Decrease in treatment intensity predicts worse outcome in patients with locally advanced head and neck squamous cell carcinoma undergoing radiochemotherapy.

PURPOSE: Radiochemotherapy (RCT) is an effective standard therapy for locally advanced head and neck squamous cell carcinoma (LA-HNSCC). Nonetheless, toxicity is common, with patients often requiring dose modifications. METHODS: To investigate associations of RCT toxicities according to CTCAE version 5.0 and subsequent therapy modifications with short- and long-term treatment outcomes, we studied all 193 patients with HNSCC who received RCT (70 Gy + platinum agent) at an academic center between 03/2010 and 04/2018. RESULTS: During RCT, 77 (41%, 95% CI 34-49) patients developed at least one ≥ grade 3 toxicity, including seven grade 4 and 3 fatal grade 5 toxicities. The most frequent any-grade toxicities were xerostomia (n = 187), stomatitis (n = 181), dermatitis (n = 174), and leucopenia (n = 98). Eleven patients (6%) had their radiotherapy schedule modified (mean radiotherapy dose reduction = 12 Gy), and 120 patients (64%) had chemotherapy modifications (permanent discontinuation: n = 67, pause: n = 34, dose reduction: n = 7, change to other chemotherapy: n = 10). Objective response rates to RCT were 55% and 88% in patients with and without radiotherapy modifications (p = 0.003), and 84% and 88% in patients with and without chemotherapy modifications (p = 0.468), respectively. Five-year progression-free survival estimates were 20% and 50% in patients with and without radiotherapy modifications (p = < 0.001), and 53% and 40% in patients with and without chemotherapy modifications (p = 0.88), respectively. CONCLUSIONS: Reductions of radiotherapy dose were associated with impaired long-term outcomes, whereas reductions in chemotherapy intensity were not. This suggests that toxicities during RCT should be primarily managed by modifying chemotherapy rather than radiotherapy.

End-of-life decision making by Austrian physicians - a cross-sectional study.

BACKGROUND: Austria has recently been embroiled in the complex debate on the legalization of measures to end life prematurely. Empirical data on end-of-life decisions made by Austrian physicians barely exists. This study is the first in Austria aimed at finding out how physicians generally approach and make end-of-life therapy decisions. METHODS: The European end-of-life decisions (EURELD) questionnaire, translated and adapted by Schildmann et al., was used to conduct this cross-sectional postal survey. Questions on palliative care training, legal issues, and use of and satisfaction with palliative care were added. All Austrian specialists in hematology and oncology, a representative sample of doctors specialized in internal medicine, and a sample of general practitioners, were invited to participate in this anonymous postal survey. RESULTS: Five hundred forty-eight questionnaires (response rate: 10.4%) were evaluated. 88.3% of participants had treated a patient who had died in the previous 12 months. 23% of respondents had an additional qualification in palliative medicine. The cause of death in 53.1% of patients was cancer, and 44.8% died at home. In 86.3% of cases, pain relief and / or symptom relief had been intensified. Further treatment had been withheld by 60.0%, and an existing treatment discontinued by 49.1% of respondents. In 5 cases, the respondents had prescribed, provided or administered a drug which had resulted in death. 51.3% of physicians said they would never carry out physician-assisted suicide (PAS), while 30.3% could imagine doing so under certain conditions. 38.5% of respondents supported the current prohibition of PAS, 23.9% opposed it, and 33.2% were undecided. 52.4% of physicians felt the legal situation with respect to measures to end life prematurely was ambiguous. An additional qualification in palliative medicine had no influence on measures taken, or attitudes towards PAS. CONCLUSIONS: The majority of doctors perform symptom control in terminally ill patients. PAS is frequently requested but rarely carried out. Attending physicians felt the legal situation was ambiguous. Physicians should therefore receive training in current legislation relating to end-of-life choices and medical decisions. The data collected in this survey will help political decision-makers provide the necessary legal framework for end-of-life medical care.

Critical evaluation of platelet size as a prognostic biomarker in colorectal cancer across multiple treatment settings: a retrospective cohort study.

