Mediastinal Origin Research Articles

Analysis of variations in the bronchovascular pattern of the lingular segment to explore the correlations between the lingular segment artery and left superior division veins.

With the development of anatomical segmentectomy, the thoracic surgeons must master the anatomical variations of the pulmonary bronchi and vessels. However, there are only a few reports showing anatomic variations of the lingular segment (LS) using three-dimensional computed tomography bronchography and angiography (3D-CTBA). Thus, the present study aimed to analyze the bronchovascular patterns of the LS and explore the correlation between the lingular segment artery (LSA) and left superior division veins (LSDV). The 3D-CTBA data of the left upper lobe (LUL) were collected from patients who had undergone lobectomy or segmentectomy at Hebei General Hospital between October 2020 and October 2022. We reviewed the clinical characteristics and variations in bronchi and pulmonary vessels and grouped them according to different classifications. Among all 540 cases of 3D-CTBA, the branching patterns of LSA included 369 (68.3%) cases with the interlobar origin, 126 (23.3%) cases with the interlobar and mediastinal origin, and 45 (8.3%) cases with the mediastinal origin. The branching pattern of LSDV could be classified into three forms: Semi-central vein type (345/540, 63.9%), Non-central vein type (76/540, 14.1%), and Central vein type (119/540, 22.0%). There were 51 cases (9.4%) with Non-central vein type, 50 cases (9.3%) with Central vein type, 268 cases (49.6%) with Semi-central vein type in the interlobar type, and 7 cases (1.3%) with Non-central vein type, 9 cases (1.7%) with Central vein type, 29 cases (5.4%) with Semi-central vein type in the mediastinal type. Moreover, the Non-central vein type, the Central vein type, and the Semi-central vein type accounted for 18 (3.3%), 60 (11.1%), and 48 (8.9%) in the interlobar and mediastinal type. Combinations of the branching patterns of the LSA and LSDV were significantly dependent (p < 0.005). The combinations of the interlobar and mediastinal type with the Central vein type, and the interlobar type and the mediastinal type with the Semi-central vein type were frequently observed. This study found the relationship between the LSA and LSDV and clarified the bifurcation patterns of the bronchovascular in the LS. Our data can be used by thoracic surgeons to perform safe and precise LS segmentectomy.

Anomalous mediastinal left lower lobe anterior segmental artery (A8) accidentally divided during left VATS upper lobectomy: a case report

: Bronchovascular anatomical variations can pose technical challenges during lobectomy and especially segmentectomy where these structures are embedded deep in the lung parenchyma. Familiarity with these anatomical variations helps avoid intraoperative injury and accidental division. Mediastinal origin of left upper lobe apicoposterior (truncus superior) and lingula arterial branches (A4+5) is common. However mediastinal origin of left lower lobe segmental pulmonary branches is very rare and has not been reported in the literature. An anomalous mediastinal origin of left lower lobe anterior segmental artery (A8) noted during a left video-assisted thoracic surgery (VATS) upper lobectomy in a 69 years old male with stage 1 lung cancer is described. The artery was mistaken for a mediastinal lingula branch (A4+5) during surgery and was divided accidentally. The injury was recognised intraoperatively and primary end to end vascular repair was done by VATS, with the patient making an uneventful recovery. Awareness of this rare anatomical variant can help prevent intraoperative vascular injury or accidental ligation during left VATS lobectomy. Injuries due to anomalous bronchovascular anatomical variations can be prevented by preoperative planning by 3-dimensional contrast enhanced computer tomography and by careful intraoperative dissection and identification of mediastinal and interlobar vessels distally into the respective segments before ligation.

Extragonadal Germ Cell Tumors of the Mediastinum and Retroperitoneum: A Surveillance, Epidemiology, and End Results-based Study.

