Extraordinary Presentation of Elastofibroma Dorsi After a Thoracotomy Procedure.

Elastofibroma dorsi is a reactive pseudotumor of connective tissue, typically located in the infrascapular region. Awareness of this benign entity is crucial for radiologists, as well as clinicians, to avoid misdiagnosis and potential patient harm. In this report, we present clinical and imaging findings of an elastofibroma dorsi after a thoracotomy procedure.

[Sarcoma of the chest wall after radiotherapy for breast carcinoma - a case report]. / Sarkom hrudní steny po ozárení pro karcinom prsu kazuistika.

INTRODUCTION: Sarcoma occurring in soft tissues after radiotherapy is a rare complication of radiation treatment of tumours. It was most often described after treatment for breast cancer as well as for non-Hodgkin lymphoma and cervical carcinoma. The time interval between the radiation therapy and the development of the sarcoma can be very wide. Treatment demands radical surgical resection of the sarcoma with the edge of the resected tissue without tumour cells. In some cases, this is followed by chemotherapy or radiotherapy. The median survival time is 23 months, the longest survival being associated with sarcomas removed in a radical way. CASE REPORT: We present the case of a female patient with recurring leiomyosarcoma of the chest wall after radiotherapy for cancer of the right breast. In 2006, this 62-year-old patient was operated on to keep her right breast with axilla exenteration. After the surgery, hormonal therapy was followed by adjuvant radiotherapy of the right breast and the adjacent axilla. We used a linear accelerator and the total amount of radiation was 50 Gy (2 Gy fractionally once a day, five days a week). Four years after the operation, leiomyosarcoma was diagnosed in the pectoral muscle at the site where the tumour of the right breast had been excised. Between 2011 and 2013, a total of five operations of re-occurring sarcoma were performed - two excisions of the tumour, a mastectomy, rib resection and, at last, block resection of the chest wall. Adjuvant oncological treatment was not indicated. The patient, now being 69 years old, is still in a good physical and mental condition without any generalization of the disease. CONCLUSION: Sarcoma of the chest wall is a relatively rare consequence of radiotherapy for breast cancer. Sarcoma treatment involves radical surgical resection of the tumour whenever possible. The surgery is mostly followed by radiotherapy which, however, is impossible in a patient after breast-preserving surgery for carcinoma with radiotherapy. Chemotherapy is not very effective in sarcomas. Therefore, the operation needs to be performed by an experienced surgeon in a sufficiently radical way.

Effect of a combined surgery, re-irradiation and hyperthermia therapy on local control rate in radio-induced angiosarcoma of the chest wall.

PURPOSE: Radiation-induced angiosarcoma (RAS) of the chest wall/breast has a poor prognosis due to the high percentage of local failures. The efficacy and side effects of re-irradiation plus hyperthermia (reRT + HT) treatment alone or in combination with surgery were assessed in RAS patients. PATIENTS AND METHODS: RAS was diagnosed in 23 breast cancer patients and 1 patient with melanoma. These patients had previously undergone breast conserving therapy (BCT, n = 18), mastectomy with irradiation (n=5) or axillary lymph node dissection with irradiation (n = 1). Treatment consisted of surgery followed by reRT + HT (n = 8), reRT + HT followed by surgery (n = 3) or reRT + HT alone (n = 13). Patients received a mean radiation dose of 35 Gy (32-54 Gy) and 3-6 hyperthermia treatments (mean 4). Hyperthermia was given once or twice a week following radiotherapy (RT). RESULTS: The median latency interval between previous radiation and diagnosis of RAS was 106 months (range 45-212 months). Following reRT + HT, the complete response (CR) rate was 56 %. In the subgroup of patients receiving surgery, the 3-month, 1- and 3-year actuarial local control (LC) rates were 91, 46 and 46 %, respectively. In the subgroup of patients without surgery, the rates were 54, 32 and 22 %, respectively. Late grade 4 RT toxicity was seen in 2 patients. CONCLUSION: The present study shows that reRT + HT treatment--either alone or combined with surgery--improves LC rates in patients with RAS.