PURPOSE: The role of mean platelet volume (MPV) as a predictor of outcomes in various cancer entities including colorectal cancer (CRC) has already been analyzed. However, data on the prognostic and predictive value of MPV in CRC over multiple lines of systemic therapy are missing. METHODS: In this retrospective single-center cohort study, 690 patients with UICC stage II, III or IV CRC receiving adjuvant and/or palliative chemotherapy were included. Primary endpoints in the adjuvant, palliative and best supportive care (BSC) setting were 3-year recurrence-free survival (RFS), 6-months progression-free survival (PFS), and 6-months overall survival (OS), respectively. Kaplan-Meier estimators, log-rank tests, and uni- and multivariable Cox models were used to analyze RFS, PFS and OS. A cut-off defining patients with low MPV was chosen empirically at the 25th percentile of the MPV distribution in the respective treatment setting. RESULTS: Three-year RFS was 76%. Median 6-month PFS estimates in 1st, 2nd and 3rd line therapy were 59, 37 and 27%, respectively. Median 6-month OS in BSC was 31%. Small platelets as indicated by low MPV did not predict for shorter RFS. In the first 3 palliative treatment lines a consistent association between low MPV and decreased 6-month PFS was not observed. In the BSC setting, patients with low MPV had numerically but not significantly shorter OS. Higher MPV levels did not consistently predict for ORR or DCR across the first 3 palliative treatment lines. CONCLUSION: Small platelets are not predicting CRC outcomes, and thus are hardly useful for influencing clinical decision making.

Long-term cardiovascular complications in stage I seminoma patients.

PURPOSE: The cure rate of stage I seminoma patients is close to 100% and so the recent focus of clinical research has shifted onto the prevention of treatment-related complications. We assessed long-term cardiovascular complications and identified risk factors for cardiovascular events (CVEs) in stage I seminoma patients. METHODS: This retrospective cohort study included 406 consecutive stage I seminoma patients. Primary endpoint was CVE rate. RESULTS: During a median follow-up of 8.6 years, we observed 23 CVEs in 406 patients [10-year CVE risk 5.6% (95% CI 3.2 to 8.8)]. In univariable competing risk analysis, higher age, positive smoking status, history of diabetes and hypertension were significantly associated with the occurrence of CVE. In multi-state analysis, new onset of diabetes, hypertension and hyperlipidemia during follow-up predicted for an excessively increased CVE risk. In multivariable analysis adjusting for age and smoking, the development of hypertension and hyperlipidemia after tumor-specific treatment prevailed as risk factors for CVE. Regarding adjuvant treatment modalities, patients receiving adjuvant radiotherapy had a significantly higher probability of CVE than patients receiving adjuvant carboplatin [16% vs. 0%; risk difference (RD) = 16%, 95% CI 6 to 25%, p = 0.001]. This difference prevailed after adjusting for age, follow-up-time, diabetes, hypertension and smoking (RD = 11%, 95% CI 1 to 20%, p = 0.025). CONCLUSION: We identified a panel of baseline risk factors and dynamically, occurring predictors of CVE in stage I seminoma patients. This information may be used for targeting comorbidity management in these patients. The observed association of adjuvant radiotherapy with higher CVE risk warrants further investigation.

The predictive impact of body mass index on the efficacy of extended adjuvant endocrine treatment with anastrozole in postmenopausal patients with breast cancer: an analysis of the randomised ABCSG-6a trial.

BACKGROUND: We investigated whether body mass index (BMI) can be used as a predictive parameter indicating patients who benefit from extended aromatase inhibitor (AI) treatment. METHODS: The ABCSG-6a trial re-randomised event-free postmenopausal hormone receptor-positive patients from the ABCSG-6 trial to receive either 3 additional years of endocrine therapy using anastrozole vs nil. In this retrospective analysis, we investigated the prognostic and predictive impact of BMI on disease outcome and safety. RESULTS: In all, 634 patients (177 normal weight, 307 overweight, and 150 obese) patients were included in this analysis. Normal weight patients with additional 3 years of anastrozole halved their risk of disease recurrence (disease-free survival (DFS) HR 0.48; P=0.02) and death (HR 0.45; P=0.06) and had only a fifth of the risk of distant metastases (HR 0.22; P=0.05) compared with normal weight patients without any further treatment. In contrast, overweight+obese patients derived no benefit from additional 3 years of anastrozole (DFS HR 0.93; P=0.68; distant recurrence-free survival HR 0.91; P=0.78; and OS HR 0.9; P=0.68). The possible predictive impact of BMI on extended endocrine treatment could be strengthened by a Cox regression interaction model between BMI and treatment (P=0.07). CONCLUSION: Body mass index may be used to predict outcome benefit of extended AI treatment in patients with receptor-positive breast cancer.

Efficacy of tamoxifen ± aminoglutethimide in normal weight and overweight postmenopausal patients with hormone receptor-positive breast cancer: an analysis of 1509 patients of the ABCSG-06 trial.

