Liposarcoma of the thyroid gland is rare with only 3 cases reported in the English literature. We present a further two patients
whom we have recently treated: a 49 year old lady with a myxoid liposarcoma and a 71 year old man with a pleomorphic
liposarcoma. Both underwent macroscopic excision of tumour but had positive margins, so were then treated with external
beam radiotherapy. The former patient died from metastases 10 months after presentation, the latter remains alive but has
developed metastatic disease on follow up at 24 months.We recommend the use of high dose radiotherapy following radical surgery as margins of excision are usually narrow in
this most difficult region. The role of chemotherapy is yet to be established.

A. Mitra

C. Fisher

P. Rhys-Evans

C. Harmer

Liposarcoma of the Thyroid

V. Kapoor

V. Sudheer

M. Waseem

A. S. Cole

D. F. Weeden

An Unusual Presentation of Chondrosarcoma of the Clavicle With Horner&#x27;s Syndrome

Background: To define the efficacy of postoperative irradiation in patients with recurrent extra-abdominal desmoid tumors in
whom surgical intervention has resulted in microscopically or grossly positive surgical margins.Methods: A retrospective analysis was performed on all patients referred to the department of radiation oncology at the
Detroit Medical Center with a diagnosis of recurrent extra-abdominal desmoid tumor. This analysis includes all patients
seen from 1 January 1990 through 31 December 1999. A total of 11 patients were treated to 13 sites. Ten had
microscopically positive margins and three had gross residual disease. Three patients were noted to have multifocal disease at
the time of initial representation. Local control, survival, follow-up, and subsequent development of new tumors are
measured from the last day of treatment with irradiation.Results: Thirteen sites were treated. Seven patients had received chemotherapy/hormonal therapy prior to surgery and/or
irradiation. The most commonly used drug was tamoxifen (n&#x3D;6). The type of radiation delivered included external beam
irradiation alone (n&#x3D;3), combined external beam irradiation and brachytherapy (n&#x3D;4), brachytherapy alone (n&#x3D;3) and
252-Cf neutron brachytherapy alone (n&#x3D;3). Follow-up has ranged from 29 to 115 months (median&#x3D;76 months). Three
patients have failed locally at 17, 24 and 29 months. One of these was treated for gross residual disease. No patient has died
of tumor-related causes. Salvage at the failed sites was possible in twom of three with re-irradiation using external neutrons
and/or aggressive surgical intervention and systemic therapy. Complications were most often noted to include decrease range
in motion, especially in joint areas, and skin reactions which were normal in presentation. In one site there was development
soft tissue necrosis.Conclusion: Based on our experience we recommend postoperative irradiation for all recurrent extra-abdominal desmoid
lesions with microscopically or grossly positive surgical margins. Furthermore, patients with recurrent desmoid tumors
involving the bony structures of the hand or feet are poor candidates for brachytherapy alone. For patients with extremity
lesions, brachytherapy may be a reasonable treatment option provided adequate margins around the tumor bed are covered.
The continued recommended use of irradiation in this group of patients is warranted.

James Fontanesi

Michael P. Mott

Michael J. Kraut

David P. Lucas

Peter R. Miller

The Role of Postoperative Irradiation in the Treatment of Locally Recurrent Incompletely Resected Extra-Abdominal Desmoid Tumors

Purpose: To determine the response and toxicity of docetaxel in recurrent osteosarcoma and related spindle cell tumours of bone.Patients and methods: Fourteen patients, 10 males and four females, were enrolled, median age 30.5 years (range, 17–46). Diagnosis was: conventional osteosarcoma, 12 patients; periosteal osteosarcoma, one patient; and malignant fibrous histiocytoma of bone, one patient. Initial chemotherapy had been with doxorubicin and cisplatin in 10 patients, and multiagent regimens in four. Nine had been treated with second line chemotherapy before receiving docetaxel. Thirteen patients had lung metastases and one intra-abdominal disease. Docetaxel 100 mg/m2 was given as a 1-h infusion every 3 weeks. Response was assessed every two cycles to a maximum of six.Results: A total of 43 cycles were given, median of two per patient (range 1–6). Thirteen patients were evaluable for response. A single partial remission was seen, for a response rate of 8%. Two patients had stable disease, and one patient a mixed response. Forty cycles were evaluable for toxicity. The principle toxicity was haematological, with a median neutrophil count of 0.9 (range 0–9.6). There were four episodes of neutropenic sepsis (10%). The only non-haematological toxicity ≥grade 3 was stomatitis, occurring in just one patient. There were no toxic deaths.Conclusion: Docetaxel at this dose and schedule is well tolerated, but is not associated with significant activity in patients with relapsed osteosarcoma.

