An elderly case of paraneoplastic anti-NMDA receptor encephalitis associated with large cell neuroendocrine carcinoma of the lung.

BACKGROUND: Recent studies have suggested that N-methyl-D-aspartate (NMDA) receptors are involved in the cell proliferation in several tumors. However, there have been no reports demonstrating the expression of NR1 subunit of the NMDA receptor in large cell neuroendocrine carcinoma (LCNEC). CASE PRESENTATION: Here, we report the first elderly case of paraneoplastic anti-NMDA receptor encephalitis associated with LCNEC of the lung with NR1 expression. Of note, NR1 subunit expression in the tumor cells of the present case was confirmed by immunohistochemistry (IHC). Radiation therapy and immunotherapies, such as corticosteroids and intravenous immunoglobulin (IVIG), shrank the tumors and improved neurological symptoms in the present case. Additionally, we also confirmed the expression of NR1 in the tumor cells obtained from three other cases with LCNEC of the lung at our hospital by IHC. CONCLUSION: Our IHC results indicate that LCNEC generally expresses NR1 subunit and NMDA receptor may be involved in the tumor development and growth.

Adenoid cystic carcinoma of the orbit without lacrimal gland involvement and with intracranial extension: A case report.

We present a 76-year-old female with a 6-year history of decreased vision in the right eye and right-sided facial neuralgia. She had a T1 isointense and T2 isointense enhancing lesion in the right orbit and the middle cranial fossa on MRI examination. Granulomatous disease or meningioma was suspected, however, after removal, the tumor was identified by pathology as adenoid cystic carcinoma (ACC). The tumor has no radiological and clinical lacrimal grand involvement. ACC shows a slow and indolent growth pattern but is associated with poor long-term outcomes, mainly due to perineural invasion, local control failure, and distant metastasis. This case highlights the importance of a pathologic diagnosis and early intervention in similar presentations.

Brain metastasis from cutaneous squamous cell carcinoma coexistent with extramammary Paget's disease: A case report.

We report a case of cutaneous squamous cell carcinoma (CSCC), initially coexisting with invasive extramammary Paget's disease (EMPD), in the scrotum of an 84-year-old man. The patient initially had a rash and pruritus before presenting with a pedunculated scrotal mass surrounded by widespread erythema. He underwent total gastrectomy for adenocarcinoma 1 year previously and had been receiving TS-1 (Tegafur/Gimeracil/Oteracil pottasium) orally. Histopathologically, the tumor consisted of invasive SCC, with invasive EMPD in the erythematous region. From the clinical presentation and histopathological findings, we assumed that CSCC developed in the background of the EMPD. The CSCC metastasized to several inguinal lymph nodes and to the brain in the following years. While the histogenesis of each of the tumors remains to be elucidated, the fact that the CSCC rather than the EMPD metastasized to a distant site in this patient is to be noted for future treatment considerations.

Atypical cause of intractable diarrhea in a hemodialysis patient, masked by Clostridium difficile-associated diarrhea and ischemic colitis: a case report.

