Extra-axial chordoma presenting as a lung mass

Chordomas are slow-growing, malignant tumors of bone that are thought to be derived from the primitive notochord and occur almost exclusively in the axial skeleton. The so-called extra-axial chordoma has been shown to demonstrate identical features to the classic chordoma, except that it is found outside the axial skeleton. Only six cases of extra-axial chordoma have been reported in the literature to date. In this report, we present another case of extra-axial chordoma for the first time originating from the lung parenchyma. A 79-year-old man presented a 7.3-cm-sized cavitary lung mass. Pathologic examination, including immunohistochemical studies, revealed that the mass was a chordoma. We report an extra-axial chordoma for the first time presenting as a lung mass.     

Adrenal ganglioneuroma: report of a new case

Although adrenal ganglioneuroma (GN) is a rare tumor originating from the neural crest tissue of the sympathetic nervous system, detection of this tumor has increased, as imaging procedures such as ultrasonography (US) and computed tomography (CT) have become prevalent. The clinical presentation for most patients is asymptomatic, and most of those tumors are hormone silent. We describe a case of adrenal GN incidentally diagnosed in a 68-year-old female patient. Physical examination, routine laboratory studies, and hormonal tests were within normal ranges. Abdominal CT and magnetic resonance imaging showed a solid oval tumor approximately 6 × 4 cm in the left adrenal gland without remarkable signs of malignancy. Left adrenalectomy was performed for treatment purposes. Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla. Adrenal GN occurs rarely in adults and preoperative diagnosis is difficult, especially in asymptomatic cases. It needs careful evaluation and surgical treatment. According to our knowledge, this is the fifth case of adrenal GN in an adult patient from Turkey in English literature.     

The First Case of Metastatic Pancreatic Leiomyosarcoma Derived from the Urinary Bladder Diagnosed Using an Endoscopic Ultrasound-guided Fine-needle Biopsy

We herein report the first case of metastatic pancreatic leiomyosarcoma derived from the urinary bladder diagnosed by an endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) in a 65-year-old woman. The patient had undergone total cystectomy for bladder leiomyosarcoma. Four years thereafter, a nodule was observed in her left lung on chest computed tomography. Suspecting primary lung cancer, pulmonologists at our hospital recommended a thoracoscopic lung biopsy, which the patient refused. Five years post-cystectomy, fluorodeoxyglucose positron emission tomography revealed enlargement of the left lung nodule and a new mass in the pancreatic head. She was referred to our department for the pathological diagnosis of a pancreatic head mass by an EUS-FNB. The EUS-FNB yielded adequate pancreatic tissue for an immunohistochemical analysis. A diagnosis of metastatic pancreatic lesion originating from the urinary bladder was made. In atypical pancreatic tumors, the utilization of an EUS-FNB and immunohistochemical analysis can help establish an accurate diagnosis.     

Primary osteosarcoma of the ureter

Primary osteosarcoma of the ureter is an extremely rare disease. Herein, the authors report a case of extraosseous osteosarcoma arising in the ureter of a 64-year-old woman. Ureteroscopy showed a papillary mass that protruded into and almost completely occluded the lumen. The tumor was successfully removed, and pathologic analysis identified mitotically active spindle cells and focal areas that contained osteoid. The tumor cells were strongly positive for vimentin but negative for cytokeratin (CK), CK7, CK20 and leukocyte-common antigen, supporting a diagnosis of high-grade extraosseous osteosarcoma of the ureter. The patient recovered well from surgery and exhibited no evidence of local recurrence or distant metastasis 6 months after the surgery. To the best of our knowledge, this is only the third reported case of primary osteosarcoma of the ureter.     