Anne Mctiernan

Jeremy S. Whelan

A Phase II Study of Docetaxel for the Treatment of Recurrent Osteosarcoma

Purpose: Few reports have been published on the evaluation of stem cell auto transplantation for chemosensitive sarcomas.
Some suggest benefit, others do not. We present results of 24 patients with sarcoma undergoing autotransplantation at a
Canadian institution.Patients and Methods: Twenty-four patients were treated between 1988 and 1998: 23 were &#x2265;18 years (median 27; range 12&#x2013;56); genders were equal; 12 patients had Ewing&#x27;s sarcoma. At diagnosis, 12 (50&#x25;) had metastatic disease. Prior to
autotransplant, all had &#x2265;1 chemotherapy regimen. Fourteen (58&#x25;) were in complete remission (CR) and seven (29&#x25;) had minimal residual disease. All received etoposide 60 mg/kg (Day &#x2013;4), melphalan 140 mg/m<mml:math id="E1" xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:msup><mml:mrow><mml:mtext></mml:mtext></mml:mrow><mml:mn>2</mml:mn></mml:msup></mml:mrow></mml:math> on (Day &#x2013;3) and a stem cell
reinfusion (Day 0).Results: Three patients (12.5&#x25;) were alive and disease-free with median follow-up of 92 months (80&#x2013;142); one was alive
with disease 32 months post-autotransplant. Twenty had died (disease, 17; transplant-related, 2; unknown, 1). Of the four alive, three had Ewing&#x27;s sarcoma, one alveolar rhabdomyosarcoma, and all were in CR at transplant. Median time to relapse
was 6 months (2&#x2013;59). Sixteen of 18 (89&#x25;) relapsed within 1 year. Median overall survival was 10 months (0&#x2013;137). A trend towards improved survival (P&#x3D;0.07) was evident for patients in CR prior to autotransplant.Conclusions: Stem cell autotransplantation does not benefit most patients with sarcoma. A subgroup of high-risk patients in CR may fare better and warrant further study.

S&#xE9;bastien J. Hotte

Anne M. Smith

Vivien H.C. Bramwell

Kang Howson-Jan

High-Dose Chemotherapy Followed by Peripheral and/or Bone Marrow Stem Cell Transplant in Patients With Advanced Sarcoma: Experience of a Canadian Centre

