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Sarcomas are rare and have a high mortality rate. Further prognostic classification, with readily available parameters, is warranted, and several studies have examined circulating biomarkers and PET parameters separately. This single-site, retrospective study aimed to examine the prognostic values of several scoring systems in combination with PET parameters. We included 148 patients with sarcoma, who were treated and scanned at Aarhus University Hospital from 1 January 2016 to 31 December 2019. The Akaike information criterion and Harrell’s concordance index were used to evaluate whether the PET parameters added prognostic information to existing prognostic models using circulating biomarkers. Of the PET parameters, metabolic tumor volume (MTV) performed best, and when combined with the existing prognostic models, the prognostic value improved in all models. Backward stepwise selection was used to create a new model, SBSpib, which included albumin, lymphocytes, and one PET parameter, MTV. It has scores ranging from zero to three and increasing hazard ratios; HR = 4.83 (1.02–22.75) for group one, HR = 7.40 (1.6–33.42) for group two, and HR = 17.32 (3.45–86.93) for group three. Consequently, implementing PET parameters in prognostic models improved the prognostic value. SBSpib is a new prognostic model that includes both circulating biomarkers and PET parameters; however, validation in another sarcoma cohort is warranted.

Background: Adjustment for comorbidity when investigating potential prognostic factors, especially in elderly cancer patients, is imperative. Patients diagnosed with chondrosarcoma are elderly and more comorbidity is expected for these patients. Demographic changes are awaited in the future resulting in more and more elderly patients with comorbidity. The aims of this study were to characterize patients with chondrosarcoma treated at a single institute and to evaluate various prognostic factors for survival adjusted for comorbidity.Material and methods: Between 1979 and 2008, 199 patients were treated at the Sarcoma Centre of Aarhus University Hospital, for chondrosarcoma. The incidence was calculated as a WHO age-standardized incidence rate (IR) per million per year. The endpoints were overall mortality and disease-specific mortality. Possible prognostic factors were analyzed for patients with intermediate/high-grade localized tumors by the uni- and multivariate Cox-proportional hazard method.Results: The WHO age-standardized IR in western Denmark in the period 1979-2008 was 2.4/million inhabitants/year (95% CI: 2.2;2.6). The 5-year overall and disease-specific mortality for the 199 patients were 29% (95% CI: 23;36) and 22% (95% CI: 16;27), respectively. The 5-year disease-specific mortality for patients with metastatic disease was significantly higher than for patients with localized disease. The median time to relapse was 2.0 years. Patients who relapse within 1 year after the primary diagnosis have a significantly higher 5-year overall mortality compared to patients who relapse after 1 year. The presence of comorbidity and high-grade tumors were independent prognostic factors for both the overall mortality and the disease-specific mortality of chondrosarcoma patients.Conclusion: Patients with comorbidity had a significantly increased overall mortality and disease-specific mortality. We found that adjusting for comorbidity is important when investigating a cohort of elderly patients.

The present case illustrates the diagnostic challenges in symptomatic patients with heart failure of unknown etiology. The patients were previously diagnosed with κ-light chain amyloidosis without cardiac involvement. Echocardiography showed heart failure with mildly reduced ejection fraction but no signs of amyloidosis. Coronary angiogram showed normal arteries and 11C-PIB positron emission tomography was negative for amyloid deposits. Exercise testing revealed severe heart failure and reduced coronary flow velocity reserve. Endomyocardial biopsies showed amyloid in the intramural coronary arteries without interstitial amyloid deposits. Hence, the patient was diagnosed with microvascular dysfunction-induced heart failure due to vessel wall amyloidosis.