Chest wall leiomyosarcoma after breast-conservative therapy for early-stage breast cancer in a young woman with Li-Fraumeni syndrome.

Li-Fraumeni syndrome (LFS) is one of the most penetrant forms of familial cancer susceptibility syndromes, characterized by early age at tumor onset and a wide spectrum of malignant tumors. Identifying LFS in patients with cancer is clinically imperative because they have an increased sensitivity to ionizing radiation and are more likely to develop radiation-induced secondary malignancies. This case report describes a young woman whose initial presentation of LFS was early-onset breast cancer and whose treatment of this primary malignancy with breast conservation likely resulted in a secondary malignancy arising in her radiation field. As seen in this case, most breast cancers in patients with LFS exhibit a triple-positive phenotype (estrogen receptor-positive/progesterone receptor-positive/HER2-positive). Although this patient met classic LFS criteria based on age and personal and family history of cancer, the NCCN Clinical Practice Guidelines in Oncology for Genetic/Familial High-Risk Assessment: Breast and Ovarian Cancer endorse genetic screening for TP53 mutations in a subset of patients with early-onset breast cancer, even in the absence of a suggestive family history, because of the potential for de novo TP53 mutations.

Post-traumatic chest wall lipoma in a violinist: fact or fiction?

Lipomas are benign soft tissue tumours that can occur anywhere on the body and are rarely encountered on the chest. The pathophysiology between soft tissue trauma and lipoma development is not fully understood, and various theories have been presented. We present the case of a violinist with a 40-year occupational history who presented with swelling of the left upper chest wall. The microscopic sample of the resected lipoma showed inflammatory cells with fat necrosis, which are features thought to be involved in the development of a lipoma following soft tissue trauma.

Patient-specific radiation dose and cancer risk for pediatric chest CT.

PURPOSE: To estimate patient-specific radiation dose and cancer risk for pediatric chest computed tomography (CT) and to evaluate factors affecting dose and risk, including patient size, patient age, and scanning parameters. MATERIALS AND METHODS: The institutional review board approved this study and waived informed consent. This study was HIPAA compliant. The study included 30 patients (0-16 years old), for whom full-body computer models were recently created from clinical CT data. A validated Monte Carlo program was used to estimate organ dose from eight chest protocols, representing clinically relevant combinations of bow tie filter, collimation, pitch, and tube potential. Organ dose was used to calculate effective dose and risk index (an index of total cancer incidence risk). The dose and risk estimates before and after normalization by volume-weighted CT dose index (CTDI(vol)) or dose-length product (DLP) were correlated with patient size and age. The effect of each scanning parameter was studied. RESULTS: Organ dose normalized by tube current-time product or CTDI(vol) decreased exponentially with increasing average chest diameter. Effective dose normalized by tube current-time product or DLP decreased exponentially with increasing chest diameter. Chest diameter was a stronger predictor of dose than weight and total scan length. Risk index normalized by tube current-time product or DLP decreased exponentially with both chest diameter and age. When normalized by DLP, effective dose and risk index were independent of collimation, pitch, and tube potential (<10% variation). CONCLUSION: The correlations of dose and risk with patient size and age can be used to estimate patient-specific dose and risk. They can further guide the design and optimization of pediatric chest CT protocols. SUPPLEMENTAL MATERIAL: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.11101900/-/DC1.

[Development of sarcoma on a ectopic extramedullary hematopoiesis secondary to hereditary spherocytosis: Case report and literature review]. / Développement d'un sarcome sur foyer ectopique hématopoïétique secondaire à une sphérocytose héréditaire : à propos d'un cas et revue de la littérature.

INTRODUCTION: Extramedullary hematopoiesis is a complication of myeloproliferative neoplasms or of chronic hemolysis. The more frequent localizations are splenic, ganglionic or paraspinal. Rarely, extramedullary hematopoiesis is associated with solid cancer. CASE REPORT: We report an original case of sarcoma located in an extramedullary hematopoiesis mass in a 72-year-old woman suffering from hereditary spherocytosis. An asymptomatic right paravertebral mass was found in 2004; the biopsy confirmed extramedullary hematopoiesis. In 2016, the patient was hospitalized due to paravertebral pain. Computed tomography showed the extension of the right paraspinal mass to pleura and mediastinum as well as vertebral bone lysis. Positron emission tomography showed an intense hypermetabolism. The biopsy showed undifferentiated sarcoma. CONCLUSION: This case report illustrates the risk of neoplastic transformation of extramedullary hematopoiesis, and the need for a biopsy when confronted to atypical aspect.