Background: Patients who have had initial curative intent therapy for non-metastatic soft tissue sarcoma, and who
subsequently relapse at the initial site without evidence of metastatic disease, have various options regarding local treatment.
The treatment options available will be determined by the extent of relapse, previous therapy rendered, and patient
characteristics. We reported on a series of 31 patients treated initially with only surgery for extremity/trunkal high-grade soft
tissue sarcoma and then seen for recurrence at our institution between 1980 and 1999. Local re-treatment consisted
of combined modality therapy, most often aggressive surgical debulking/resection and irradiation, in an effort to reduce
the need for amputation and, where anatomically allowable, to maintain a functional limb. We report our results in
re-establishing local control, subsequent survival, and complication rates.Methods: Thirty-one patients with locally recurrent, non-metastatic high-grade soft tissue sarcoma, (excluding extraabdominal
desmoid) were retrospectively reviewed to determine local control, survival, and complication rates associated
with the relapsed disease. All patients had multimodality re-treatment most often utilizing aggressive surgical debulking and
irradiation. The irradiation consisted of either external beam alone, brachytherapy alone, or a combination of external beam
and brachytherapy. Nine patients also received multi-agent, multi-cycle chemotherapy using various regimens. In addition,
the impact of surgical margin at the time of re-resection (gross versus microscopic disease), radiation treatment type, total
radiation dose delivered, size of relapse, histological sub-type, sex and age, were evaluated to determine if they had any
impact on the re-establishment of local control and subsequent survival.Results: Local control was re-established in 25 of 31 (80.6%) patients. Two additional patients with isolated local relapse
after irradiation were salvaged with amputation and remain NED at last follow-up. With these patients a total of 27/31 (87%)
are now with local control. At last follow-up, which ranged from 23 to 192 months, 23 of 31 (74%) remained alive. Of the
eight patients who have died, four had evidence of local and distant failure. Two additional patients died of distant failure
while the treated sites remained in local control and two patients, both NED, died of intracurrent processes. Follow-up for
those patients who had re-established local control has ranged from 23 to 192 months (median=60.5 months). Time to
local failure following re-treatment ranged between 3 and 72 months following re-treatment (median=12 months). Five
patients had significant treatment related complications. Included are two patients in which amputation was required due to
local recurrences. Two patients developed a soft tissue necrosis and one patient had a wound healing problem that resolved
with conservative management. No statistical significance in the development of local control could be found based on
surgical margin status, total dose of irradiation (greater or less than 60 Gy), size of recurrence (greater than 5 cm),
histological sub-type, sex, or age (greater than 50 years). There was a trend for negative impact for those patients receiving
only external beam irradiation.Conclusion: Selective locally recurrent, non-metastatic soft tissue sarcoma of the extremity/trunkal regions should still be
considered eligible for aggressive limb-sparing therapy. Our experience suggests that a majority of patients re-establish local
control following aggressive surgical resection/debulking and irradiation and this appears to be durable in its nature. The role
of chemotherapy in this group of patients remains investigational. In a surprising finding, one patient re-relapsed in the
re-treatment site at 72 months, thus justifying continued strict surveillance not only in the primary site but also for
subsequent metastatic disease.

David R. Lucas

Peter  R. Miller

The Role of Irradiation in the Management of Locally Recurrent Non-Metastatic Soft Tissue
                   Sarcoma of Extremity/Trunkal Locations

We present a case of giant osteofibrous dysplasia (OFB) of the proximal tibia with 15 years of follow-up. The tumor recurred
after first biopsy and curettage done at the age of 6 years and, again, 4 years later. Following recurrence, the option of
amputation was suggested. Staged treatment of curettage, cryosurgery, bone cement as a temporary spacer with internal
fixation at age 12 years, followed by bone grafting at age 14 years, led to complete healing. The staged protocol for treatment
is proposed as an alternative to more radical solutions. It is suggested to postpone surgical treatment towards skeletal
maturity.

Eitan  Segev

Josephine  Issakov

Eli  Ezra

Shlomo  Wientroub

Itzchak  Meller

Giant Osteofibrous Dysplasia (Ossifying Fibroma) of the Tibia: Case Report and Review of Treatment Modalities

Objective. We have investigated whether there is evidence that patients with soft tissue sarcomas do better if treated in a
specialist centre compared with district general hospitals.Patients. All patients diagnosed with soft tissue sarcomas who were residents of WMRHA between 1994 and 1996, with
minimum follow up of 5 years, excluding head and neck or retroperitoneal tumors.Methods. We reviewed data from the Royal Orthopaedic Hospital Oncology Service (ROHOS) database and the Cancer
Intelligence Unit (CIU) Database, with medial record review where necessary. Main outcome measures were local
recurrence and overall survival.Results. A total of 260 patients were diagnosed as having STS over the 3-year period (incidence=1.62per 100000 per
year): 37% of patients had the majority of treatment at the specialist centre under the care of three surgeons, whilst the other
63% were treated at a total of 38 different hospitals. The rate of local recurrence was 39% at the district general hospitals
compared with 19% at the specialist centre despite the fact that tumours treated at the district hospitals were smaller and of
lower grade. The most significant factors affecting survival were grade (high versus low) and depth of the tumour. Patients
treated at the specialist centre had a small survival advantage after multivariate testing.Conclusion. Soft tissue sarcomas are rare. Centralization of treatment improves local control in all patients and survival in
some. Appropriate mechanisms for ensuring that patients with soft tissue sarcomas are seen and treated at specialist centres
should be developed.

A. A. Bhangu

J. A. S. Beard

R. J. Grimer

Should Soft Tissue Sarcomas be Treated at a Specialist Centre?

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Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.

Sarcoma