BACKGROUND: Patients with end-stage kidney disease (ESKD) most commonly complain of gastrointestinal symptoms, such as diarrhea. Diarrhea negatively affects patient quality of life and has miscellaneous etiologies, such as Clostridium difficile-associated diarrhea (CDAD) and ischemic colitis. However, it is sometimes extremely difficult to determine the true etiology given the comorbidities and complications the patients have. A rare cause of diarrhea is ulcerative colitis (UC), which commonly affects the rectum and proximal colon in a continuous fashion. UC with rectal sparing or segmental distribution, although atypical, sometimes leads to misdiagnosis. Herein, we present a case of UC in a patient on hemodialysis with intractable diarrhea; we initially considered that the diarrhea was caused by CDAD and ischemic colitis. CASE PRESENTATION: A 69-year-old man with a history of hypertension, bilateral thalamic hemorrhage, and decreased kidney function was admitted to our hospital because of congestive heart failure. Volume control was impossible due to renal dysfunction and he was started on hemodialysis. Thereafter, he received various antibiotics for bacterial infections. Simultaneously, he experienced continuous watery, and sometimes bloody, diarrhea, which was diagnosed as CDAD owing to a positive stool test for Clostridium difficile toxins. Antibiotic treatment for CDAD did not result in symptom relief. Subsequently, we performed colon biopsy via colonoscopy, and the pathology showed virtually no inflammation with rectal sparing and segmental distributions. These findings favored the presence of ischemic colitis due to arteriosclerosis and ESKD rather than infections. He died of cardiac arrest before the diarrhea was alleviated. Finally, UC was revealed on autopsy as the main cause of the uncontrollable diarrhea. CONCLUSIONS: Patients with ESKD have a greater risk of developing CDAD and ischemic colitis, which have clinical features that sometimes overlap with those of UC, as in the present case. This case emphasizes the importance of correctly diagnosing the etiology of intractable diarrhea and the fact that other diarrhea etiologies can obscure the existence of inflammatory bowel disease, which should be considered and treated properly when patients on hemodialysis present with intractable diarrhea.

Severe pleuritis and pericarditis associated with very-late-onset systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem disorder, which occurs mostly in young women. However, late-onset SLE does exist and sometimes presents with an atypical, diversified course. We describe an 85-year-old woman who was admitted to our hospital for lower extremity edema and hand grip weakness. Chest computed tomography scan 4 days after admission demonstrated rapid accumulation of pleural and pericardial effusions, which did not exist on admission. She was diagnosed with pleuritis and pericarditis associated with very-late-onset SLE. Methylprednisolone pulse therapy resulted in a drastic improvement in serositis. Our case exemplifies the fact that patients with late-onset SLE sometimes follow an atypical course, which makes the clinical diagnosis difficult.

Intrapelvic Bulky Tumor as an Unusual Presentation of Erdheim-Chester Disease.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis, which is known to affect various organs; however, there have been no reports of its intrapelvic involvement. We herein describe the case of 69-year-old man who died of a rapidly-growing intrapelvic tumor, which was finally diagnosed as ECD at autopsy. Immunohistochemically, the tumor cells were positive for CD68 and BRAF V600E, and negative for CD1a. Since BRAF V600E has recently been reported to be specific to ECD, it can be a useful biomarker for diagnosis, especially in atypical cases.

[Intrapulmonary hematoma masquerading as metastatic carcinoma of lung cancer].

We herein report a case of intrapulmonary hematoma after lobectomy for lung cancer. A 51-year-old male underwent right upper lobectomy for lung cancer in a thoracoscopic procedure. Three months after the operation, chest computed tomography showed a 16-mm well-circumscribed nodule in the right lower lobe suggesting metastatic carcinoma for lung cancer. We performed the partial resection of the right lower lobe. The histological examination confirmed the diagnosis of an intrapulmonary hematoma. When pulmonary shadow appears after lung operations, intrapulmonary hematoma is one of a choice of differential diagnosis. Magnetic resonance imaging may be useful in establishing the diagnosis when intrapulmonary hematoma is suspicious clinically.

[Malignant peripheral nerve sheath tumor causing hemothorax; report of a case].

We herein report a case of malignant peripheral nerve sheath tumor that caused a hemothorax. A 39-year-old male was found to have an abnormal shadow on a chest X-ray. Chest computed tomography showed a 60-mm tumor in the right posterior mediastinum. Fourteen months earlier, he underwent drainage of a hemothorax with a medaistinal tumor but refused to undergo further examinations. We performed a tumor resection combined with the right lower lobectomy and the partial resection of the pericardium because of invasion. The histological examination confirmed the diagnosis of a malignant peripheral nerve sheath tumor. Postoperative radiotherapy was performed. The patient has been well without recurrence for 9 months.

