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. 2017 Jan 16;16(3):433–440. doi: 10.1007/s10689-017-9965-1

Table 3.

Cancer diagnoses in participants

Pt Sex Age Mutation Abnormality (score) seen on WB MRI Further investigations Cancer Treatment
1 F 33 c.455C > T
p.Pro152Leu
Right temporal lobe cyst (4) Dedicated brain MRI with contrast Astrocytoma Complete resection
2 F 51 c.659A > G
p.Tyr220Cys
Left lateral abdominal wall mass—probable sarcoma (4) US guided biopsy Myxosarcoma Complete resection
3 F 45 c.586C > T
p.Arg196Ter
Suspicious right renal mass (4) Abdominal CT, nephrectomy Chromophobe renal cell carcinoma Complete resection
Uterine fibroids (2) Pelvic MRI, TAH Leiomyosarcoma
4 F 24 c.844C > T
p.Arg282Trp
Liver lesion, possible focal nodular hyperplasia or hepatic adenoma (3) 1. Dedicated renal and liver MRI with contrast. Suspected sarcomas, nephrectomy and partial hepatectomy 1. Renal EAML
Liver EAML
1. Complete resection of both tumours
Right kidney lesion, possible complex renal cyst or solid lesion (3) 2. Follow-up pelvic MRIs for EAMLs detected progressive changes in sacro-iliac joint 2. Sacro-iliac osteosarcoma 2. MAP chemotherapy completed; surgery advised but patient pursuing proton beam therapy in USA
5 F 48 c.916C > T
p.Arg306Ter
Pericardial cyst (1) Nil
Non study MRI and PET revealed a 12.6 cm hilar mass with small left pleural effusion
Mediastinal liposarcoma grade 3 Resection with microscopic positive margins (0/8 lymph nodes involved) and chemotherapy
6 M 27 c.818G > A
p.Arg273His
Nil N/A Diagnosed with B ALL (not seen on WB MRI) Chemotherapy

EAML Epithelioid angiomyolipoma, MAP methotrexate, doxorubicin and cisplatin, TAH total abdominal hysterectomy