BACKGROUND: There exists evidence that body mass index (BMI) impacts on the efficacy of aromatase inhibitors in patients with breast cancer. The relationship between BMI and the efficacy of tamoxifen is conflicting. We investigated the impact of BMI on the efficacy of single tamoxifen and tamoxifen plus an aromatase inhibitor in the well-defined prospective study population of the ABCSG-06 trial. METHODS: ABCSG-06 investigated the efficacy of tamoxifen vs tamoxifen plus aminoglutethimide in postmenopausal women with hormone receptor-positive breast cancer. Taking BMI at baseline, patients were classified as normal weight (BMI=18.5-24.9 kg m(-)(2)), overweight (BMI=25-29.9 kg m(-)(2)), and obese (30 kg m(-)(2)) according to WHO criteria. RESULTS: Overweight+obese patients had an increased risk for distant recurrences (hazard ratio (HR): 1.51; Cox P=0·018) and a worse overall survival (OS; HR: 1·49; Cox P=0·052) compared with normal weight patients. Analysing patients treated with single tamoxifen only, no difference between overweight+obese patients and normal weight patients regarding distant recurrence-free survival (HR: 1.35; Cox P=0·24) and OS (HR: 0.99; Cox P=0·97) could be observed. In contrast, in the group of patients treated with the combination of tamoxifen plus aminoglutethimide, overweight+obese patients had an increased risk for distant recurrences (1.67; Cox P=0·03) and a worse OS (1.47; Cox P=0·11) compared with normal weight patients. CONCLUSION: BMI impacts on the efficacy of aromatase inhibitor-based treatment but not single tamoxifen.

Asymptomatic deep vein thrombosis and superficial vein thrombosis in ambulatory cancer patients: impact on short-term survival.

BACKGROUND: Asymptomatic venous thrombotic events (VTEs) are possible findings in ambulatory cancer patients. Data regarding the incidence and clinical impact of asymptomatic VTEs are conflicting. We therefore conducted a study to evaluate the occurrence of asymptomatic VTEs of the lower limbs in ambulatory cancer patients to further evaluate the association of these asymptomatic VTEs on survival during a 9-month follow-up period. METHODS: In our prospective cohort, we included 150 consecutive ambulatory cancer patients who were free of any clinical symptoms for VTEs. Compression ultrasound to detect deep vein thrombosis (DVT) and superficial venous thrombosis (SVT) of the lower limbs was performed by a vascular specialist in all patients at baseline. In case of pathological findings the patients were treated with low molecular weight heparin (LMWH) because of current established guidelines. The occurrence of death was investigated during a 9-month follow-up period. RESULTS: A total of 27 (18%) patients with VTEs were detected, which included 13 patients (8.7%) with a SVT and 16 patients (10.7%) showing a DVT. Two patients had both, a SVT and a DVT as well. During the 9-month follow-up period the occurrence of a VTE at baseline was associated with a 2.4-fold increased risk for death (HR 2.4 (1.2-5.3); P=0.03). CONCLUSION: Asymptomatic VTEs of the lower limbs in ambulatory cancer patients are frequently occurring concomitant features and are associated with poor survival during a 9-month follow-up period despite anticoagulation with LMWH.

Long-term control of bone marrow carcinosis and severe thrombocytopenia with standard-dose chemotherapy in a breast cancer patient: a case report.

BACKGROUND: Primary metastatic breast cancer with bone marrow involvement and pronounced thrombocytopenia is rare. The myelosuppressive effect of most cytotoxic drugs limits chemotherapy in patients with cytopenia due to marrow involvement. CASE REPORT: A 62-year-old patient, who presented with locally and systemically advanced breast cancer, is reported. The initial work-up revealed bone marrow carcinosis with thrombocytopenia of less than 20,000/mm3 lung and osseous metastases without signs of suppressed erythropoiesis and leucopoiesis. The patient was stabilized with 6 different standard-dose chemotherapy regimens, antihormonal therapy, and trastuzumab before dying 57 months after first diagnosis. The patient received only platelet transfusions on 2 instances with platelets of 2,000/mm3. CONCLUSION: This case illustrates that aggressive standard chemotherapy may be feasible in selected patients with bone marrow carcinosis-associated thrombocytopenia without major bleeding episodes.

The nasal bone in fetuses with trisomy 21: sonographic versus pathomorphological findings.