OBJECTIVE:Certain biomarkers such as the C-reactive protein, serum albumin, and the neutrophils to lymphocyte ratio are of prognostic significance regarding survival in different types of cancers. Data from sarcoma patients are sparse and mainly derived from soft tissue sarcoma and/or metastatic cases. Adjusting for confounders such as comorbidity and age is an essential safeguard against erroneous conclusions regarding the possible prognostic value of these biomarkers. The aim of this study was to assess the prognostic value of a battery of pretreatment biomarkers in the serum of patients with localized bone sarcomas and to adjust for potential confounders.MATERIAL AND METHODS:All patients diagnosed with localized intermediate and high-grade bone sarcoma during 1994 to 2008 were extracted from the Aarhus Sarcoma Registry. The serum levels of albumin, C-reactive protein, hemoglobin, neutrophils, lymphocytes, and sodium were collected from the patient records. The prognostic values of overall and disease-specific mortality were tested for each individual biomarker as well as for the Glasgow prognostic score (GPS) and for a new composite score incorporating five biomarkers (Aarhus composite biomarker score: ACBS). Adjustments were made for comorbidity as well as other possible prognostic factors, such as size, histological type, margin, chemotherapy, and soft tissue extension, using the Cox proportional hazard model.RESULTS:A total of 172 patients with high- or intermediate-grade localized bone sarcoma were included. Of these patients, 63 were diagnosed with chondrosarcoma and 109 patients with Ewing/osteosarcoma. The median age was 55 years for chondrosarcoma and 19 years for Ewing/osteosarcoma patients. The overall 5-year mortality was 31% [95% confidence interval (CI): 21-44] and 41% (95% CI: 33-51), whereas the 5-year disease-specific mortality was 21% (95% CI: 12-34) and 39% (95% CI: 31-49) for chondrosarcoma and Ewing/osteosarcoma, respectively. Comorbidities were present in 12% of the Ewing/osteosarcoma patients and in 24% of the chondrosarcoma patients. After adjustment for comorbidity and other confounders, it was found that elevated levels of CRP, low hemoglobin, low sodium, high GPS, and high ACBS were associated with increased overall mortality. Furthermore, elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were associated with increased disease-specific mortality.CONCLUSION:Elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were all independent prognostic factors for both overall and disease-specific mortality. ACBS is a new three-level score of five biomarkers, but its value has to be confirmed in an independent data set.

AIM: The aim of this study was to contribute to the collected knowledge of prognostic factors in primary breast sarcomas (PBS) to the benefit of possible future prospective studies and therapeutic guidelines.METHOD: All patients with pathologically verified PBS in the period of 1979-2014 were extracted from a hospital-based database at Aarhus University Hospital. All records were reviewed for patient and tumor characteristics. Primary endpoints were overall survival, disease-free survival (DFS) and disease-specific survival (DSS). Adjustments were made for age, tumor location, surgical strategy, size, histological classification, prior radiation and grade. Prognostic factors were determined by the use of Cox proportional hazard ratio.RESULTS: In total 42 patients were identified. Surgical resection was the main method of treatment. Nineteen (45%) patients were initially selected for lumpectomy, of these 68% needed at least one re-excision to attain wide margins. In total 55% experienced recurrence, loco regional in 43%. Five-years overall survival was 49%, five-year DFS was 48% and five-year DSS was 40%. Significant prognostic factors were size and grade. A trend towards better survival in those with superficial tumors was observed as well as an increased incidence in radiation-induced angiosarcoma (AS) of the breast, however, prognosis was no different from non-radiation-induced AS.CONCLUSION: Prognostic factors in PBS patients were size and grade with a trend towards better survival in those with superficial tumors. There was no difference in survival between radiation-induced and spontaneous breast sarcomas. High rate of local recurrence suggests the need for aggressive surgical approach or the routine addition of postoperative radiotherapy in those selected for breast conserving surgery (BCS).

Ga-PSMA PET/CT was performed in a 75-year-old man with newly diagnosed prostate cancer because of an equivocal lesion in the spine both on Tc-bone-SPECT/CT and MRI. Because of increased PSMA activity on PET/CT, the bone lesion was interpreted as metastasis from prostate cancer. Later, the patient was diagnosed as having monoclonal gammopathy of unknown significance. A biopsy was performed, and histological examination revealed multiple myeloma with PSMA expression in the neovessels but no metastatic prostate cancer cells. The patient was downstaged, and the treatment was changed accordingly. This case illustrates the importance of biopsies from PSMA PET-positive lesions.

