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BMC Surg 2022 Jun 11;22(1):226. Epub 2022 Jun 11.

Department of Thoracic Surgery, University Medical Center Mainz, Johannes Gutenberg University Mainz, Mainz, Germany.

Background: Ectopic Cushing syndrome (ECS) is a sporadic condition. Even uncommon is an ECS that derives from a carcinoid tumor of the thymus. These tumors may pose several diagnostic and therapeutic conundrums. Read More

BMJ Case Rep 2022 Jun 7;15(6). Epub 2022 Jun 7.

Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

Functional mediastinal paragangliomas (PGs) are rare, catecholamine-secreting tumours. Surgical resection is the preferred treatment, but it can be complicated by catecholamine-related symptoms, involvement of cardiac structures and/or tumour supply from major blood vessels. We report a case of a man in his 30s with a subcarinal functional PG complicated by all three factors. Read More

J Cardiothorac Surg 2022 Jun 6;17(1):143. Epub 2022 Jun 6.

Department of Chest Surgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima, 960-1295, Japan.

Posterior mediastinal paraganglioma (PM-PGL) is a rare disease that is difficult to diagnose. If PM-PGL is misdiagnosed preoperatively, surgeons may encounter severe tachycardia and hypertension and easy bleeding from the tumor during the operation. Therefore, it is essential to include PGL as a differential diagnosis for mediastinal tumors. Read More

Mod Pathol 2022 Jun 3. Epub 2022 Jun 3.

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 350W 11th Street, Room 4086, Indianapolis, IN, 46202, USA.

Neoplastic cartilage is a common component of teratomas in type II germ cell tumors. Although IDH1/2 mutations have been well-described in somatic cartilaginous tumors, ranging from benign enchondromas to highly aggressive dedifferentiated chondrosarcomas, the presence of IDH1/2 mutations in cartilaginous neoplasms arising from germ cell tumors has not been previously investigated. To better understand the relationship between these tumors and their bone/soft tissue counterpart, we studied the IDH1/2 mutational status of 20 cases of primary mediastinal mixed germ cell tumors with areas of readily identifiable cartilaginous differentiation. Read More

Arkh Patol 2022 ;84(3):52-59

B.V. Petrovsky Russian Research Center of Surgery, Moscow, Russia.

A case of a mature anterior mediastinal teratoma with a predominance of the pancreatic component (80% of the tumor) and signs of nesidioblastosis in a 46-year-old woman is presented. During histological examination, all components of the pancreatic parenchyma were determined - acini with interlobular and intralobular ducts, endocrine cells and islets of Langerhans of various sizes and shapes, as well as islet-duct complexes scattered in the acinar tissue. In addition to pancreatic tissue, cartilage, bronchial respiratory epithelium, small intestine tissue, hair follicles, and sebaceous glands were found in the neoplasm. Read More

Am J Case Rep 2022 May 28;23:e936138. Epub 2022 May 28.

Department of Surgery, King Salman Armed Forces Hospital in North-Western Region, Tabuk, Saudi Arabia.

BACKGROUND Ganglioneuromas are differentiated tumors originating from the neural crest. Although their occurrence is rare, they usually involve the posterior mediastinum and retroperitoneum. However, they rarely occur in the adrenal gland. Read More

Kyobu Geka 2022 May;75(5):357-361

Department of Cardiovascular Surgery, Yokohama Sakae Kyosai Hospital, Yokohama, Japan.

We report a case of a 53-year-old man with superior vena cava( SVC) syndrome due to large cell neuroendocrine carcinoma (LCNEC) in the mediastinum. His chief complaint was general fatigue. On physical examination, both jugular veins were distended and his face and bilateral upper limbs were swollen. Read More

Indian J Pathol Microbiol 2022 Apr-Jun;65(2):369-373

Department of Pathology, Anyang Tumor Hospital, The Fourth Affiliated Hospital of Henan University of Science and Technology, Anyang, Henan, China.

Purpose: To investigate the clinicopathological features of mature teratoma with malignant transformation.

Methods: Retrospectively analysis of 1179 cases mature teratoma was done from August 1999 to December 2019 in Institution. 14 cases of mature teratoma with malignant transformation were discussed mainly for the pathological characteristics and clinical manifestations. Read More

J Cardiothorac Surg 2022 Apr 5;17(1):67. Epub 2022 Apr 5.