恶性肿瘤患者腹部CT检查偶然发现的肺结节的临床意义

目的分析在恶性肿瘤患者腹部CT检查中偶然发现的肺结节及其临床意义。方法在医院PACS系统以“占位”、“结节”或“肿块”为关键词搜索,对1年内的腹部CT报告进行搜索并评估。排除之前有胸部或腹部CT检查且/或非癌患者。结果9159次腹部CT检查中有1889例报道了肺部的“占位”,“结节”或“肿块”(1889/9159,20.6%),这其中只有252名为初诊患者,在这252名患者中,43名随访了胸部CT、腹部CT或胸腹部CT,且确诊为恶性肿瘤。既往没有接受过胸部或腹部CT,而1324名(1324/1576,84.0%)既往接受过CT检查,既被排除在研究之外。在剩余的252名患者中,有43名患者(43/252,17.1%)诊断为恶性肿瘤且接受了以胸部CT(n=16),腹部CT(n=13)或胸腹部(n=14)形式的随访CT。恶性肿瘤包括:原发性肝癌(n=21,其中肝细胞肝癌19例,肝内胆管细胞癌2例),结、直肠癌(n=8),胰腺癌(n=5),胃癌(n=5),膀胱癌(n=2),子宫颈癌(n=2)。43名患者中有21名(48.9%)患者的肺结节诊断为恶性。转移的肺结节多为类圆形、边界清楚的实性结节,常位于肺外围。结论在恶性肿瘤患者腹部CT上同时观察肺部情况是有必要的。胸部CT肺结节的良恶性特征鉴别可能也适用于恶性肿瘤患者腹部CT上偶然发现的肺结节。     

Transrectal EUS-guided FNA biopsy of a presacral chordoma--report of a case and review of the literature

Chordomas are rare tumors which originate from the remnants of the notochord. These tumors are locally aggressive and have a predilection for the ends of the axial skeleton. An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis. The present case is the first report of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma. A review of the prior computer tomography （CT） scans allowed us to calculate the tumor volume doubling time （18.3 mo）. Transrectal biopsy of chordomas is controversial, however we believe that such concerns are not justified.     

Stromal tumor of the pancreas with expression of c-kit protein: report of a case

We report on a case of a stromal tumor, similar to a gastrointestinal stromal tumor, originating from the pancreas. The patient was a 54-year-old woman, who was seen at the Kofu Municipal Hospital because of an abdominal tumor. On abdominal computed tomography and splenic arteriography, the tumor was detected in the pancreatic tail. The patient underwent distal pancreatectomy with splenectomy. Macroscopically, the cut surface of the tumor showed almost completely surrounded by the normal pancreatic tissue. Microscopically, the tumor composed of spindle-shaped cells that were immunoreactive for vimentin, CD34, and c-kit protein. Therefore, the tumor was diagnosed as a stromal tumor of the pancreas. The expression of c-kit protein suggests that this pancreatic stromal tumor may originate from primitive mesenchymal cells which can be a logical candidate for the origin of gastrointestinal stromal tumors and extra-gastrointestinal stromal tumors.     

Two cases of tuberculous uveitis

Uveitis has many etiologies, but tuberculous uveitis is rare. We herein report 2 cases of uveitis due to tuberculosis infection. The first case was a 28-year-old man who was showed abnormal shadows in the chest radiographic examination performed in search of the etiology of uveitis. Computed tomography (CT) of the chest revealed hilar and mediastinal lymphadenopathy, small nodules, and consolidation, with a small cavity in the right upper lobe. An ulcerated nodule in the truncus intermedius and stenosis of the right middle lobe bronchus were found on bronchoscopy. The biopsy of the nodule in the truncus intermedius showed a small granuloma containing giant cells, consistent with mycobacterial infection. The culture of bronchial washings from the right upper lobe grew Mycobacterium tuberculosis. Diagnosis of pulmonary tuberculosis, tuberculous lymphadenitis, bronchial tuberculosis, and tuberculous uveitis was made. The patient was treated with antituberculosis drugs and his disease, including uveitis, improved. The second case was a 36-year-old man who presented with right hemiparesis, dysarthria, and visual loss of the left eye. He was diagnosed with neuro-Sweet disease causing optic neuritis and visual loss. His chest CT showed a nodule with centrilobular opacities in the left lower lobe that suggested mycobacterial infection. PCR of the bronchial washing from the left lower lobe was positive for M.tuberculosis and the diagnosis of pulmonary tuberculosis was established. Treatment with antituberculosis drugs and corticosteroids was initiated and his pulmonary lesion improved. However, bilateral tuberculous uveitis developed 15 days after initiation of the treatment. The uveitis gradually deteriorated thereafter despite continuation of antituberculosis therapy. Photocoagulation finally halted the disease progression. In both patients with uveitis presented here, chest radiographs and CT scans were important in determining the etiology of the uveitis. It is difficult to find the etiology of uveitis, and general examinations including the lungs are helpful to pinpoint tuberculosis as the etiology of uveitis. As tuberculous uveitis is sometimes asymptomatic and resistant to treatment, ophthalmological examination is recommended for patients with pulmonary tuberculosis.     