OBJECTIVE: Reverse transcription quantitative real-time polymerase chain reaction is efficient for quantification of gene expression, but the choice of reference genes is of paramount importance as it is essential for correct interpretation of data. This is complicated by the fact that the materials often available are routinely collected formalin-fixed, paraffin-embedded (FFPE) samples in which the mRNA is known to be highly degraded. The purpose of this study was to investigate 22 potential reference genes in sarcoma FFPE samples and to study the variation in expression level within different samples taken from the same tumor and between different histologic types.METHODS: Twenty-nine patients treated for sarcoma were enrolled. The samples encompassed 82 (FFPE) specimens. Extraction of total RNA from 7-μm FFPE sections was performed using a fully automated, bead-base RNA isolation procedure, and 22 potential reference genes were analyzed by reverse transcription quantitative real-time polymerase chain reaction. The stability of the genes was analyzed by RealTime Statminer. The intrasamples variation and the interclass correlation coefficients were calculated. The linear regression model was used to calculate the degradation of the mRNA over time.RESULTS: The quality of RNA was sufficient for analysis in 84% of the samples. Recommended reference genes differed with histologic types. However, PPIA, SF3A1, and MRPL19 were stably expressed regardless of the histologic type included. The variation in ∆Cq value for samples from the same patients was similar to the variation between patients. It was possible to compensate for the time-dependent degradation of the mRNA when normalization was made using the selected reference genes.CONCLUSION: PPIA, SF3A1, and MRPL19 are suitable reference genes for normalization in gene expression studies of FFPE samples from sarcoma regardless of the histology.

e-Cigarettes have gained worldwide popularity as a substitute for smoking, but concern has been raised regarding the long-term effects associated with their use. We report a case of a 45-year-old female consumer of e-cigarettes who presented with 4 months of abdominal pain and fever. Initial imaging discovered multiple pulmonary nodules and liver lesions suspicious of widespread metastases; however, an extensive evaluation found no evidence of malignancy. Results of a lung biopsy revealed an area with multinucleated giant cells suggestive of a foreign body reaction to a lipophilic material. Upon cessation of e-cigarette use (known as vaping), the lung nodules disappeared, and the liver lesions regressed. Our case report suggests that vaping can induce an inflammatory reaction mimicking metastatic cancer.

According to the WHO histological classification of odontogenic tumours, odontomas originate from odontogenic epithelium and odontogenic ectomesenchyme, with or without hard tissue formation. They are generally classified into two types: complex and compound. Odontomas are usually intraosseous and often associated with delayed eruption of teeth. However, they can be extraosseous and are then referred to as either peripheral complex or compound odontoma. Peripheral odontomas are rare entities. We report a case of an 11 year old boy referred to the Department of Oral and Maxillofacial Surgery, Aarhus University Hospital by the boy's dentist due to fibrous mass in the marginal gingiva in the anterior lower mandible. Removal of the mass revealed a peripheral complex odontoma and at the final 5-month postoperative inspection there was satisfying healing observed, a small, elevated, hyperplastic fibrous marginal gingiva.

Background. Comorbidity is an important prognostic factor for survival in other cancers, but the importance in soft tissue sarcoma has not yet been clarified. The aims of this study were to examine the prevalence of comorbidity in soft tissue sarcoma patients, and estimate the impact of comorbidity on overall and disease-specific mortality.Material and methods. Overall, 1210 adult patients with soft tissue sarcoma in the extremities or trunk wall were identified through the Aarhus Sarcoma Registry, a validated population-based database. Information on comorbidity was obtained through the National Patient Registry, and a Charlson's Comorbidity score was calculated for each patient. The prevalence of comorbidity was assessed overall, as well as according to age and year of diagnosis. Overall and disease-specific mortality rates according to level of comorbidity were computed. The prognostic value of comorbidity was estimated using crude and adjusted Cox proportional hazard models.Results. The overall prevalence of comorbidity was 25%. The prevalence increased with increasing age, and patients with comorbidity had a larger proportion of adverse prognostic factors when compared to patients without comorbidity. The five-year disease-specific mortality was 26% (95% CI 24–29) for patients without comorbidity, compared to 33% (95% CI 24–42), 41% (95% CI 32–50), and 44% (95% CI 33–55) for patients with mild, moderate, and severe comorbidity, respectively. After adjusting for age, sex, stage, tumor size, depth, grade, surgical margin, radiotherapy, and chemotherapy, comorbidity was independently associated with an increased overall and disease-specific mortality.Conclusion. Patients with comorbidity had significantly increased overall and disease-specific mortality compared to patients without comorbidity, even when adjusting for important prognostic factors including age.