Extragonadal germ cell tumors (EGCT) are a rare entity, most of them being located in the mediastinum and retroperitoneum. Information on these tumors is scarce, requiring carrying out large population-based studies to better understand these diseases. We aimed to determine the clinical features and prognosis of patients with EGCT of the mediastinum and retroperitoneum. Demographic and clinicopathological features of patients diagnosed with EGCT of the mediastinum and retroperitoneum from 1975 to 2016 were retrieved from the Surveillance, Epidemiology, and End Results (SEER) database. A total of 1674 patients were included, 1297 (77.5%) of mediastinal origin and 377 (22.5%) of retroperitoneum. Nonseminomatous tumors (56.3%) were slightly more frequent than seminomas (43.7%) with similar distribution between mediastinum and retroperitoneum. After a median follow-up of 137 months, the median overall survival was 263 months (95% CI, 220-296) whereas the median cause-specific survival (CSS) has still not been reached. The 10-year overall survival and CSS were 57.4% (95% CI, 55-59.7) and 63% (95% CI, 60.6-65.2) respectively. Multivariate analysis showed that older age, mediastinal location, nonseminomatous histology, and distant disease at diagnosis were independent prognostic factors correlated with a worse prognosis. Patients with mediastinal choriocarcinoma and embryonal carcinoma have the worst prognosis, both with a median CSS of only 12 months. Despite a decreasing incidence observed in recent decades, EGCT continues to represent a challenge for oncologists. The prognosis of choriocarcinoma and embryonal carcinoma of the mediastinum remains poor and treatment strategies need to be improved urgently.

Gastric volvulus associated with anomalous mediastinal origin of the diaphragm.

Pediatrics InternationalVolume 64, Issue 1 e15097 Clinical Notes Gastric volvulus associated with anomalous mediastinal origin of the diaphragm Go Miyano, Corresponding Author Go Miyano go@juntendo.ac.jp orcid.org/0000-0003-0076-3748 Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, Japan Correspondence: Go Miyano, MD, Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. Email: go@juntendo.ac.jpSearch for more papers by this authorHiroshi Murakami, Hiroshi Murakami Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorHiroki Nakamura, Hiroki Nakamura Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorGeoffrey J. Lane, Geoffrey J. Lane Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorAtsuyuki Yamataka, Atsuyuki Yamataka Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this author Go Miyano, Corresponding Author Go Miyano go@juntendo.ac.jp orcid.org/0000-0003-0076-3748 Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, Japan Correspondence: Go Miyano, MD, Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. Email: go@juntendo.ac.jpSearch for more papers by this authorHiroshi Murakami, Hiroshi Murakami Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorHiroki Nakamura, Hiroki Nakamura Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorGeoffrey J. Lane, Geoffrey J. Lane Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this authorAtsuyuki Yamataka, Atsuyuki Yamataka Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Tokyo, JapanSearch for more papers by this author First published: 12 March 2022 https://doi.org/10.1111/ped.15097Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume64, Issue1January/December 2022e15097 RelatedInformation

Clinical and Pathological Characteristics of Hyaline-Vascular Type Unicentric Castleman Disease: A 20-Year Retrospective Analysis.

The first case of hyaline vascular type of unicentric Castleman disease (HV-UCD) was reported more than six decades ago. Since patients with HV-UCD are often asymptomatic and this condition is generally discovered incidentally on imaging tests, most of the previous reports were of mediastinal origin detected by chest radiography. In recent years, improved access to imaging modalities has provided new insights in the diagnosis of this condition. In this study, we reviewed the detailed clinical and pathological findings of 38 HV-UCD cases (20 males and 18 females, mean age: 42.8 years). The most common site involved was the abdominal cavity (34.2%), followed by mediastinum (23.7%) and retroperitoneum (15.8%). In the abdominal cavity, mesenteric origin was the most common. The mean size of masses was 4.8 cm. Pathologically, thick hyalinized collagen fibers surrounding large blood vessels and calcification were observed (81.6% and 23.7%, respectively). Multinucleated giant cells resembling Warthin–Finkeldey cell were also observed in occasional cases (23.7%). This is a unique paper that summarizes detailed clinical and pathological findings of a large series of a rare disease. The clinical information presented in this paper is more plausible than previous views and is useful for accurate diagnosis and understanding of the disease.