Direct external imaging of nascent cancer, tumor progression, angiogenesis, and metastasis on internal organs in the fluorescent orthotopic model

Mouse tumor models have undergone profound improvements in the fidelity of emulating human disease. Replacing ectopic s.c. implantation with organ-specific orthotopic implantation reproduces human tumor growth and metastasis. Strong fluorescent labeling with green fluorescent protein along with inexpensive video detectors, positioned externally to the mouse, allows the monitoring of details of tumor growth, angiogenesis, and metastatic spread. However, the sensitivity of external imaging is limited by light scattering in intervening tissue, most especially in skin. Opening a reversible skin-flap in the light path markedly reduces signal attenuation, increasing detection sensitivity many-fold. The observable depth of tissue is thereby greatly increased and many tumors that were previously hidden are now clearly observable. This report presents tumor images and related quantitative growth data previously impossible to obtain. Single tumor cells, expressing green fluorescent protein, were seeded on the brain image through a scalp skin-flap. Lung tumor microfoci representing a few cells are viewed through a skin-flap over the chest wall, while contralateral micrometastases were imaged through the corresponding skin-flap. Pancreatic tumors and their angiogenic microvessels were imaged by means of a peritoneal wall skin-flap. A skin-flap over the liver allowed imaging of physiologically relevant micrometastases originating in an orthotopically implanted tumor. Single tumor cells on the liver arising from intraportal injection also were detectable. Possible future technical developments are suggested by the image, through a lower-abdominal skin-flap, of an invasive prostate tumor expressing both red and green fluorescent proteins in separate colonies.     

Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome

Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.     

Malignant glomus tumor in pleural cavity

Glomus tumors, an uncommon hypervascular tumor, arise from modified smooth muscle cells of the glomus body that plays a significant role in the regulation of skin circulation. The tumors are usually located in the extremities, typically in the subungual region of the fingers. Primary glomus tumors of the chest are extremely rare, and to our knowledge, there are no cases have been described in thoracic cavity to date. We here report a case of intrathoracic glomus tumor in a 31-year-old man who presented with a persistent chest pain. Chest computed tomography scans demonstrated an irregularly shaped mass in the left thorax. Left thoracotomy was performed under the suspicious diagnosis of unexplained thorax tumor, and a tumor located in the left upper portion of thorax was founded. Complete resection of tumor along with the partial structure of chest wall was performed. Postoperative diagnosis was malignant glomus tumor.     

Epithelioid hemangioendothelioma presenting with severe myelofibrosis and a high serum hyaluronan level

Epithelioid hemangioendothelioma (EHE) is a rare tumor originating from the vascular endothelium; it has an intermediate malignant potential. EHEs affect all age groups and mostly originate from the soft tissues of the extremities, lungs, and liver. Spinal EHEs, especially those occurring in the bone marrow region, are extremely rare. We report a case of EHE with massive involvement of the liver, vertebrae, and cranial bones that caused severe myelofibrosis (MF) in a 67-yr-old-male patient. Hyaluronan deposits were diffusely observed in the tumor tissue biopsies obtained from both the liver and bone marrow. Furthermore, the serum hyaluronan level increased markedly along with rapid progression of the disease. To the best of our knowledge, this is the first report of MF occurring in an EHE; hyaluronan may have played an important role in the pathogenesis of fibrosis in this case.     