Patients with cardiac amyloidosis are at increased AV-block and syncope risk. Therefore, a prophylactic pacemaker is often implanted. However, this case illustrates that other mechanisms should be ruled out prior to pacemaker implantation. The patient studied had mitral valve thickening without increased left ventricular outflow track (LVOT) velocity. However, bicycle exercise-stress test with simultaneous echocardiography revealed a stepwise decrease in blood pressure, a substantial increase in the LVOT velocity, and severe systolic anterior motion of the mitral valve. The patients’ symptoms were likely explained by these findings. Therefore, a comprehensive clinical evaluation is warranted prior to pacemaker implantation in cardiac amyloidosis patients.

BACKGROUND: For decades, tumour hypoxia has been pursued as a cancer treatment target. However, prognostic and predictive biomarkers are essential for the use of this target in the clinic. This study investigates the prognostic value of a hypoxia-induced gene profile in localised soft tissue sarcoma (STS).METHODS: The hypoxia-induced gene quantification was performed by real-time quantitative PCR (RT-qPCR) of formalin-fixed, paraffin-embedded tissue samples. The gene expression cut-points were determined in a test cohort of 55 STS patients and used to allocate each patient into a more or a less hypoxic group. The cut-points found in the test cohort were applied to a cohort of 77 STS patients for validation.RESULTS: For patients with localised high-grade STS treated with surgery with or without postoperative radiation therapy, the prognostic value of the hypoxia-induced gene profile was proved in the test cohort and confirmed in the validation cohort. After adjustment for confounders, the hazard ratio (HR) was 3.2 (95% CI: 1.5; 7.0) for patients with more hypoxic tumours compared with patients with less hypoxic tumours regarding disease-specific survival. Moreover, for the development of metastatic disease, the HR was 2.61 (95% CI: 1.27; 5.33).CONCLUSIONS: The hypoxia-induced gene profile is a validated independent prognostic marker that may help identify STS patients needing more aggressive or different adjuvant treatment.

Solitary sellar plasmacytomas are exceedingly rare and difficult to distinguish from other pituitary tumors. We report a case of a 62-year-old woman presenting with blurred vision of the right eye and tenderness of the right temporal region, which was interpreted as temporal arteritis. MRI revealed a pituitary mass lesion (20mm×14mm×17mm) without compression of the optic chiasm and her pituitary function was normal. Pituitary surgery was undertaken due to growth of the lesion, and histopathological examination showed a highly cellular neoplasm composed of mature monoclonal plasma cells. Subsequent examinations revealed no evidence of extrasellar myeloma. The patient received pituitary irradiation and has remained well and free of symptoms apart from iatrogenic central diabetes insipidus. Until now, only eight cases of solitary sellar plasmacytoma have been reported. Most frequent symptoms stem from compression of the cranial nerves in the cavernous sinus (III, IV, V), whereas the anterior pituitary function is mostly intact. Learning points A solitary plasmacytoma is a rare cause of a sellar mass lesion. The radiological and clinical features are nonspecific, but cranial nerve affection and intact pituitary function are usually present. The diagnosis is made histologically and has important therapeutic implications. Background Plasmacytomas are characterized by monoclonal plasma cell proliferation in a single lesion, whereas multiple myeloma is a multifocal plasma cell neoplasm characterized by elevated serum monoclonal protein levels and/or increased bone marrow clonal cells with related organ or tissue impairment (increased calcium levels, renal insufficiency, anemia, and bone lesions). Solitary plasmacytomas are uncommon and can either arise from bone or soft tissue (1). Solitary sellar plasmacytomas are exceedingly rare and to date there has only been eight reported cases without progression to multiple myeloma (2, 3, 4, 5, 6, 7). Clinically and radiologically, sellar plasmacytomas mimic benign pituitary tumors, which make the differential diagnosis difficult (3, 6). We present the ninth case of a solitary intrasellar plasmacytoma, which revealed several emblematic features.

