was undertaken and the
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tumor recurrence was completely resected macro- scopically. After surgery, he was commenced on ad- juvant chemotherapy with cisplatin, doxorubicin, 5-flu- orouracil, vincristine, and cyclophosphamide. He remained well until June of 1989, when a routine fol- low-up Ga-67 scan again revealed recurrence in the pre- vertebral area of the upper abdomen. A third laparotomy was performed that confirmed recurrence; however, the tumor could be only partially resected. Despite intensive chemotherapy with different agents (ifosfamide, etopo- side, carboplatin), the tumor continued to progress, as seen on Ga-67 studies (Fig. 3). The patient died in No- vember of 1989 (46 months after original presentation).
Discussion
Adrenocortical carcinoma is a rare tumor in child- hood, and often presents with a large abdominal mass and signs of precocious puberty (1). The tumor often is advanced at time of diagnosis. Ultrasonography, CT, and MRI studies are the main imaging investigations in assessing the local extent of the tumor and invasion
into the inferior vena cava. Radionuclide bone and Ga-67 studies were performed at initial staging to de- tect metastatic disease and as a baseline for long-term follow-up to detect recurrence of tumor and metasta- ses. Ga-67 scintigraphy has been used effectively in pediatric solid tumors (2-4). There are few data on Ga-67 scintigraphy in adrenocortical carcinoma (5-7), and no reports were found of childhood disease. The radionuclides initially used in these tumors were iodo- 131 cholesterol analogues. Seabold et al (8) described five cases studied with I-131-6-B-iodomethyl-19-nor- cholesterol; three of these also had Ga-67 studies. The latter group showed positive uptake in metastases in the skeleton, liver, and lung. The authors made the comment that “both nuclides appeared to show similar patterns of uptake in metastatic adrenal cortical carcinoma.” Ward et al (6) and Parathasarathy et al (7) reported Ga uptake in metastases in the lung and soft tissues of the thigh in adrenocortical carcinoma. Jackson et al (5) reported up- take in a benign adrenal adenoma. The tumor in our patient showed marked Ga avidity. In follow-up of the child, Ga-67 was an effective tumor marker; it detected recurrence of disease before other imaging modalities and helped confirm that the tumor was not responding to intensive chemotherapy.
The SPECT studies were extremely useful because the planar images at the time of the initial recurrence were marginally abnormal; the SPECT study clearly detailed the site and extent of the recurrence. The management decisions as to the length of time to con- tinue with intensive chemotherapy after the third lap- arotomy were assisted by the Ga-67 studies, which showed increasing spread and avidity of the tumor despite the chemotherapy.
References
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