A desmoplastic fibroblastoma that developed in the anterior mediastinum: a case report

BackgroundDesmoplastic fibroblastoma (also known as collagenous fibroma) is a benign, slowly growing soft-tissue tumor. Most desmoplastic fibroblastomas develop in the limbs, neck, or trunk. A mediastinal origin is quite rare.Case presentationA 32-year-old Asian female was referred to us for the diagnosis and treatment of an anterior mediastinal tumor. The tumor was 80 mm in the largest diameter and was located on the pericardium. No invasion was evident. She underwent resection of the tumor via video-assisted thoracoscopic resection. The tumor was totally encapsulated, and its pedicle was on the pericardium. The resected specimen was very rigid, making it difficult to remove from the intercostal space. Histologically, the tumor was composed of a paucicellular dense collagenous tissue. Mitosis was rarely observed, and cellular atypia was not evident, suggesting that the tumor was benign. We diagnosed the tumor as a desmoplastic fibroblastoma by morphology and immunohistochemistry.ConclusionsDesmoplastic fibroblastoma of the mediastinum is an extremely rare disease. Preoperative diagnosis is difficult. Early surgical resection is suitable for diagnosis and treatment planning.

Two cases (a cat and a dog) illustrating computed tomographic features distinguishing an accessory lung lobe mass from a caudal mediastinal origin

AbstractA seven‐year‐old female Chinchilla cat and a 13‐year‐old male Cocker Spaniel were presented to Dick White Referrals for respiratory difficulties and cough, respectively. Based on radiographic and CT findings, including the midline location of the lesion and its mass effect on mediastinal structures, a caudodorsal mediastinal mass was considered more likely than a pulmonary origin in both cases. However, further investigations in the form of thoracic ultrasound and ultrasound‐guided fine needle aspirates in the first case and histopathology in the second, revealed the presence of an accessory lobe pulmonary carcinoma in both cases. Retrospective review of these cases allowed identification of CT features that can be used to distinguish mediastinal and accessory lung lobe masses in future cases, including absence of part or all of the accessory lobe and its lobar bronchus, presence of gas in the lesion and the shape of the lesion mimicking a lung lobe.

Analysis of anatomical variations of the lingular artery of the left upper lobe using 3D computed tomography angiography and bronchography.

BackgroundThoracic surgeons must be familiar with the anatomy of the pulmonary artery during segmentectomy and segmentectomy. But pulmonary arteries have numerous variations and aberrant branching patterns. The purpose of the present study was to analyze the anatomical variations and frequencies of the lingular artery of the left upper lobe (LUL) using 3D computed tomography angiography and bronchography (3D-CTAB).MethodsWe retrospectively studied 166 patients having undergone lobectomy or segmentectomy from January to December 2020 at Fujian Medical University Cancer Hospital’s Department of Thoracic Surgery. All patients underwent 3D reconstruction using 3D-CTAB before surgery.ResultsThe lingular segment was supplied by 1 artery in 45.18% of cases, 2 arteries in 46.39% of cases, and 3 arteries in 8.43% of cases. The branching patterns of the lingular artery included 119 (71.68%) cases with interlobar origin, 35 (21.08%) cases with interlobar and mediastinal origin, and 13 (7.83%) cases with mediastinal origin. The interlobar lingular artery include superior lingular artery (A4) and inferior lingular artery (A5). The interlobar lingular artery type was A4a, A4b, A5 in 7.23% of cases; A4 and A4b+5 in 3.01% of cases; and A4b and A4a+5 in 4.82% of cases. The mediastinal lingular artery was divided into the following 5 types: ‘A4’, ‘A4b’, ‘A4b+5’, ‘A4b+5a’, and ‘A4+5’. The most common type was A4 (12.05%, 20/166) in 166 patients. The interlobar lingular artery had the following 5 patterns of variation: ‘A4+5’, ‘A4, A5’, ‘A4a, A4b, A5’, ‘A4a, A4b+5’, and ‘A4b, A4a+5’. The single interlobar lingular artery (A4+5) was the most common type in 38.55% of cases. In 24.10% of cases, A5 came from A8 or A8+9. Besides In 8.43% of cases, the origin of A5 was close to A8 or A8+9.ConclusionsWe identified the left various lingular artery branching patterns with 3D-CTAB in patients and defined the frequency of anatomic variations. 3D-CTAB is useful for finding these variations.

Primary mediastinal chondrosarcomas: do they really exist?

