Outcome of Radiation-Related Osteosarcoma After Treatment of Childhood and Adolescent Cancer: A Study of 23 Cases

PURPOSE: We analyzed the clinical features and outcome of patients with radiation-associated osteosarcoma treated during the era of contemporary chemotherapy. PATIENTS AND METHODS: The characteristics and outcome of 23 patients (17 males and six females) treated during childhood or adolescence for a solid tumor who later developed osteosarcomas within the radiation field between 1981 and 1996 were reviewed. RESULTS: The median dose of radiation delivered to the first cancer was 47 Gy. Nineteen patients also received chemotherapy. The median time between radiotherapy and the diagnosis of secondary osteosarcoma was 8 years. Histologic slide review showed conventional central osteosarcoma with various differentiation patterns in 21 cases, together with one case of high-grade surface osteosarcoma and one of periosteal osteosarcoma. The sites of involvement were the craniofacial bones in six cases, the first cervical vertebra in one, the girdle bones in seven, and the extremities of long bones in nine. Three patients had metastatic disease at the diagnosis of osteosarcoma. Palliative therapy was administered to seven patients. The aim of treatment was curative for 16 patients, two of whom underwent amputation without further therapy. Intensive chemotherapy regimens were administered to 14 patients before and/or after surgery. Fifteen patients achieved complete surgical remission. Twelve patients were alive and disease-free at a median follow-up duration of 7.5 years. Overall and event-free survivals at 8 years were 50% and 41%, respectively. CONCLUSION: Patients with radiation-related osteosarcoma and resectable lesions can be cured with surgery and intensive preoperative and postoperative chemotherapy.

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Validation of a clinicopathological score for the prediction of post-surgical evolution of pituitary adenoma: retrospective analysis on 566 patients from a tertiary care centre

Acta Endocrinologica ◽

10.1530/eje-18-0749 ◽

2019 ◽

Vol 180 (2) ◽

pp. 127-134 ◽

Cited By ~ 19

Author(s):

S Asioli ◽

A Righi ◽

M Iommi ◽

C Baldovini ◽

F Ambrosi ◽

...

Keyword(s):

Pituitary Adenomas ◽

Proliferative Activity ◽

Disease Free Survival ◽

Low Grade ◽

High Grade ◽

Tumour Type ◽

Disease Evolution ◽

Free Survival ◽

Disease Free

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.

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Dedifferentiated Liposarcoma of the Transverse Colon

The American Surgeon ◽

10.1177/00031348211054524 ◽

2021 ◽

pp. 000313482110545

Author(s):

Katie Fitzgerald ◽

Eliza M. Slama ◽

Irina Bernescu

Keyword(s):

Transverse Colon ◽

Soft Tissue Sarcomas ◽

Surgical Excision ◽

Dedifferentiated Liposarcoma ◽

High Grade ◽

Nodal Involvement ◽

Segmental Colectomy ◽

Aggressive Tumor ◽

Disease Free

While liposarcoma is one of the most common soft tissue sarcomas, it is rarely seen within the gastrointestinal tract, and even less frequently seen within the colon. Dedifferentiated liposarcoma is a subtype of liposarcoma, which along with the pleomorphic subtype is considered a high-grade, aggressive tumor; both possess the ability to metastasize and are associated with decreased survival. Despite complete resection, recurrence is common. While surgical excision is the cornerstone of treatment for liposarcoma of the colon, there is no consensus on adjuvant therapies. We present the case of a 66-year-old woman who presented with abdominal pain with rectal bleeding and was found on colonoscopy to have a high-grade dedifferentiated liposarcoma of the transverse colon. She underwent robotic segmental colectomy. Due to absence of nodal involvement or distal metastasis, adjuvant therapy was not administered. On 1-year follow-up, the patient remains disease free.

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Radiotherapy as initial treatment for bulky stage II testicular seminomas.