A 38-year old woman, with a history of monostotic fibrous dysplasia (MFD) of the right femur, developed secondary transformationto both an osteosarcoma and an aneurysmal bone cyst. The patient was initially treated with wide resection and insertion of a hipprosthesis and postoperative chemotherapy. After 3 months she had a recurrence and a hemipelvectomy was performed, followedby a new series of chemotherapy. Seven years after treatment the patient is still alive with no signs of metastasis. It is unknownwhether the osteosarcoma developed from the MFD or from the aneurysmal bone cyst. Secondary transformation of fibrous dysplasia into osteosarcoma is a rare event, especially in MFD without concomitant McCune-Albright syndrome or in patients with MFD without prior radiation therapy. Transformation of an aneurysmal bone cyst is aneven more rare event. This case is reported due to the rare event of malignant transformation of MFD with a concomitant aneurysmal bone cyst and dueto the favorable outcome, in spite of the poor prognosis associated with the condition.

Angiosarcoma (AS) is a rare and often high-grade malignant vascular tumor [1]. They represent below 2% of all soft tissue sarcomas and present in a broad variety of anatomic locations [1,2]. Predis...

We describe a case of cardiac amyloidosis in a 69-year-old man. Genetic analysis demonstrated a rare mutation in transthyretin (TTR): TTRA45S, p.(Ala65Ser) previously only described in two patients...

A 64-year-old previously healthy woman consulted her general practitioner because of recurrent episodes of right-sided monocular transient visual loss (ie, amaurosis fugax). At first, these symptoms were followed over time, but as the attacks worsened, and were accompanied by dizziness and general discomfort, the patient was admitted to the department of neurology for further investigations. CT of the brain was normal; however, during admission, the patient developed rapid atrial fibrillation and was transferred to the department of cardiology. Transthoracic echocardiography revealed a massive tumour on the atrial side of the anterior mitral valve leaflet, partly obstructing the mitral valve inflow. The tumour was excised and a biological prosthetic mitral valve inserted. The tumour was histologically determined to be a highly malignant dedifferentiated chondrosarcoma. After 6 months, the tumour relapsed and expanded aggressively to completely obstruct the mitral valve inflow, ultimately leading to cardiac arrest and death.

e-Cigarettes have gained worldwide popularity as a substitute for smoking, but concern has been raised regarding the long-term effects associated with their use. We report a case of a 45-year-old female consumer of e-cigarettes who presented with 4 months of abdominal pain and fever. Initial imaging discovered multiple pulmonary nodules and liver lesions suspicious of widespread metastases; however, an extensive evaluation found no evidence of malignancy. Results of a lung biopsy revealed an area with multinucleated giant cells suggestive of a foreign body reaction to a lipophilic material. Upon cessation of e-cigarette use (known as vaping), the lung nodules disappeared, and the liver lesions regressed. Our case report suggests that vaping can induce an inflammatory reaction mimicking metastatic cancer.

We have recently published a case report describing the first case of grafting frozen/thawed ovarian tissue for puberty induction to a young girl who had recovered from a malignant disease [1]. She had one of her ovaries frozen at 9 years of age prior to undergoing treatment for Ewing sarcoma, which included pre— and post operative chemotherapy comprising (total accumulated dose per m2), vincristine (21 mg), ifosfamide (60 g), doxorubicin (360 mg), etoposide (2.7 mg), actinomycin (9 mg), and cyclophosphamide (10.5 g) according to the EURO EWING-99 protocol. Subsequently, she underwent gross total surgical resection. Histological examination confirmed resection into healthy tissue. Postoperatively, a total irradiation dose of 41.2 Gy to the tumor bed during 23 treatments was given. When she was 13 years and had no ovarian activity, two of ten pieces of ovarian cortex were grafted to the remaining postmenopausal ovary. The grafted tissue regained activity and stimulated her pubertal development.