Chondrosarcomas primarily originating in the mediastinal compartment are vanishingly rare. Contrary to other unusual tumors of the mediastinum, chondrosarcomas have been described in either anterior or posterior mediastinum. The histopathological spectrum that has been described in these mediastinal tumors is similar to that described in soft tissues. In our current practice, diagnostic imaging plays an important role before any of these neoplasms is rendered as of mediastinal origin. It is also important to highlight that even though a tumor may be presenting as a mediastinal tumor, such information in essence does not constitute that the tumor is not in fact associated with another structure within the thoracic cavity, thus the importance of strict radiological correlation. Furthermore, molecular characterization of tumors that in the past were coded under the spectrum of chondrosarcomas, are now believed to represent different tumoral conditions, which raises important issues in tumor classification. Therefore, in this review, we will outline a more specific criteria regarding the diagnosis of chondrosarcomas, as wells as the essentials in the histopathological assessment of these tumors. In addition, we will discuss the current knowledge of these entities as well as their differential diagnosis, which inevitably will depend on the anatomic distribution of the tumor—anterior or posterior compartment.

Fluorescence In Situ Hybridization for the X and Y Chromosome Centromeres Helps Differentiate Between Gestational and Nongestational Choriocarcinoma in Clinically Ambiguous Cases.

Gestational choriocarcinoma usually presents during the reproductive years, typically within 1 year of pregnancy, although presentation remote from pregnancy also occurs and may cause confusion with other tumors, including choriocarcinoma of germ cell origin and somatic carcinomas with choriocarcinomatous differentiation. It is important to separate these tumors for treatment and prognostic reasons. To assess the utility of fluorescence in situ hybridization for the X and Y chromosome centromeres in determining the gestational origin of clinically ambiguous extrauterine choriocarcinomas in women. A review of female patients with extrauterine choriocarcinomas who had no evidence of prior gestational trophoblastic disease was performed. Samples were analyzed by fluorescence in situ hybridization for the X and Y chromosome centromeres using standard methodologies. Five cases met the criteria, all of which displayed trophoblastic cells and necrosis. Three cases (60%) had Y chromosomes by fluorescence in situ hybridization, which confirmed gestational origin. Although the 2 cases without a Y chromosome would ordinarily require molecular genotyping for paternal genetic material to establish gestational origin, in one of these cases a subsequent recurrence of yolk sac tumor allowed confirmation of its mediastinal origin. Fluorescence in situ hybridization for detection of the X and Y chromosome centromeres is an effective screening test for gestational choriocarcinoma. It provided a definitive diagnosis of metastatic gestational choriocarcinoma in 3 of 5 potential cases that lacked a clinical history of gestational trophoblastic disease. An additional benefit is that more laboratories have the capability to perform fluorescence in situ hybridization than can perform molecular genotyping for definitive diagnosis.

Clinical manifestation of pediatric mediastinal tumors, a single center experience.

Medical decisions should be well-planned to improve prognosis and reduce complications of mediastinal tumors. In this study, we analyzed the clinical presentations of pediatric mediastinal tumors and their correlation with long-term clinical outcome.Forty patients under 18 years of age diagnosed with mediastinal tumors at China Medical University Children's Hospital between 2001 and 2016 were enrolled. The patients’ sex, age of onset, initial clinical symptoms, and treatment outcomes were analyzed.75% of the patients with mediastinal tumors in this study were men, and the median age of onset was 13 years old (age range: 0–17 years). The overall mortality rate was 40%. The most common tumors were lymphoma (47.5%), followed by germ cell tumors (12.5%), neuroblastoma (12.5%), and thymoma (7.5%). Neuroblastoma was more prevalent in girls younger than 5 years old. The initial presentations of these patients included breathing difficulty (65%), productive cough (47.5%), pleural effusion (54.5%), superior vena cava (SVC) syndrome (35%), neck mass (35%), airway compression (32.5%), fever (30%), chest pain (27.5%), and pericardial effusion (25%). Lymphomas were more likely to be accompanied by neck mass (52.6% vs19.0%, P = .04) and SVC syndrome (52.6% vs 19.0%, P = .026), yet also had a better 1-year-survival rate (68.4% vs 52.4%, P = .02).Overall, lymphoma should be suspected when children present with neck mass and SVC syndrome. Neuroblastoma with a posterior mediastinal origin should be suspected among children younger than 5 years old. Tumor-related airway obstruction, pleural effusion, and pericardial effusion were leading cause of cardiopulmonary instability during sedation for invasive procedures, which should be managed cautiously.