Journal of Clinical Oncology ◽

10.1200/jco.1985.3.10.1333 ◽

1985 ◽

Vol 3 (10) ◽

pp. 1333-1338 ◽

Cited By ~ 32

Author(s):

S R Smalley ◽

R G Evans ◽

R L Richardson ◽

G M Farrow ◽

J D Earle

Keyword(s):

Initial Treatment ◽

Stage Ii ◽

Uicc Stage ◽

Median Dose ◽

Bulky Disease ◽

Median Size ◽

Initial Recurrence ◽

Primary Radiotherapy ◽

Disease Free

Sixteen consecutive patients with bulky stage II seminoma were treated with primary radiotherapy from 1971 to 1982. Bulky stage II seminoma was defined as either Union Internationale Contre le Cancer (UICC) stage IIC (retroperitoneal metastases greater than 5 cm) or IID (palpable retroperitoneal metastases) with no evidence of visceral or supradiaphragmatic disease. The median age was 38 years (range, 26 to 52) and the median size of retroperitoneal disease was 11.5 cm (range, 5 to 25 cm). Patients were treated with generous radiation ports (such as wide hockey-stick or whole abdomen) often followed by boosts to the sites of bulky disease. Median tumor dose was 3,235 cGy (range, 2,700 to 5,668 cGy). Mediastinal (with or without supraclavicular) prophylactic radiation was administered to 15 of the 16 patients with a median dose of 2,590 cGy (range, 1,200 to 3,700 cGy). Treatment toxicity was mild. All 16 patients achieved a complete remission (CR) with radiotherapy. Median follow-up from the time of diagnosis was 60 months, and all patients are currently disease-free. Two patients recurred after therapy but were rendered disease-free with further radiation. These two relapsing patients have remained disease-free, following initial recurrence, for 8 years. The excellent results obtained with modern imaging and radiotherapeutic techniques justify radiotherapy as the initial treatment of choice for bulky stage II seminomas.

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Prognosis of hepatic failure with the albumin-bilirubin model for hepatocellular carcinoma after stereotactic body radiotherapy with and without transplant.

Journal of Clinical Oncology ◽

10.1200/jco.2018.36.4_suppl.384 ◽

2018 ◽

Vol 36 (4_suppl) ◽

pp. 384-384

Author(s):

Shaakir Hasan ◽

Alexander V. Kirichenko ◽

Paul Renz ◽

Vijay Kudithipudi ◽

Molly Vincent ◽

...

Keyword(s):

Hepatocellular Carcinoma ◽

Overall Survival ◽

Hepatic Failure ◽

Stereotactic Body Radiotherapy ◽

Disease Free Survival ◽

Median Dose ◽

Free Survival ◽

Prognostic Assessment ◽

Disease Free

384 Background: The Albumin-Bilirubin (ALBI) model is a validated prognostic assessment of cirrhosis in hepatocellular carcinoma (HCC), stratifying patients to grades 1(ALBI-1), 2(ALBI-2), or 3(ALBI-3). We reported that ALBI distinguishes patients at higher risk for hepatic failure(HF) after stereotactic body radiotherapy (SBRT) within the Child Pugh(CP) A population. We now apply the ALBI model to both CP-A and CP-B patients after SBRT with or without orthotropic liver transplant (OLT), and assess its prognostic capability of overall survival (OS) and HF relative to the CP model. Methods: From 2009-2017, 68 patients with 81 HCC lesions and CP-A (45) or CP-B (23) cirrhosis completed SBRT in this IRB approved study. The median dose was 45 Gy (35 - 57 Gy) in 4-7 fractions. Initial ALBI and CP scores were measured against OS and progression of CP class, which was recorded every 3-4 months. Median follow-up = 18 months. Results: The median age = 62 and tumor size = 3.5 cm (1.1 Ð 11 cm). 26 patients were ALBI-1, 31 ALBI-2, and 11 ALBI-3 prior to SBRT. For all patients, 2-year local control was 96%. 1 and 2 year OS was 77% and 54%, disease free survival was 71% and 40%, and freedom from CP progression was 71% and 56%, respectively. OS was significantly different between ALBI-1, ALBI-2, and ALBI-3 patients (P = 0.01), as was progression of CP class (P<0.001). When stratified by initial CP class, there were no significant differences in survival or CP progression [Table 1]. In a subset of 37 CP-A and 15 CP-B without OLT, rates of progressive cirrhosis were better predicted by ALBI (P<0.001) than CP class (P=0.09). Conclusions: Compared to the CP model, the ALBI index more precisely predicted HF and OS in HCC patients for both early and intermediate cirrhosis. Its application may help better select candidates for OLT after SBRT, who may be at higher risk for HF than initially predicted. [Table: see text]