Department of Thoracic Surgery, SUNY Upstate Medial University, 750 East Adams Street, Syracuse, NY, 13210, USA.

Background: Mediastinal teratomas are rare tumors that frequently occur in the anterior mediastinum. The majority of these tumors are benign and slow growing. Due to their low malignant potential, the treatment for these tumors is surgical resection. Read More

BMJ Case Rep 2022 Apr 1;15(4). Epub 2022 Apr 1.

Pathology and Laboratory Medicine, Medical Sciences Campus, University of Puerto Rico, San Juan, Puerto Rico.

Choriocarcinoma is a highly malignant tumour emerging from the syncytiotrophoblast divided into gestational and non-gestational presentations. Primary choriocarcinoma of the mediastinum is rare. Metastases to the brain often occur; however, brainstem involvement has not been reported for non-gestational choriocarcinoma. Read More

J Med Case Rep 2022 Mar 31;16(1):128. Epub 2022 Mar 31.

Institute of Pathology Anatomy, Faculty of Medicine, University of Prishtina, Prishtina, 10000, Kosovo.

Background: Primitive neuroectodermal tumors are extremely rare and highly aggressive malignant small round cell tumors that arise from the primitive nerve cells of the nervous system or outside it. These tumors share similar histology, immunohistologic characteristics, and cytogenetics with Ewing's sarcoma. Peripheral primitive neuroectodermal tumors of the chest wall are rare malignant tumors seen in children and young adults. Read More

Mediastinum 2022 25;6. Epub 2022 Mar 25.

Interdisciplinary Group for Translational Research and Clinical Trials, Urological Cancers (GIRT-Uro), Candiolo Cancer Institute, FPO-IRCCS, Candiolo, Turin, Italy.

Objective: The aim of this review is to analyze feasibility and toxicities of high-dose chemotherapy (HDCT) in comparison to standard dose chemotherapy (SDCT) in patients affected by mediastinal germ cell tumors (MGCTs), discussing factors that may affect therapeutic choices, such as: management of residual disease, early response predictors for chemotherapeutic efficacy and determinants of chemotherapeutic resistance. In this review, we discuss the main clinical experiences with HDCT and SDCT in germ cell tumor (GCT) patients specifically in those affected by MGCT.

Background: MGCTs represent a very small subset characterized by a poor prognosis, despite improvements in their clinical management and in understanding their biology. Read More

Mediastinum 2022 25;6. Epub 2022 Mar 25.

Service of medical oncology, Oncology Institute of Southern Switzerland, EOC, Bellinzona, Switzerland.

Objective: In this review, we summarize the current state of the art of primary mediastinal germ cell tumours (PMGCTs) and we highlight challenges and future research directions for this disease.

Background: PMGCTs account for 1-3% of all germ cell malignancies and for 15% of adult anterior mediastinal cancers. In 60-70% of cases PMGCTs are represented by nonseminomatous germ cell tumours (GCTs), and in 30-40% of cases by seminomas. Read More

Mediastinum 2022 25;6. Epub 2022 Mar 25.

Department of Pathology, Vita-Salute San Raffaele University, IRCCS San Raffaele Scientific Institute, Milan, Italy.

Objective: Mediastinal extragonadal germ-cell tumors (MEGCTs) are rare neoplasms with a multifaceted clinical behavior. This paper is devoted to review their main characteristics, including histological patterns and different factors of aggressiveness in MEGCTs. Proper understanding of the latter can help to better stratify patients' prognoses and improve clinical management. Read More

Magn Reson Med Sci 2022 Mar 17. Epub 2022 Mar 17.

Department of Radiology, Faculty of Medicine, Saga University.

The anterior mediastinum is the most common location of mediastinal tumors, and thymic epithelial tumors are the most common mediastinal tumors. It is important to differentiate thymic epithelial tumors from malignant lymphomas and malignant germ cell tumors because of the different treatment strategies. Dynamic contrast-enhanced MRI and diffusion-weighted imaging can provide additional information on the differential diagnosis. Read More

Zentralbl Chir 2022 Feb 2;147(1):99-120. Epub 2022 Mar 2.