Desmoid-type fibromatosis arising in a bifid rib chest wall.

Desmoid-type fibromatosis is a rare soft tissue tumor and the chest wall is one of the common sites of its extra-abdominal occurrence. A bifid rib is one of the congenital rib abnormalities. We report a case of desmoid-type fibromatosis arising in a chest wall's bifid rib. A 42-year-old female complained of right chest pain without remarkable medical, traumatic, or familial history. Chest-computed tomography revealed a chest wall tumor located adjacent to a bifid costal cartilage of third rib. We performed chest wall resection of second and third ribs. Pathologically, the tumor was diagnosed a desmoid-type fibromatosis of the chest wall. We surmise mechanical stimulation due to the bifid rib may be related to the occurrence of the tumor. In case of desmoid-type fibromatosis without somatic gene mutation, traumatic history, wound, implants, or use of female hormonal agents, we should search also local congenital abnormality.

Risk factors and clinical features for post-transplant thoracic air-leak syndrome in adult patients receiving allogeneic haematopoietic stem cell transplantation.

Post-transplant thoracic air-leak syndrome (ALS) is rare but potentially life-threatening in patients receiving allogeneic haematopoietic stem cell transplantation (HSCT). Nevertheless, papers on thoracic ALS are limited, and this complication remains largely unknown. We reviewed 423 adult patients undergoing allogeneic HSCT from 2003 to 2014. Risk factors, clinical features and survival for thoracic ALS were collected and analysed. Thirteen out of 423 patients (3.1%) developed post-transplant thoracic ALS, including two ALS patients in the early phase. The median age at HSCT was 33 years among 13 patients with thoracic ALS. Male patients were predominant (69%). The median onset time was 253 days (range: 40-2680) after HSCT. Multivariate analysis revealed that grade III-IV acute graft-versus-host disease (GVHD) (p = 0.017), extensive chronic GVHD (cGVHD) (p = 0.019) and prior history of pulmonary invasive fungal infection (p = 0.007) were significant risk factors for thoracic ALS. In patients with cGVHD, those with thoracic ALS had a significantly worse survival than those without thoracic ALS (p = 0.04). Currently, published data analysing and exploring post-transplant thoracic ALS are limited. Our study employed a large patient cohort and determined the risk factors and clinical features for post-transplant thoracic ALS.

Exceptionally rapid response to pembrolizumab in a SMARCA4-deficient thoracic sarcoma overexpressing PD-L1: A case report.

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a new clinical entity characterized by SMARCA4 inactivation and has a dismal prognosis because of rapid growth. Effective treatments for SMARCA4-DTS have not yet been developed. Most recently, anti-programmed cell death 1 receptor (PD-1) blockade has been effective for SMARCA4-deficient lung cancer and malignant rhabdoid tumor-like tumors. Here, we describe a patient with SMARCA4-DTC who experienced a marked response to the administration of pembrolizumab. A 70-year-old female was referred to our department for treatment of SMARCA4-DTC. Positron emission tomography-computed tomography had revealed a left mediastinal tumor, peritoneal dissemination and multiple cutaneous metastases at diagnosis. Immunohistochemical analyses revealed 60% of tumor cells expressed programmed cell death ligand 1 (PD-L1). The patient was given pembrolizumab as first-line treatment. Pembrolizumab suppressed tumor growth dramatically, with only one dose leading to a partial response. Our case suggests the immunohistochemical analysis of PD-L1 expression be undertaken for patients with SMARCA4-DTS and that pembrolizumab treatment may be a promising strategy for PD-L1-positive SMARCA4-DTS.

Second primary neoplasms in survivors of Wilms' tumour--a population-based cohort study from the British Childhood Cancer Survivor Study.