PUB041 Potentially Overlooked Branches of the Left Pulmonary Artery

During pulmonary resection, we sometimes encounter ‘pitfall branches’ of the pulmonary artery (PA) which could cause serious vessel injury. Current computed tomography (CT) instrument and computer processing technology can identify the PA along the peripheral bronchus, thereby revealing the true rates of the different PA branching patterns. Knowledge of these branching patterns allows safe and definitive surgery. For 186 cases of left lung resection performed at our institution from January 2006 to September 2011, two general thoracic surgeons and one radiologist independently reviewed CT images and examined the branches of pulmonary artery arising from pars mediastinalis. Branching patterns of lingular artery included17 cases with mediastinal origin (9.2%), 50 cases with interlobar and mediastinal origins (26.9%), and 119 cases with interlobar origin (63.9%). The two types of lingular artery of mediastinal origin, which were potentially overlooked during surgery, were observed in 36.1% of cases. This was a higher rate than previously reported. Moreover, mediastinal basal PA (A4+5+8+10 abnormal branching) was seen in 1 case. Potentially overlooked branches of the left PA arising from pars mediastinalis are much higher than we expected. It is crucial to understand the branching pattern of the PA by preoperative CT assessment.

Potentially overlooked branches of the left pulmonary artery.

During pulmonary resection, we sometimes encounter "pitfall branches" of the pulmonary artery, which could cause serious vessel injury. Current computed tomography and computer-processing technology can identify the pulmonary artery along the peripheral bronchus, thereby revealing the true rates of different pulmonary artery branching patterns. Knowledge of these branching patterns allows safe and definitive surgery. In 186 cases of left lung resection performed at our institution from January 2006 to September 2011, two general thoracic surgeons and one radiologist independently reviewed computed tomography images and examined the branches of the pulmonary artery arising from the pars mediastinalis. Branching patterns of the lingular artery included 17 (9.2%) cases with mediastinal origin, 50 (26.9%) with interlobar and mediastinal origin, and 119 (63.9%) with interlobar origin. The 2 types of lingular artery of mediastinal origin, which were potentially overlooked during surgery, were observed in 36.1% of cases. This was a higher rate than previously reported. Moreover, a mediastinal basal pulmonary artery (A(5+8+10) abnormal branching) was seen in one case. Potentially overlooked branches of the left pulmonary artery arising from the pars mediastinalis are much more frequent than we expected. It is crucial to understand the branching pattern of the pulmonary artery by preoperative computed tomography assessment.

Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case.

Follicular dendritic cell sarcoma is a rare malignant neoplasm originating from follicular dendritic cells, and most of them develop in lymph nodes of the head and neck. One third of follicular dendritic cell sarcomas occur in the extranodal sites such as the tonsils, mesentery, and retroperitoneal organs, but those of mediastinal origin are rare. Here, we present the case of a 16-year-old female with a large follicular dendritic cell sarcoma of posterior mediastinal origin. The tumor was found by a chest X-ray mass examination at her high school, and she had no subjective symptoms or significant past medical history. The tumor was diagnosed as a follicular dendritic cell sarcoma by computed tomography-guided needle biopsy. Although the tumor compressed the mediastinal organs and showed moderate uptake in 18-fluorodeoxyglucose positron emission tomography imaging, it was completely resected through posterolateral incision. Histological examination revealed that spindle-shaped tumor cells formed fascicular or storiform pattern with cellular pleomorphism. By immunohistochemical examination, the tumor cells were found to be positive for CD21 and follicular dendritic cell antigen. Two years after surgery, the patient remains alive with no signs of tumor recurrence.

Abstract B09: Differential effect of microenvironment on tumor phenotype using an immature T cell lymphoma mouse model