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Diverticulectomy in the Management of Intradiverticular Bladder Tumors: A Twelve-Year Experience at a Single Institution

Advances in Urology ◽

10.1155/2016/2345306 ◽

2016 ◽

Vol 2016 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Ali Bourgi ◽

Elias Ayoub ◽

Sleiman Merhej

Keyword(s):

Radical Cystectomy ◽

Tumor Resection ◽

Complete Removal ◽

Nodal Metastasis ◽

High Grade ◽

Case Review ◽

Bladder Tumors ◽

Retrospective Case ◽

Disease Free

Purpose. In this retrospective case review we analyze the outcomes of patients treated for intradiverticular bladder tumors (IDT).Materials and Methods. A retrospective case review was done between January 2002 and May 2014 in Hotel-Dieu de France hospital. The series included 17 patients diagnosed with IDT, all males with a mean age of 49.8 years.Results. One patient was treated with tumor resection and adjuvant BCG instillation with no recurrence on follow-up cystoscopies and urine cytologies. 64% of patients were treated by diverticulectomy. Mean follow-up time was 38.7 months. At the end of the follow-up, 81% were disease-free. One patient had a radical cystectomy 6 months after diverticulectomy for recurrent high grade tumor; another one had a nodal metastasis 10 months after diverticulectomy and was managed with chemotherapy. 29% of patients were treated with radical cystectomy. Mean follow-up time was 28.4 months. No recurrence was documented on annual CT scans.Conclusions. Our data support a conservative approach for tumors confined to the bladder diverticulum, even in high grade or in the presence of CIS provided complete removal is feasible and close follow-up is ensured.

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Autologous bone marrow transplantation for high-grade lymphoid malignancy using melphalan/irradiation conditioning without marrow purging or cryopreservation. The Northern Regional Bone Marrow Transplant Group

Blood ◽

10.1182/blood.v77.7.1593.bloodjournal7771593 ◽

1991 ◽

Vol 77 (7) ◽

pp. 1593-1598

Author(s):

PJ Carey ◽

SJ Proctor ◽

P Taylor ◽

PJ Hamilton

Keyword(s):

Bone Marrow ◽

Disease Free Survival ◽

Autologous Bone Marrow ◽

Marrow Transplant ◽

High Grade ◽

Lymphoid Malignancy ◽

Free Survival ◽

First Remission ◽

Disease Free

We report the safety and efficacy of 34 consecutive autologous bone marrow transplant (ABMT) procedures performed in adult patients with high-grade lymphoid malignancy after remission induction therapy. Fifteen patients with acute lymphoblastic leukemia (ALL) and six with high-grade non-Hodgkin's lymphoma (NHL) received pretransplant conditioning with intravenous (IV) melphalan and fractionated total body irradiation (TBI). Thirteen other patients with NHL were conditioned with melphalan alone, having previously received local involved field radiotherapy. Unmanipulated noncryopreserved autologous marrow was reinfused within 48 hours of harvesting. Engraftment occurred in all patients with medians of 10 days of neutropenia (neutrophils less than 0.5 x 10(9)/L), 4-day platelet transfusion requirement, 3 U packed RBC transfusion, and 18 days in hospital posttransplant. There were no procedure-related deaths. Actuarial disease-free survival in the 13 patients with ALL receiving autotransplant early in first remission is 48% with a median follow-up of 3 years. Two other ALL patients who had autotransplants after a period of maintenance therapy also remain in complete remission (CR). These results compare favorably with our 34% disease-free survival (DFS) in 15 allogeneic ALL transplant patients and 21% DFS in 19 patients on standard maintenance after a common induction schedule. No relapses have occurred in the 17 NHL patients transplanted in remission (median follow-up 2 years), but the two NHL patients who developed recurrent disease before ABMT died of progressive disease after temporary responses. We conclude that this method of ABMT results in rapid reengraftment with lack of toxicity and that the conditioning treatment used shows good efficacy against disease. It is applicable in high-grade lymphoid malignancy in first remission, and our results call into question the need for marrow purging in ALL and NHL patients transplanted in first remission.