Benign thyroid teratomas manifest painful cystic and solid composite nodules: three case reports and a review of the literature

Benign thyroid teratomas are rare in adolescents and adults. We report on three cases of benign thyroid teratomas that presented as painful tumors in the neck after puberty. The tumor adjacent to the thyroid in each case showed rapid enlargement with predominant cystic lesions within several months. Ultrasonography and computed tomography revealed few findings suggesting the origin of the tumor. Cytological examination and culture of the aspirate failed to show cells originating from the thyroid or infectious findings, but revealed a small population of columnar epithelial cells or squamous epithelial cells. Chemical analysis of the aspirate showed levels of pancreatic enzymes higher than those in serum. The accumulation of cystic fluid in each case was refractory to drainage treatment or percutaneous ethanol injection therapy. The patients subsequently underwent resection of the tumor, and microscopic examination revealed various types of tissue including pancreas, adipose, cartilage, muscle, and skin, and the cystic wall was lined by gastric, intestinal, respiratory, and stratified squamous epithelium. Surgical resection was curative, and subsequent histologic examination revealed mature benign teratomas of the thyroid. The main characteristic of our cases presented the painful tumors due to the enlarged cystic formation lined by a variety of different types of epithelium, which agreed with previous cases of benign thyroid teratomas in adolescents and adults.     

Asymptomatic Left Ventricular Myxoma in a 12-Year-Old Male

Cardiac myxoma is the most common cardiac tumor in patients of all ages; the majority are encountered as single left atrial tumors. Left ventricular myxomas are exceedingly rare, having been recorded in a small number of case reports involving children worldwide. We report a case of a left ventricular myxoma with left ventricular outflow tract obstruction in a previously healthy, asymptomatic adolescent black male. Transthoracic echocardiograms revealed a single, large (2.5 × 5-cm), lobulated, mobile mass within the left ventricular cavity that oscillated into the outflow tract, thereby causing moderate obstruction during systole. Advanced images delineated the location and tissue composition of the mass, characterizing it as a myxoma. Complete surgical excision of the mass was accomplished via aortotomy. Gross examination and histology confirmed the diagnosis of myxoma.     

Pure yolk-sac tumor of the lung

Primary germ cell tumors of the chest often localize in the anterior mediastinal compartment. Such tumors originating from lungs and pleura are rare. Chest tomography revealed a mass in the middle lobe of the right lung in a 25-year-old man. A middle lobe medial segmentectomy was performed, and chemotherapy was applied postoperatively.     

Cardiac Lipoma at Unusual Location – Mimicking Atrial Myxoma

Cardiac lipomas are one of the rare primary benign neoplasms composed of mature fat cells. The tumor originates mostly in the subendocardium and subepicardium but very rarely within the myocardium. Clinically, this tumor is asymptomatic and found incidentally in the vast majority of cases. We report a 26‐year‐old female presenting with atypical chest pain. Transthoracic echocardiogram showed a mass in relation to interatrial septum suggestive of atrial myxoma, but was confirmed to be cardiac lipoma on magnetic resonance imaging. Owing to the asymptomatic character and the benign nature of the tumor, a decision for conservative management was made.     

Late recurrence of a cardiac glomus tumor

Glomus tumors, also known as paragangliomas or chemodectomas, arise from well-differentiated mesenchymal cells that are known to be benign. These tumors account for 2% of all soft tissue tumors. The first case of a cardiac glomus tumor was reported in 1924 by Masson.1 Glomus tumors located within the pericardial tissue are extremely rare. We present the case of a recurrent cardiac glomus tumor. The preoperative investigation, diagnostic problems and surgical treatment of this case will be highlighted. Moreover we wish to emphasize the importance of such an entity and to remind readers that it must be included in the differential diagnosis of a pericardial tumor.     

