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Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 441 - 441
1 Jul 2010
Ash S Kachko L Katz J Mukamel M Weigel D Kornreich L Feinmesser M Yaniv I
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Hemangioendothelioma is a rare vascular tumor that is infrequently recognized in bone. It can be multicentric and often painful with an indolent course. The treatments of choice include curettage, resection, radiation, systemic medications or a combination of these modalities.

O.G. 5 years old girl, presented with left ankle pain and limping, without response to non steroidal anti-inflammatory drugs for few months. Radiological investigation (MRI) showed a lytic vascular lesion in the methadiaphysis, invading the epiphysis of the distal left tibia and lateral cartilage of the ankle, with atrophy of the left lower limb. Bone scan showed high uptake in this area. Histology showed fragments of bone, infiltrated by a vascular lesion with nodular pattern, well differentiated vascular spaces and endothelial cells with few mitotic figures. Immunostains were positive for CD31 and F8. The pathology report confirmed hemangioendothelioma.

As the lesion invaded the growth plate of the distal tibia, surgical or radiation therapy at this age could cause a permanent damage. We therefore successfully treated the child with Interferon α–2β 0.5 million IU three times a week for 18 months. She was pain free after the first few months of therapy with full recovery of daily function and activity. Radiological evaluation showed improvement on X-ray and MRI, and shrinkage of the lesion to the epiphysis area only.

Unfortunately, 3 years later the pain and limping reappeared. MRI showed a lytic lesion in the diamethaphysis of the left tibia. Re-biopsy supported the diagnosis of recurrent hemangioendothelioma. She was retreated with Interferon α–2β using the same protocol with considerable improvement of the pain and limping.

We present here a non invasive option for therapy with Interferon α–2β for bony lesion of hemangioendothelioma that enable us to spare the growth plate in a growing prepubertal child.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 456 - 456
1 Jul 2010
Goshen Y Kornreich L Stein J Ash S Cohen I Feinmesser M Yaniv I
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The detection of hepatic nodules during follow-up of survivors of solid tumors in childhood raises a diagnostic dilemma. Focal nodular hyperplasia (FNH) is an uncommon, benign tumor and must be differentiated from late hepatic metastasis.

We retrospectively analyzed patients, treated for pediatric solid tumors between January 1990 and December 2007, and performed abdominal imaging as part of the follow-up.

Four survivors with FNH were detected, out of 450 who received chemotherapy with/out irradiation including patients who underwent autologous bone marrow transplantation (ABMT). Case 1: A 23 years(y) adolescent, presented at age 10y with acute abdomen due to embryonal sarcoma of liver, she received VACAIEx4, relapsed locally, and underwent ABMT with high-dose carboplatin/melphalan and radiotherapy. Asymptomatic multiple liver lesions were disclosed by US and MRI 5y later, biopsy proved FNH. Case 2: A 21y adolescent who at age 3y had alveolar rhabdomyosarcoma of the calf with positive inguinal nodes. She received VACAIE x6, and VP16/carboplatin x3 with local radiation. She developed ovary disorder and received oral contraceptive (OC) at age 14.5y, routine US 1.5y later disclosed nodular lesions in liver, diagnosed as FNH by CT, pills were stopped. At follow-up some lesions reduced in size and few disappeared. Case 3: A 9y old girl, operated for choroid plexus carcinoma at age 1.5y, received VP16/carboplatin x16 and underwent ABMT preceded by thiotepa/melphalan. Abdominal US at age 5.5y disclosed multiple liver lesions, biopsy proved FNH, that disappeared 2y later. Case 4: An 11y old girl operated at age 8 months for retroperitoneal germ cell tumor, received VIP/BVPx4, routine US at 10y disclosed 2 liver lesions diagnosed by CT as FNH.

We conclude that FNH can be differentiated from late metastasis by imaging; in questionable cases by biopsy, close follow-up is recommended, alkylating agents especially during ABMT, and OC may be risk factors.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 458 - 458
1 Jul 2010
Ash S Cohen I Goshen Y Toledano H Yaniv I
Full Access

Increased intensity of therapy for osteosarcoma in the last 30 years has improved prognosis. 70–80% of patients with non metastatic osteosarcoma can now be cured, but late side effects occur. Fertility of survivors is becoming of greater importance.

We retrospectively studied all consecutive female long term survivors of localized osteogenic sarcoma of childhood and adolescence treated at the Schneider Children‘s Medical Center of Israel. Patients were treated with 3 different protocols including the use of Methotrexate, Adriamycin, Cisplatin, Bleomycin, Cytoxan, Vincristine, Actinomycin D, Melphalan and Ifosfamide.

Sixteen female survivors of non metastatic osteogenic sarcoma were treated from 1/1977 to 12/2001, with a minimum follow up of 6.3 years (max. 29 years) from the end of therapy. Median age at diagnosis was 11.7 (range 9.0–16.8) years. Twelve out of 16 (75%) are married and have between them 31 children, mean 2.7 (range 1–7) children. Of these 11 have children and one is currently pregnant with her first child. None of the females reported difficulties in conceiving their first child. The maximum interval from marriage to first delivery was 2.5 years. Two females had 3 spontaneous abortions between the 2nd–4th pregnancies. Four out of 9 female survivors who received > 360mg/m2 of adriamycin were treated with cardiomimetic drugs and/or ACE inhibitors during pregnancy. All four had 2–4 children/ female survivor. The children of survivors are healthy with no birth defects. Mean length of pregnancy was 38.6 weeks and mean birth weight was 2865 grams. No survivors had undergone invasive fertility preservation procedure and only one unmarried patient was using GnRH analogs.

Despite reports of transient disturbances in menstruation, all married females were fertile. Our results question the need for fertility preservation using GnRH analogs or invasive procedures such as ovary or egg preservation for non metastatic osteogenic sarcoma female patients.