Chirurgische Klinik Campus Charité Mitte, Charité Universitätsmedizin, Berlin, Deutschland.

If mediastinal tumours cause symptoms these are related to their anatomical localization or a paraneoplastic syndrome. The differential diagnosis is based on the clinical situation with finding the lesion, and, furthermore, taking into account the age and sex of the patient, and the mediastinal compartment where the lesion is located. Cross-sectional radiographic diagnostic is essential for defining the therapeutic strategy. Read More

J Neuroendocrinol 2022 Mar 14;34(3):e13100. Epub 2022 Feb 14.

Institute of Pathology, University of Bern, Bern, Switzerland.

In recent years the WHO classification of neuroendocrine neoplasms (NEN) has evolved. Nomenclature as well as thresholds for grading have changed leading to potential confusion and lack of comparability of tumour reports. Therefore, the European Neuroendocrine Tumour Society (ENETS) has set-up an interdisciplinary working group to develop templates for a pathology data set for standardised reporting of NEN. Read More

Int J Colorectal Dis 2022 Mar 12;37(3):541-551. Epub 2022 Feb 12.

School of Medicine and Dentistry, Menzies Health Institute Queensland, Griffith University, Gold Coast, QLD, Australia.

Purpose: The purpose of the study is to assess the global risk of extracolonic secondary primary cancers (SPCs) in patients with colorectal cancer (CRC).

Methods: Studies of SPC in patients with CRC were included if they reported the standardised incidence ratio (SIR) for extracolonic SPCs in patients with CRC compared with the general population. Pooled summary estimates were calculated using a random-effects model. Read More

Diagn Pathol 2022 Feb 12;17(1):29. Epub 2022 Feb 12.

Department of Pathology and Translational Research, Gifu University Graduate School of Medicine, Yanagido 1-1, Gifu, 501-1194, Japan.

Background: SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4-deficient thoracic sarcoma (SMARCA4-DTS) is a rare disease that has recently been described as an entity. It is characterized by an aggressive clinical course and specific genetic alterations. As an immunohistological feature, the tumors are deficient in SMARCA4 and SMARCA2 and express sex-determining region Y (SRY)-box 2 (SOX2). Read More

Cancer Epidemiol 2022 04 7;77:102116. Epub 2022 Feb 7.

Division of General Thoracic Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan.

Background: Neuroendocrine neoplasms (NENs) are rare and can originate from any body part. However, there are only few epidemiological studies, especially on lung and mediastinal NENs. This study investigated the epidemiological trends and differences between lung and mediastinal NENs in Japan. Read More

Mediastinum 2021 25;5:19. Epub 2021 Jun 25.

Department of Thoracic Surgery, Aichi Cancer Center Hospital, Nagoya, Japan.

This report presents an unusual case of life-threatening massive bleeding in the pulmonary trunk adjacent to the right ventricular outflow tract during resection of a large primary mediastinal nonseminomatous germ cell tumor (PMNSGCT) in the absence of cardiovascular surgeons. The patient was a 21-year-old male whose large mediastinal tumor was diagnosed as an extragonadal PMNSGCT, which was a mixture of a yolk sac tumor and an immature teratoma. Generally, chemotherapy causes extensive peripheral tumor necrosis of PMNSGCTs, thus enabling their complete resection. Read More

Am J Case Rep 2022 Jan 25;23:e932616. Epub 2022 Jan 25.

Department of Pathology, One Brooklyn Health System, Brooklyn, NY, USA.

BACKGROUND Mediastinal masses can originate from anatomical structures normally located in the mediastinum, or from structures that travel through the mediastinum during embryogenesis. Initial presenting symptoms usually vary from shortness of breath, cough, chest pain, and superior vena cava syndrome to nonspecific constitutional symptoms (eg, fever, weight loss, fatigue). However, the initial presentation of a mediastinal mass with acute pericarditis has not been reported in the literature as far as we know. Read More

Front Endocrinol (Lausanne) 2021 5;12:790975. Epub 2022 Jan 5.

Department of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu, China.