OBJECTIVE: To compare the sonographic findings of the nasal bone in fetuses with trisomy 21 with pathomorphological findings to determine whether the bone is truly absent. METHODS: Seventeen first-trimester fetuses with trisomy 21 were identified; the median gestational age was 12 weeks (range, 11-14) and the median maternal age was 38 (range, 27-47) years. Transabdominal ultrasound examination, preceding transabdominal chorionic villus sampling (TA-CVS) for karyotyping, included assessment of the fetal nose. The nasal bone was determined to be 'hypoplastic' or 'absent' and its length was measured. All pregnancies underwent termination after diagnosis. Serial sagittal sectioning with hematoxylin and eosin-staining of formalin fixed tissue was performed. RESULTS: Of the 17 cases, the nasal bone was sonographically evident, but with severe hypoplasia in 10 cases, absent in six, and in the remaining case it was not able to be assessed due to fetal position. Histomorphologically, in 16 cases a nasal bone was present, detectable by the evidence of an ossification center, and in one case the ossification structure was not clearly visualized. Retrospective review of ultrasound images could identify nasal bones in five of the six cases in which they were initially reported as being absent on ultrasound examination. These were visible, but less distinct and had decreased echogenicity, hence misinterpretation led to the false finding of an absent nasal bone when it was in fact present but hypoplastic. CONCLUSION: Sonographic assessment of the fetal nasal bone should not distinguish between 'present' and 'absent', but instead between 'normal' and 'hypoplastic'. For reproducible results it is necessary to standardize the sonographic examination. The sonographic landmarks of the fetal nose are: the nasal bone, the skin above and the cartilaginous tip of the nose.

Seminoma metastases mimicking primary pancreatic cancer.

BACKGROUND: A case of seminoma clinical stage III, arising from the right testis and mimicking a primary pancreatic malignancy is reported. CASE REPORT: A 57-year-old male patient presented with obstructive jaundice. He suffered from recurrent abdominal pain and significant weight loss over the past 4 months. Abdominal CT scan showed a tumor in the head of the pancreas and multiple pathologically enlarged peripancreatic lymph nodes. In the laboratory findings there were signs of cholestasis and infection. A laparoscopic biopsy out of a suspicious lesion of the head of the pancreas and a surrounding lymph node was done. Histopathological examination reported metastasis of seminoma in a lymph node. Further laboratory findings showed an elevation of the human placental alkaline phosphatase (HPLAP) and urological examination revealed a suspect right testis. The patient underwent castration of the right testis and histopathological examination confirmed a seminoma. 4 cycles of chemotherapy including cisplatinum, etoposide and bleomycin led into complete response that is still ongoing. CONCLUSION: This case shows a seminoma with metastases at retroperitoneal site, mimicking a primary pancreatic neoplasm. It provides an example of the possibility of an uncommon clinical appearance of seminoma metastases and again underlines the importance of exact radiological and histopathological examination to distinguish between curable and incurable tumor.

[Teutschländer disease. A rare benign differential diagnosis in proliferating space-occupying lesions of soft tissues]. / Morbus Teutschländer. Eine seltene benigne Differenzialdiagnose proliferativer Raumforderungen im Weichteilgewebe.

Tumoral calcinosis is a very rare benign soft tissue calcification. It occurs in all age-groups and prefers the shoulder, hip and elbow region as localisation. In most cases, local pain is the leading symptom. The aetiology remains unclear, so far, however, a certain connection to an imbalance of the calcium-/phosphate homeostasis is proposed. The adequate therapy is the complete surgical removal. Our presented case describes an extended occurrence at an unusual localisation and discusses characteristic signs in contrast of the differential diagnoses.

Overproduction of a functional A1 ATPase from the archaeon Methanosarcina mazei Gö1 in Escherichia coli.

Single subunits of the A1 ATPase from the archaeon Methanosarcina mazei Gö1 were produced in E. coli as MalE fusions and purified, and polyclonal antibodies were raised against the fusion proteins. A DNA fragment containing the genes ahaE, ahaC, ahaF, ahaA, ahaB, ahaD, and ahaG, encoding the hydrophilic A1 domain and part of the stalk of the A1AO ATPase of M. mazei Gö1, was constructed, cloned into an expression vector and transformed into different strains of Escherichia coli. In any case, a functional, ATP-hydrolysing A1 ATPase was produced. Western blots demonstrated the production of subunits A, B, C, and F in E. coli, and minicell analyses suggested that subunits D, E, and G were produced as well. This is the first demonstration of a heterologous production of a functional ATPase from an archaeon. The A1 ATPase was sensitive to freezing but lost only about 50% of its activity within 18 days on ice. Inhibitor studies revealed that the heterologously produced A1 ATPase is insensitive to azide, dicyclohexylcarbodiimide and bafilomycin A1, but sensitive to diethylstilbestrol and its analogues dienestrol and hexestrol. The expression system described here will open new avenues towards the functional and structural analyses of this unique class of enzymes.