Background Cancer-specific survival estimates rely on precise and correct data on the cause of death; however, these data can be difficult to acquire, particularly in elderly patients where comorbidity is common. Furthermore, while some deaths are directly related to cancer, others are more complex, with cancer merely contributing. Another, more precise, method is to assess the relative mortality, i.e., mortality in patients compared to the general population. The aims of this study were to describe the relative mortality in soft tissue sarcoma, and to compare the relative mortality with the cancer-specific mortality. Methods We included 1246 patients treated for soft tissue sarcoma and 6230 individually age- and sex-matched individuals from the general population. The relative mortality was estimated as rates, and rate ratios adjusted for comorbidity. Mortality rate ratios were computed using the Cox proportional hazard model for 0–5 years and 5–10 years, according to age, sex and level of comorbidity. The cancer-specific mortality was compared to the corresponding relative mortality. Results The overall 5- and 10-year relative mortality was 32.8% and 36.0%. Patients with low-grade soft tissue sarcoma did not have increased mortality compared with the general population. Soft tissue sarcoma patients had a 4.4 times higher risk of dying within the first five years after diagnosis and a 1.6 times higher risk between five and ten years compared with the general comparison cohort. The relative mortality varied according to age, grade, stage at diagnosis, and level of comorbidity, being highest in younger patients and in patients without comorbidity. The overall 5- and 10-year cancer-specific mortality was underestimated by 1.5 and overestimated by 0.7 percentage points compared to the relative mortality, respectively. No statistical significant difference between the relative and the cancer-specific mortality was found. Conclusion The relative mortality provides an unbiased and accurate method to differentiate between cancer-specific and non-cancer-specific deaths. However, when data on the cause of death is of a sufficient quality, there is no difference between relative mortality and disease-specific mortality based on death certificates. Electronic supplementary material The online version of this article (doi:10.1186/1471-2407-14-682) contains supplementary material, which is available to authorized users.

Background. Treatment of high-grade osteosarcoma remains a major challenge in orthopedic oncology as no major breakthrough in overall survival has occurred in the past 20 years. Due to the rarity of the disease, comparing the results of a single institution to best standard practice needs the establishment of clinical databases. The aim of this study was to report the cumulative 30-years' experience of a single institution and to assess the incidence, survival and prognostic factors of high-grade osteosarcoma using a recently validated, hospital-based database, representing all citizens living in western Denmark, the Aarhus Sarcoma Registry. Material and methods. Between 1979 and 2008, 169 patients were treated at the Sarcoma Centre of Aarhus University Hospital for high-grade osteosarcoma. The incidence was calculated as a WHO age-standardized incidence per million per year. The endpoint was overall survival, analyzed by the Kaplan-Meier method and log-rank. Possible prognostic factors were analyzed by the uni- and multivariate Cox proportional hazard method. Results. The incidence of high-grade osteosarcoma in western Denmark from 1979 to 2008 was 2.7/million inhabitants/year. The five-year overall survival was 42% (95% CI 34; 49) for the whole cohort of patients with high-grade osteosarcoma and 54% (95% CI 43; 64) for patients with localized disease treated with wide excision and chemotherapy. For patients treated with curative intent, no soft tissue extension, treatment with sufficient surgical margin and standard chemotherapy, as well as a high degree of necrosis after chemotherapy were all independent prognostic factors for overall survival. Conclusion. The data from this hospital-based, validated database confirms the relevance of the known prognostic factors of high-grade osteosarcoma and emphasizes the importance of adequate surgical margins and chemotherapy.

BACKGROUND AND PURPOSE: Previous studies of soft tissue sarcoma (STS) have identified a number of possible prognostic factors; however, the majority of these include highly selected populations, with unclear validation of data and insufficient statistical methods. We identified prognostic factors in a validated, population-based 30-year series of STS treated at a single institution, using an advanced statistical approach.PATIENTS AND METHODS: Between 1979 and 2008, 922 adult patients from western Denmark were treated at the Aarhus Sarcoma Center for non-metastatic STS in the extremities or trunk. The endpoints were local recurrence (LR) and disease-specific mortality (DSM). Prognostic factors were analyzed using a proportional hazard model, including continuous variables as cubic splines. Directed acyclic graphs were used to depict the causal structure.RESULTS: The 5-year LR was 16% and the 5-year DSM was 24%. Important prognostic factors for both LR and DSM were age, duration of symptoms, tumor size, grade, margin, and radiotherapy, while anatomical location (upper, lower extremity, trunk) was prognostic for DSM.INTERPRETATION: In this population-based series of adult, non-metastatic STS, we included directed acyclic graphs, cubic splines, and a competing risk model in order to minimize bias, and demonstrated that these statistical methods are feasible. Using these statistical methods on a large, validated dataset, we excluded depth as a prognostic factor and established that age, duration of symptoms, size, grade, margin, and radiotherapy were important prognostic factors for both local recurrence and disease-specific mortality.