Diffuse esophageal leiomyomatosis with a pedunculated polyp

Although leiomyomas are the most common benign tumors of the esophagus, esophageal leiomyomatosis is a rare pathological entity, and pedunculated presentation is even rarer. A 61-year-old man was found, incidentally, to have an esophageal tumor on a survey of chest computed tomography (CT) examination for a pulmonary nodule. Endoscopy disclosed a pedunculated polyp covered by nearly normal esophageal mucosa, with surrounding annular extension of a submucosal elevation. Endoscopic ultrasonography (EUS) revealed a hypoechoic tumor, with a maximum diameter of 3cm originating from the thickened muscularis mucosa layer. The underlying muscularis propria layer was also prominently thickened. The polypoid lesion was then removed by endoscopic resection with wire-loop ligation, followed by snare electrocoagulation. The pathological diagnoses of the polyp and the surrounding submucosal lesions were both leiomyoma. Diffuse esophageal leiomyomatosis was suspected in this situation because of the characteristic pathological distribution. In this patient, the EUS findings corresponded well to the characteristic features of diffuse esophageal leiomyomatosis noted in previous reports, and this was of great help for the diagnosis, in addition to the endoscopic findings. This case report is presented with a particular focus on the problems associated with accurate diagnosis.     

Primary salivary gland-type polymorphous adenocarcinoma in the lung: A case report and literature review

Rationale:Polymorphous low-grade adenocarcinoma is a low-risk infiltrative malignant tumor of the salivary glands. However, some of these tumors are more malignant than the low-grade tumors and therefore, according to the most recent recommendation of the World Health Organization, they are renamed as polymorphous adenocarcinomas (PACs). Primary polymorphous low-grade adenocarcinomas/PACs of the lungs are rare. Herein, we report a case of primary PAC of the lung with bronchial cartilage and perineural invasion, and lymph node metastasis.Patient concerns:A 58-year-old man had developed fever half a month prior, without chills or other accompanying symptoms, and the underlying reasons were unknown. His self-measured temperature was up to 39°C, accompanied by cough and expectoration, yellow and thin sputum, and shortness of breath. The patient''s general state was normal, and respiratory sounds originating from the right lung were weak. Enhancement computed tomography revealed that the bronchial lumen of the basal segment of the lower lobe of the right lung was narrow; soft tissue density nodules were seen, with a range of approximately 2.4 cm × 1.3 cm.Diagnosis:Based on clinical information, morphological features, and immunohistochemistry results, the pathological diagnosis was primary PAC of the lungs.Intervention:Thoracoscopic resection of the middle and lower lobes of the right lung was performed, further extended dissection of the mediastinal lymph nodes was performed.Outcomes:The postoperative course was uneventful.Lessons:Primary PAC of the lung is rare and may cause misdiagnosis. When encountering a lung tumor with diverse tissue structures, uniform cell type and nerve invasion, we should consider the possibility of PAC. Morphological and immunohistochemical features can be useful for diagnosing primary PAC of the lungs.     

Constrictive pericarditis as the first sign of lung cancer

Symptoms such as cough and hemoptysis in patients with lung cancer can be the consequence of local bronchopulmonary disease, tumor growth that leads to compression of surrounding structures, distant metastases, diverse systemic effects (anorexia, asthenia, weight loss), or paraneoplastic syndromes associated with tumor production of certain hormones. Approximately 10% of patients are asymptomatic at diagnosis. We report the case of a 77-year-old man with dyspnea, pleuritic chest pain, and lower limb edema. The patient died within a few days. The cause of the clinical picture was constrictive pericarditis secondary to metastases from lung carcinoma.