A double-blind randomized-phase II trial comparing immunization with antiidiotype goat antibody vaccine SCV 106 versus unspecific goat antibodies in patients with metastatic colorectal cancer.

This article reports on the first double-blind randomized clinical study with an antiidiotype antibody vaccine in patients with metastatic colorectal carcinoma. The study was performed to determine immunological parameters, efficacy, and tolerability of the vaccine. Forty-two patients with metastatic colorectal cancer were randomly assigned to multiple immunizations with goat IgG antiidiotype vaccine SCV 106 (n = 21) or unspecific goat IgG as controls (n = 21). The antiidiotype vaccine mimicked the 17-1A glycoprotein antigen associated with colorectal cancer. Of the 42 patients entered, 39 were evaluable for efficacy (SCV 106, n = 18; controls, n = 21). Twenty-nine patients raised antibodies to the vaccines (immunological responders, SCV 106, n = 12; controls, n = 17). Only in the SCV 106 group was a significant increase (p = 0.002) of titers with specificity of antitumor antibody 17-1A found. According to the International Union Against Cancer (UICC) criteria no tumor response was observed. However, in the SCV 106 group the relative increase of carcinoembryonic antigen (CEA) levels between entry and observed disease progression was lower (p = 0.03) and disease progression was determined less frequently by development of new metastases (p = 0.001). On an intention-to-treat basis, the survival time difference between the two groups was not significant. Comparison of immunological responders in both groups revealed a significant survival advantage of the SCV 106-treated patients compared with controls (mean 67 versus 39 weeks; p = 0.01). Immunizations were well tolerated. Vaccination of immunologically responding metastatic colorectal carcinoma patients with SCV 106 leads to slowed disease progression and tumor dissemination and significantly prolongs survival time.

Combined treatment of metastatic osteosarcoma of the spine.

We report on a 28-year-old male with a single metastasis of an osteosarcoma in the twelfth thoracic vertebra occurring 9 years after initial diagnosis of the primary tumour in the left distal femur and neoadjuvant treatment according to a modified T-10 protocol. After pre-operative second-line combination chemotherapy with doxorubicin, carboplatin and etoposide leading to regression of the primarily inoperable metastasis wide resection of the tumour employing total spondylectomy was done. The duration of response had been 65 months since the end of subsequent postoperative chemotherapy with the same regimen.

How pregnancy influences renal function in nephropathic type 1 diabetic women depends on their pre-conceptional creatinine clearance.

Pregnancy in type 1 diabetic women with overt nephropathy can lead to a further deterioration in renal function but it is not clear at what level of pre-conceptional GFR the risk for worsening of renal function begins to increase. Therefore we investigated the influence of pregnancy on renal function in 12 women (14 pregnancies) with pre-conceptional macroproteinuria and near-normal creatinine clearance (range 37-93 ml/min/1.73m2). S-creatinine, creatinine clearance (CrCL), HbA1c and blood pressure (BP) were measured before conception, during each trimester (12th and 24th week of gestation and last week before delivery) and three and six months post-partum. In five diabetic women with six pregnancies (group A) there was a physiological increase in CrCl of 36% up until the 24th week of gestation; their pre-conceptional mean CrCl was 80 (range 70-93) ml/min/1.73m2. In seven women with eight pregnancies (group B) CrCl decreased by 16% during the first two trimesters; the mean CrCl before conception was 61 (37-73) ml/min/1.73m2. In the last week before delivery CrCl worsened transiently in three cases in group A and four in group B, due to pre-eclampsia. Three months post-partum the mean CrCl in group A was 78 (70-91) ml/min/1.73m2, approximately the same as before pregnancy. In group B the mean CrCl was 39 (22-68) ml/min/1.73m2 at this same time; this was 36% lower than the pre-conceptional clearance. Mean HbA1c in both groups were approximately the same, but mean BP tended to be higher during pregnancy in group B, especially in the week before delivery (p<0.05). We conclude that in a high percentage of nephropathic diabetic women with significantly low CrCl before conception, renal function worsens during and after pregnancy. Inadequate antihypertensive therapy may contribute to this.