Abstract Human T cell lymphomas of an immature phenotype with mediastinal origin are classified as T cell lymphoblastic lymphomas (T-LBL) and comprise 85-90% of all lymphoblastic lymphomas. Treatment of T-LBL can be challenging because of the metastasizing nature of the disease, which commonly localizes to the bone marrow and can be found in other organs such as liver and spleen. We have developed a mouse T-LBL model by establishing a cell line from a spontaneous mediastinal tumor found in a T cell receptor (TCR) transgenic mouse strain. The resulting T-LBL like cell line, JMR4, has a known TCR (2C), known alloreactivity (H-2Ld), and known stimulating self-peptide (SIYR). JMR4 has an immature T cell phenotype, which includes a mixture of CD4+/CD8+, CD4lo/CD8+, CD4+/CD8lo and CD4+ cells. Other T cell markers, such as CD5, CD24 and CD69 are expressed at various levels depending on the phenotypic subset. The 2C TCR, which can be detected using the 1B2 idiotypic mAb, has been shown to be functional using flow cytometric analysis following TCR stimulation using peptide loaded thymic stromal feeder cells. In addition, JMR4 can be phenotypically altered (CD4/CD8/CD5/CD69 expression levels) differentially in vitro using the bone marrow stromal cell line OP9 and OP9DL, suggesting that it has pluripotency. Finally, when JMR4 is injected into sub-lethally irradiated mice, it develops into a multi-organ lymphoma. Interestingly, the phenotype of the JMR4 cells, based on CD4 and CD8 expression levels, is differentially regulated depending on organ type and MHC of the host, making JMR4 an ideal model to study the complex interactions of a tumor with its microenvironment. Understanding the mechanisms involved in tumor microenvironment (TME) induced changes to tumor cells should be important in understanding metastatic tumor progression and in designing new treatment regimens. Citation Format: Philip J. Lucas, Ronald E. Gress. Differential effect of microenvironment on tumor phenotype using an immature T cell lymphoma mouse model. [abstract]. In: Proceedings of the Fourth AACR International Conference on Frontiers in Basic Cancer Research; 2015 Oct 23-26; Philadelphia, PA. Philadelphia (PA): AACR; Cancer Res 2016;76(3 Suppl):Abstract nr B09.

Acute lymphoblastic leukemia and lymphoma in the context of constitutional mismatch repair deficiency syndrome

Constitutional mismatch repair deficiency (CMMRD) syndrome is one of the rare diseases associated with a high risk of cancer. Causative mutations are found in DNA mismatch repair genes PMS2, MSH6, MSH2 or MLH1 that are well known in the context of Lynch syndrome. CMMRD follows an autosomal recessive inheritance trait and is characterized by childhood brain tumors and hematological malignancies as well as gastrointestinal cancer in the second and third decades of life. There is a high risk of multiple cancers, occurring synchronously and metachronously. In general, the prognosis is poor. About one third of CMMRD patients develop hematological malignancies as primary (sometimes the only) malignancy or as secondary neoplasm. T-cell non-Hodgkin lymphomas, mainly of mediastinal origin, are the most frequent hematological malignancies. Besides malignant diseases, non-neoplastic features are frequently observed, e.g. café-au-lait spots sometimes resembling neurofibromatosis type I, hypopigmented skin lesions, numerous adenomatous polyps, multiple pilomatricomas, or impaired immunoglobulin class switch recombination. Within the present review, we summarize previously published CMMRD patients with at least one hematological malignancy, provide an overview of steps necessary to substantiate the diagnosis of CMMRD, and refer to the recent most relevant literature.

Giant Thoracocervical Lymphangioma with Multivisceral Involvement: A Different Concept

ABSTRACT Background Lymphangiomas are benign lymphatic malformations frequently seen in head and neck region. Most reported cases are cervical lesions with mediastinal extension.1,2 Most are conceived as due to failure of fetal lymph sacs. Synchronous or metachronus lymphangiomatosis with cystic hygroma of the neck is also reported.3 Case details Massive multicompartmental mediastinal lymphangioma with cervical extension and concomitant visceral involvement is presented. It is proposed as a hamartomatous tumor rather than malunion of fetal jugular lymphatic sacs.2 Solid intrathoracic component with cystic neck extension supports mediastinal origin. Cell culture lines at 4 months were positive for endothelial cell lines positive for factor VIII antigen. Conclusion Giant thoracocervical lymphangioma is more likely to be a tumor rather than simple fetal failure of lymphatic sac fusion. Surgical excision is curative. How to cite this article Prasad GR, Nori M, Naseeruddin MD Rao JVS, Quadri SS. Giant Thoracocervical Lymphangioma with Multivisceral Involvement: A Different Concept. Int J Phonosurg Laryngol 2014;4(2):63-66.