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Adjuvant Postoperative Radiotherapy in Locally Advanced Rectal and Rectosigmoidal Cancer

Tumori Journal ◽

10.1177/030089168006600506 ◽

1980 ◽

Vol 66 (5) ◽

pp. 595-600 ◽

Cited By ~ 15

Author(s):

Roberto Zucali ◽

Gianstefano Cardani ◽

Fabio Volterrani

Keyword(s):

Clinical Evidence ◽

Postoperative Radiotherapy ◽

Locally Advanced ◽

Distant Metastases ◽

Pelvic Recurrence ◽

Advanced Stage ◽

Median Dose ◽

Disease Free

A pelvic recurrence is the cause of death in about 1/3 of radically operated patients for rectal and rectosigmoidal cancer without clinical evidence of distant metastases. Preoperative and postoperative radiotherapy are largely used to reduce the incidence of locoregional relapses and to improve disease-free and overall survival and quality of life. Benefits from radiotherapy have been widely demonstrated, and adjuvant postoperative radiotherapy is at present strongly recommended. Twenty-one patients with locally advanced (stage B2, B3, C) rectal (11 cases) and rectosigmoidal cancer (10 cases) were treated with postoperative radiotherapy at the National Cancer Institute of Milan from 1975 to 1978. The pelvis received a median dose of 4500 rad (range, 4000–5200 rad) in 5 to 7 weeks through AP, PA opposed fields; 6 patients received a boost of 1000 rad on the perineum. Median follow-up after surgery is 83 months (range, 24–63 months). Only 1 case (« 5%) had a pelvic recurrence, at the perineum. The expected recurrence rate after surgery alone is 40 %, and our favorable results after postoperative radiotherapy are comparable with recent data from other institutions. Radiotherapy side effects were moderate and transient; no late damages to small bowel were observed.

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Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) of gastrointestinal (GI) tract: A single institution experience.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.4_suppl.633 ◽

2020 ◽

Vol 38 (4_suppl) ◽

pp. 633-633

Author(s):

Manik A. Amin ◽

Nikolaos Trikalinos

Keyword(s):

Surgical Resection ◽

Poor Prognosis ◽

Braf V600e ◽

Low Grade ◽

Sequencing Analysis ◽

High Grade ◽

Gi Tract ◽

Intermediate Grade ◽

Disease Free

633 Background: MiNENs are heterogeneous group of rare tumors and represent less than 1% of all GI malignancies. These are pathologically classifiable according to neuroendocrine component into low grade, intermediate grade and high grade MiNENs but and managed based upon non-neuroendocrine component rather than true NENs. They have poor prognosis and less is known about treatment options. Methods: We identified 34 cases of MiNENs from 1/1/2000 to 1/1/2018 from Siteman Cancer Registry Database at Siteman Cancer Center, Washington University, St. Louis. Treatment and follow up information was obtained from medical charts of patients. Results: Of 34 identified cases of MiNENs, our database showed equal distribution of MiNENs in male and female patients (N = 17 each). Site of origin included appendix (N = 14), colon (N = 10), esophageal (N = 2), stomach (N = 3), rectum (N = 3), small intestine (N = 1) and ampulla of Vater (N = 1) locations. Histology was high grade (N = 22), intermediate grade (N = 8), low grade (N = 3) and unknown (1). Twenty nine (85%) underwent surgical resection with curative intent and 13 (40%) patients were disease free at five years of follow up. Disease was localized in 15 patients (44%) and distant metastasis were reported in 13 patients (40%). Ten out of 15 patient with localized disease were disease free with combined modality therapy of surgery and chemotherapy. Most common chemotherapy regimen used in the metastatic setting was FOLFOX (11), 5FU/capecitabine (3), and carboplatin plus etoposide (5). Next generation sequencing analysis was available on limited patients which showed MMR proficient (3), MMR deficiency (1), BRAF V600E mutation (1) and KRAS wild type (1). Conclusions: MiNENs of GI tract are rare and aggressive tumors and represent a distinct entity. Surgical resection whenever possible offers curative option. Our database of GI MiNENs showed efficacy with commonly used GI chemotherapy regimens. Patients with low grade tumor histology had better survival as compared to moderately and poorly differentiated tumors. Genomic testing through NGS would be recommended for personalized treatment decisions given poor prognosis in these patients.