Ectopic adrenocorticotrophic hormone (ACTH) syndrome is not common, which is more unusual when caused by paraganglioma. We herein present a 40-year-old Chinese male who was diagnosed with ACTH-dependent Cushing's syndrome. However, the localization of the ACTH source was troublesome due to the inconsistent results of the high-dose dexamethasone suppression test and the desmopressin stimulation test. Read More

Diagn Pathol 2022 Jan 14;17(1). Epub 2022 Jan 14.

Charles University and University Hospital Pilsen, Pilsen, Czech Republic.

Background: Yolk sac tumor (YST) is a germ cell tumor. It is primarily located in the gonads but can also occur extragonadally (extragonadal yolk sac tumor - EGYST), most commonly in the pelvis, retroperitoneum or mediastinum. Only a few YSTs of the urachus have been described. Read More

Rom J Morphol Embryol 2021 Apr-Jun;62(2):587-592

Department of Radiology and Medical Imaging, Department of Extreme Conditions Medicine, University of Medicine and Pharmacy of Craiova, Romania;

Fetus in fetu (FIF) is a rare entity, with a difficult preoperative diagnosis, frequently being an intraoperative surprise. In recent literature, theories pertaining to its development either assimilate the tumor with a monochorionic twin that halted its evolution, or with a highly differentiated mature teratoma. We present the case of a 27-year-old patient, with a cystic tumor in the anterior mediastinum, which intraoperatively proved to be a rare case of intrathoracic FIF. Read More

Interact Cardiovasc Thorac Surg 2022 01;34(1):159-161

Department of Cardiothoracic Surgery, Wujin Hospital Affiliated with Jiangsu University, Changzhou, China.

A female patient with a right-sided encapsulated pleural effusion was misdiagnosed preoperatively as having an encapsulated empyema. However, a giant mass in the anterior mediastinum was found via thoracoscopy, and a mature teratoma was detected based on the pathological result. Herein we report this case and provide lessons for cardiothoracic surgeons. Read More

J Int Med Res 2022 Jan;50(1):3000605211069202

Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China.

Germ cell tumors (GCTs) often occur in male testes and female ovaries. Extragonadal GCTs account for approximately 2% to 5% of all GCTs and mainly occur in the mediastinum, retroperitoneum, and pineal gland. In this study, we reported a rare case of gastric adenocarcinoma with GCT components. Read More

In Vivo 2022 Jan-Feb;36(1):49-56

Department of Pediatrics, Division of Pediatric Hematology and Oncology, Penn State College of Medicine, Hershey, PA, U.S.A.

Background/aim: Neuroblastoma is clinically and molecularly heterogeneous, with poor outcomes despite multimodal treatment strategies. The primary tumor site is an independent predictor of survival; adrenal tumors have the worst outcomes, while posterior mediastinum tumors carry a more favorable prognosis.

Materials And Methods: To elucidate the role of the primary tumor microenvironment in mediating survival outcomes, we developed a mouse model for the study of extra-adrenal neuroblastoma by injecting luciferase-tagged cells into either the subpleural space of the posterior chest or the adrenal gland. Read More

Int J Surg Case Rep 2022 Jan 14;90:106683. Epub 2021 Dec 14.

Department Cardiovascular Disease, Ain shams University Hospitals, Cairo, Egypt.

Introduction: Teratomas are rare neoplasms made up of mature embryonic germ cell layers. Extra-gonadal teratomas are rare and primarily in the mediastinum. Mature cystic teratomas (MCT) are slow-growing benign tumors accounting for a small number of mediastinal germ cell tumors. Read More

Eur J Radiol 2022 Jan 13;146:110107. Epub 2021 Dec 13.

Division of Molecular Imaging and Theranostics, Department of Nuclear Medicine, University Hospital Salzburg, Paracelsus Medical University, 5020 Salzburg, Austria. Electronic address:

Purpose: The 2-fluorodeoxyglucose positron emission tomography/computed tomography (2-[F]FDG PET/CT) is used for the evaluation of response to immunotherapy in malignant melanoma. Here, we evaluated the prognostic value of various metabolic parameters in baseline and different time points after therapy.

Methods: In this retrospective study, 51 metastatic melanoma patients, who had received immunotherapy, were included. Read More