We report a 79-year-old woman with a left sided neck mass that appeared to be a thoracic duct cyst (TDC). A TDC is a rare condition. Generally, it may cause local symptoms, but most likely it presents as an asymptomatic swelling. The pathogenesis of TDC is not known, but different theories on the development, including weakness of the duct wall and obstruction of the lymphoid flow, have been suggested. Sonography with needle aspiration, examination of the cyst fluid, and CT scan/MRI may raise the suspicion of the TDC diagnosis. Surgery with total excision of the cyst, followed by histopathological examination confirms the diagnosis.

Katja Maretty-Nielsen,1,2 Ninna Aggerholm-Pedersen,1–3 Johnny Keller,1,4 Akmal Safwat,1,3 Steen Baerentzen,1,5 Alma B Pedersen6 1Sarcoma Centre of Aarhus University Hospital, 2Department of Experimental Clinical Oncology, 3Department of Oncology, 4Department of Orthopedic Surgery, 5Department of Pathology, 6Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Background: The aim of the present study was to validate the data in the Aarhus Sarcoma Registry (ASR), to determine if this registry is population-based for western Denmark, and to examine the incidence of sarcomas using validated, population-based registry data. Methods: This study was based on patients with bone and soft tissue sarcoma treated at the Sarcoma Centre of Aarhus University Hospital between January 1, 1979 and December 31, 2008. The validation process included a review of all medical files by two researchers using a standardized form. The Danish Cancer Registry was used as a reference to assess the completeness of registration of patients in the ASR. Crude and World Health Organization age-standardized incidence, as well as age-, gender-, and year-specific incidences were estimated. Results: The validation process added 385 to the 1442 patients who were registered in the ASR. Before validation, on average, 70.5% of the data for the variables was correct. Validation improved the average completeness of the registered variables from 83.7% to 99.3%. The 1827 patients in the ASR after validation include 85.3% of the patients registered in the Danish Cancer Registry. The overall World Health Organization age-standardized incidence of sarcoma in the trunk or extremities in western Denmark in the period 1979–2008 was 2.2 per 100,000, being 0.8 for bone sarcomas and 1.4 for soft tissue sarcomas. Conclusion: The validation process significantly improved the completeness of the variables and the quality of the ASR data. ASR is now a valuable population-based tool for epidemiological research and quality improvement in the treatment of sarcoma. It is our recommendation that documented validation of registries should be a prerequisite for publishing studies derived from them. Keywords: clinical databases, cancer registry, sarcoma, incidence, completeness, validity&nbsp

To the Editor,With an average annual incidence rate of only 0.8 per 100 000 person-years [1], pheochromocytoma (PHEO) and paraganglioma (PGL) are rare neuroendocrine tumors (NET). The tumors arise ...

Infantile myofibromatosis is a common fibrous tumor of infancy, however still considered a rarity. A newborn boy was examined due to inspiratory stridor and subcostal retractions since birth. Several subcutaneous nodules were present on the trunk and legs. Transnasal fiberoptic laryngoscopy and trachea-laryngoscopy showed discrete edema of the anterior part of the vocal cords but no other apparent abnormality. MRI raised suspicion of a laryngeal tumor. Through an external laryngofissure a lesion extending from the right hemilarynx was exposed and excised in fractions. Pathological examination showed infantile myofibromatosis. Stridor in newborns is frequently caused by congenital laryngeal anomalies, i.e. laryngomalacia but a neoplastic process should be considered as a differential diagnosis.