Rekonstruktive Chirurgie bei Patienten mit Herzunterstützungssytemen (Ventricular Assist Device, VAD) - eine interdisziplinäre Herausforderung

Ventricular assist devices (VAD) are implanted with a growing frequency in patients with end-stage heart failure. In spite of technical and therapeutic advances, there is still a high incidence of device infections and healing disturbances with a considerable mortality. Reconstructive plastic surgery is able to provide help in erradicating infections and covering defects. 11 patients with device infections and soft tissue defects were treated in our institution. All cases were treated with local myocutaneous or muscle flaps. Three patients had postoperative haematomas which had to be revised surgically. One patient had a persistent fistula of mediastinal origin but without clinical symptoms of infection. One patient exhibited an enterocutaneous fistula after defect coverage. Patients with VADs have a high possibility of perioperative complications. Therefore a close interdisciplinary approach with plastic and cardiovascular surgeons is absolutely essential.

Primary Sarcoma of the Mediastinum: A Report of 16 Cases Referred to the British Columbia Cancer Agency

Sarcoma arising in the mediastinum is a rare entity. This study evaluates treatment and survival in a cohort of patients with primary mediastinal sarcoma. Between 1990 and 2006, 16 patients were referred to the British Columbia Cancer Agency with histologically confirmed sarcoma of mediastinal origin. Outcomes examined were disease-free survival (DFS) and overall survival (OS). There were nine male and seven female patients. The median age at diagnosis was 56 years (range 21-70 years). Thirteen (81%) patients had localized disease, and three (19%) patients had distant metastasis at diagnosis. Surgical resection was performed in 8 of 13 patients with localized disease. At a median follow-up of 18 months, 12 patients have died of disease, three were alive with disease, and one was alive with no evidence of disease. In the entire cohort, median DFS was 12 months (range 0-107 months), and median OS was 18 months (range 1-193 months). Patients who underwent surgery experienced improved DFS (p = 0.054) and OS (p = 0.034). Eastern Cooperative Oncology Group performance status 0 to 1 was associated with improved DFS (p = 0.038) and OS (p = 0.007). The histologic subtype with the longest survival was well-differentiated liposarcoma. Age, gender, tumor location, T and N stage, tumor size, location, and grade were not associated with significant survival differences. Surgical resection was associated with more favorable survival in patients with mediastinal sarcoma. However, the high rates of progression and mortality underscore the need for more effective adjuvant treatments.

Comparative proteome analysis of gonadal and extragonadal germ cell tumors

14530 Background: Germ cell tumors (GCTs) are the most common malignancy in young Caucasian males aged 20–40. Even though highly effective treatment strategies have been developed for the majority of patients (pts) with GCT, long term survival rate in pts with primary mediastinal non-seminomas nowadays remains below 50%. The molecular mechanism underlying the increased resistance to treatment remains elusive. Therefore, a systematic approach of global proteome analysis was used as a screening tool to compare GCT cell lines of gonadal and extragonadal origin. Methods: Comparative protein expression analysis of two GCT cell lines, NTERA2 (of gonadal origin) and NCCIT (of mediastinal origin) was performed using 2-dimensional gelelectrophoresis. Differentially expressed proteins were analyzed using matrix-assisted laser desorption/ionization - time of flight mass spectrometry (MALDI-TOF). Identification of proteins was performed using peptide mass-fingerprinting and NCBI/SwissProt database search. Results: On average, 761 spots on each gel were detected. Of these, 66 proteins were found to be differentially expressed in an at least in 2-fold manner. Expression of 47 proteins was found to be higher in NCCIT than in NTERA2, 19 proteins showed lower expression in NCCIT than in NTERA2. All 66 spots were analyzed, and 35 (51%) proteins were positively identified by peptid mass fingerprinting and database search. These proteins could be grouped into different categories: 9 are involved in metabolism, 5 each in protein folding and DNA-binding/transcription, 4 in cytoskeleton/cell movement, 3 in cell signaling/differentiation, and 1 each in ion transport and membrane/vesicle trafficking. Of 7 proteins, the function is currently unknown. Conclusions: This analysis demonstrates that proteomic tools are valuable in studying similarities and differences between gonadal and primary extragonadal GCTs. Further characterization and functional analysis of selected proteins is ongoing, with special emphasis on factors potentially involved in treatment resistance and sensitivity. Furthermore, the acquired proteome map of untreated cells will help to identify cellular changes occuring under different experimental conditions in vitro like induction of differentiation and response to cytotoxic treatment. No significant financial relationships to disclose.