A British population-based cohort study was carried out to determine the risk of second primary neoplasms in survivors of Wilms' tumour. The cohort was obtained from the British Childhood Cancer Survivor Study, a population-based cohort study of treatment toxicities in 18,044 individuals diagnosed with childhood cancer, at an age of less than 15 years, between 1940 and 1991 in Britain. There were 1,441 Wilms' tumour survivors in the cohort: 732 males (50.8%) and 709 females (49.2%). Total follow-up from 5-year survival was 27,841 person years, mean follow-up of 19.3 years per survivor. There were 81 second primary neoplasms, including 52 solid neoplasms, 3 acute myeloid leukaemias and 26 basal cell carcinomas. Thirty-five of the 39 solid neoplasms that developed in the thoracic, abdominal or pelvic region occurred within irradiated tissue. The standardised incidence ratio for all solid second primary neoplasms was 6.7 (95% CI: 5.0-8.8). Cumulative incidence for all solid second primary neoplasms by ages 30, 40 and 50 years was 2.3% (1.4-3.5%), 6.8% (4.6-9.5%) and 12.2% (7.3-18.4%). The overall risk of second primary neoplasms in survivors of Wilms' tumour treated between 1940 and 1991 was substantial, and solid second tumours tended to develop in the irradiated tissue. Continued follow-up of these survivors is important to monitor such late effects of treatment. It is also important to evaluate the risk of second primary neoplasms following more recent lower radiation dose treatment practices.

EBV-associated diffuse large B-cell lymphoma arising in the chest wall with surgical mesh implant.

In Japan, most cases of malignant lymphoma arising in the thorax are pyothorax-associated lymphoma, which develops in patients who have undergone artificial pneumothorax, used in the past as surgical therapy for pulmonary tuberculosis. Pyothorax-associated lymphoma consist mostly of diffuse large B-cell lymphoma and have a strong association with EBV. Herein is reported the case of a diffuse large B-cell lymphoma arising in the left thoracic wall after left lung resection for squamous cell carcinoma and chest wall reconstruction with polyethylene terephthalate (PET) surgical mesh. The tumor was found 20 years after the operation and was confined to the chest wall adjacent to the PET mesh. The patient did not have a clinical history of pyothorax after surgery. The lymphoma cells were of the large cell type and were positive for CD20, EBV-encoded small RNA--in situ hybridization, LMP-1 and EBNA-2. The present case demonstrates that EBV-related B-cell lymphoma can occur after surgery other than artificial pneumothorax. In the present case, long-standing chronic inflammation induced by PET mesh may have been associated with the development of lymphoma.

Oncogenic viruses in AIDS: mechanisms of disease and intrathoracic manifestations.

OBJECTIVE: The objective of this article is to introduce the reader to the thoracic manifestations of neoplasms that are related to common oncogenic viruses in HIV-infected patients. We review the pathologic basis of the infections and illustrate the imaging features of their thoracic manifestations. CONCLUSION: The intrathoracic manifestations of oncogenic viral infection in AIDS patients are protean. Understanding their epidemiologic, pathologic, and imaging features is crucial to diagnosing and managing these often-treatable conditions.

[Case of both T- and B-cell markers positive pyothorax-associated lymphoma].

A 84-year-old man with a history of pulmonary tuberculosis admitted to our hospital due to painful swelling in right front chest wall. His chest CT shows a tumor at right chest wall and right chronic empyema. Histopathologic findings from biopsy revealed both T- and B-cell markers positive non-Hodgkin's lymphoma, and we diagnosed him pyothorax-associated lymphoma. Irradiation resulted in tumor shrinkage, and a pain of tumor disappeared. T- and B-cell markers positive pyothorax-associated lymphoma is rare.

Desmoid Tumor After a Minimally Invasive Atrial Septal Defect Closure in an Adolescent.

The surgical closure of secundum atrial septal defect (ASD) is frequently performed. The minimally invasive approach, currently adopted by many centers as a way to preserve aesthetic appearance, particularly in female patients, seems to be safe. We describe the case of an adolescent who developed a desmoid tumor soon after a minimally invasive operation for surgical closure of a secundum ASD.