Adjuvant radiotherapy in male breast cancer.

PURPOSE: To determine retrospectively the outcome of postoperative radiation therapy in male breast cancer. Local/distant control was assessed with attention to age, stage, lymph node involvement, histopathological differentiation and hormone receptor status. MATERIALS AND METHODS: Thirty-one male patients were irradiated postoperatively at the chest wall (mean dose 50 Gy) and 16 patients received radiation to regional lymph nodes. Tumour distribution by stage was: stage 0 (9.7%), stage I (22.6%), stage II (32.2%) and stage III (35.5%). Nine patients were subjected to additional hormone therapy and three patients to chemotherapy. RESULTS: Local control was achieved in 30/31 (96.8%) patients. Kaplan-Meier estimates of overall survival (OS), disease specific (DSS) and disease free survival (DFS) at 5 years were 77% (95% confidence interval (CI), 0.61-0.93), 84% (CI, 0.69-0.98) and 73% (CI, 0.57-0.91), respectively. Five-year DFS for stage 0 + I, II and III was 100, 56.3 and 67.3%, respectively. Favourable results were observed in patients with negative axillary nodes with 5-year OS/DFS of 90.9% (CI, 0.74-1.0). For lymph node positive patients DFS was 71% (CI, 0.4-1.0). Patients who presented lymph node metastases with extracapsular extension the 5-year OS was 80% (CI, 0.45-1.00), but the DFS was 0%. Stage of disease, lymph node involvement and histological differentiation were found to have statistically significant influence on DFS, but not on OS. CONCLUSION: Application of postoperative radiotherapy approved in females resulted in one local relapse in our study population. Other treatment modalities (hormone therapy/chemotherapy) should continue to be considered a necessary treatment option for appropriately selected patients.

Dose intensification of epidoxorubicin and cyclophosphamide in metastatic breast cancer: a randomised study with two schedules of granulocyte-macrophage colony stimulating factor.

A randomised phase II/III study was conducted in patients with advanced breast cancer to determine the dose intensity achievable through an acceleration of administration of chemotherapy with epidoxorubicin and cyclophosphamide (EC) alone, as compared with the combination of this regimen with two different schedules of granulocyte-macrophage colony stimulating factor (GM-CSF). 73 patients received EC intravenous (i.v.) (epidoxorubicin 100 mg/m2, cyclophosphamide 600 mg/m2) on day 1 (group A), or the same chemotherapy plus sub-cutaneous (s.c.) GM-CSF (5 micrograms/kg/day) either from days 3 to 12 (group B) or from days -6 to -3 (group C). The primary objective of the study was the investigation of dose intensity delivered in the three treatment arms, whereas the secondary objective was response rate. A significant increase (P = 0.006) in dose intensity of 21% was observed for treatment group B, whereas the increase in dose intensity achieved in group C (7%) was not significant (P = 0.086). Response rates (complete response (CR) + partial response (PR)) of 56% were observed in group A, 65% in group B, and 57% in group C, respectively. This difference in response rates did not reach statistical significance (P = 0.271). We thus conclude that an acceleration of the EC regimen over the standard schedule could be accomplished with postchemotherapeutic GM-CSF support, leading to an increase in dose intensity, whereas pretherapeutic short-term GM-CSF administration did not reach this goal.

Sequential treatment of recurrent mesenteric desmoid tumor.

BACKGROUND: The optimal management of inoperable desmoid tumors is still unclear. We report a 26 year-old female patient with familial adenomatous polyposis suffering from a recurrent inoperable intraabdominal desmoid tumor and its sequential treatment. METHODS: Treatment strategies included low-dose tamoxifen (30 mg orally per day), high-dose tamoxifen (90 mg orally per day), and a subsequent combination of goserelin acetate (3.6 mg subcutaneously once every four weeks) plus low-dose tamoxifen, medroxyprogesterone acetate (1000 mg orally per day) and interferon gamma (3 Mio IU subcutaneously 3 times a week). RESULTS: The combination of goserelin acetate and low-dose tamoxifen resulted in a decrease in tumour size and a complete relief of symptoms for 17 months. Thereafter the tumor progressed and again growth was stopped with interferon gamma therapy for another 6 months. All other treatment modalities had no effect. CONCLUSIONS: This study demonstrates long-term regression of a desmoid tumor with combined endocrine therapy using goserelin acetate plus tamoxifen. Tumor progression after 17 months was again stopped by a combination of interferon-gamma and goserelin acetate.