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Periosteal Osteosarcoma: A Review of 17 Cases with Mean Follow-up of 52 Months

Sarcoma ◽

10.1080/1357714021000066368 ◽

2002 ◽

Vol 6 (4) ◽

pp. 123-130 ◽

Cited By ~ 24

Author(s):

Matthew P. Revell ◽

Neeta Deshmukh ◽

Robert J. Grimer ◽

Simon R. Carter ◽

Roger M. Tillman

Keyword(s):

Adjuvant Therapy ◽

World Literature ◽

Surgical Excision ◽

High Grade ◽

Future Treatment ◽

The World ◽

Periosteal Osteosarcoma ◽

Low Incidence ◽

Consistent Approach

Purpose:Periosteal osteosarcomas are rare cartilage-rich bone tumours characterized by a juxtacortical eccentric position and are normally regarded oncologically as of intermediate to high grade.Their low incidence is mirrored by a small number of reported cases in the world literature. While there is general agreement that wide surgical excision is required, there is a paucity of evidence regarding adjuvant therapy. Previous reports have not indicated any consistent approach to this to allow appraisal.Patients and methods:We report 17 cases treated at our centre over 16 years. Our policy was to use chemotherapy when the tumour showed any features of high grade.Results:To date, no deaths have resulted from recurrence or metastasis of the tumour although there have been two deaths from other causes.Discussion:Comparison of survival with existing studies is made to draw conclusions regarding future treatment of this condition in terms of surgical and adjuvant approaches.

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Autologous bone marrow transplantation for high-grade lymphoid malignancy using melphalan/irradiation conditioning without marrow purging or cryopreservation. The Northern Regional Bone Marrow Transplant Group

Blood ◽

10.1182/blood.v77.7.1593.1593 ◽

1991 ◽

Vol 77 (7) ◽

pp. 1593-1598 ◽

Cited By ~ 48

Author(s):

PJ Carey ◽

SJ Proctor ◽

P Taylor ◽

PJ Hamilton

Keyword(s):

Bone Marrow ◽

Disease Free Survival ◽

Autologous Bone Marrow ◽

Marrow Transplant ◽

High Grade ◽

Lymphoid Malignancy ◽

Free Survival ◽

First Remission ◽

Disease Free

Abstract We report the safety and efficacy of 34 consecutive autologous bone marrow transplant (ABMT) procedures performed in adult patients with high-grade lymphoid malignancy after remission induction therapy. Fifteen patients with acute lymphoblastic leukemia (ALL) and six with high-grade non-Hodgkin's lymphoma (NHL) received pretransplant conditioning with intravenous (IV) melphalan and fractionated total body irradiation (TBI). Thirteen other patients with NHL were conditioned with melphalan alone, having previously received local involved field radiotherapy. Unmanipulated noncryopreserved autologous marrow was reinfused within 48 hours of harvesting. Engraftment occurred in all patients with medians of 10 days of neutropenia (neutrophils less than 0.5 x 10(9)/L), 4-day platelet transfusion requirement, 3 U packed RBC transfusion, and 18 days in hospital posttransplant. There were no procedure-related deaths. Actuarial disease-free survival in the 13 patients with ALL receiving autotransplant early in first remission is 48% with a median follow-up of 3 years. Two other ALL patients who had autotransplants after a period of maintenance therapy also remain in complete remission (CR). These results compare favorably with our 34% disease-free survival (DFS) in 15 allogeneic ALL transplant patients and 21% DFS in 19 patients on standard maintenance after a common induction schedule. No relapses have occurred in the 17 NHL patients transplanted in remission (median follow-up 2 years), but the two NHL patients who developed recurrent disease before ABMT died of progressive disease after temporary responses. We conclude that this method of ABMT results in rapid reengraftment with lack of toxicity and that the conditioning treatment used shows good efficacy against disease. It is applicable in high-grade lymphoid malignancy in first remission, and our results call into question the need for marrow purging in ALL and NHL patients transplanted in first remission.

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