[Pulmonary mucosal associated lymphoid tissue( MALT) lymphoma with 7-year natural history; computed tomography( CT) scan findings and pathological correlations].

A 58-year-old man was found to have an abnormal shadow on chest computed tomography (CT). The size and density of the nodule increased gradually over 7 years. Partial lung resection was performed by a thoracoscopic procedure. The pathological examination and gene analysis revealed that the tumor was primary pulmonary B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma). The CT findings showed that the level of substantial shadow with air bronchograms gradually increased in the center of the mass shadow, while a radial filamentous and ground glass shadow increased in the periphery. The pathological findings showed a cellular lymphocytic infiltrate that had expanded without destroying the existing blood vessels and bronchi in the center area of the tumor, while had expanded in the interstitial area along with vessel bundles in the border area. The CT findings were consistent with the pathological findings.

A thymic cyst in the middle mediastinum: report of a case.

BACKGROUND: Thymic cysts are usually located in the neck or anterior mediastinal compartment. Thymic cysts arising in the middle mediastinum are extremely rare, with only 2 previously reported cases. We herein describe the third case of a thymic cyst in the middle mediastinum. CASE: A 41-year-old female was referred to our department because of an incidental mediastinal mass detected on chest X-ray. Computed tomography showed a well-defined 7 cm mass located in the retroinnominate vein area. Magnetic resonance imaging showed a cystic lesion without any soft tissue density in the circumference. The border of the tumor was clear, without any invasion and continuity with the surrounding tissue. We performed thoracoscopic surgery. After aspiration of the serous contents in an end-pouch, the tumor was removed from the thoracic cavity. A pathological examination showed a fibrotic cyst wall containing the thymic cyst, which diagnosed it as thymic cyst. CONCLUSION: Thymic cysts in the middle mediastinum are extremely rare. A surgical resection provided the histological diagnosis of a thymic cyst in the present case.

Myelosuppression induced by concurrent chemoradiotherapy as a prognostic factor for patients with locally advanced non-small cell lung cancer.

The aim of the present study was to assess whether myelosuppression during concurrent chemoradiotherapy is a prognostic factor for patients with locally advanced non­small cell lung cancer (NSCLC). We retrospectively analyzed 86 patients with NSCLC who received concurrent platinum-based chemoradiotherapy. Patients were classified into two groups (grades 0-2 and 3-4) according to the most severe neutropenia, anemia or thrombocytopenia observed during concurrent chemoradiotherapy, and survival time and progression-free survival (PFS) time were analyzed. Univariate analysis revealed that overall survival time was significantly longer in patients with grade 0-2 anemia than in those with grade 3-4 anemia (p=0.02). Survival time did not differ significantly on the basis of the severity of neutropenia or thrombocytopenia. Although pre-treatment white blood cell count was a further prognostic factor in univariate analysis, multivariate analysis revealed that the only independent prognostic factor for overall survival time was anemia. Disease stage was an independent prognostic factor for PFS (p=0.04), whereas neutropenia, anemia and thrombocytopenia were not. In conclusion, the severity of anemia during concurrent chemoradiotherapy may be a useful prognostic factor in patients with locally advanced NSCLC.

Mucosa associated lymphoid tissue (MALT) lymphoma of the thymus with trisomy 18.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of the thymus is rare and little is known about its karyotype abnormality. MALT lymphoma in general shows a good prognosis, but some reports suggest that the presence of trisomy 18 predicts recurrence. Here, we report a patient with MALT lymphoma of the thymus and the left parotid gland accompanied by Sjogren's syndrome. The karyotype analysis revealed that this is the first case of thymic MALT lymphoma with trisomy 18, which we believe is worth reporting. We also review cases with thymic MALT lymphoma previously reported in the literature.

A case of a duodenal gastrointestinal stromal tumor diagnosed with the aid of diffusion-weighted magnetic resonance imaging.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of gastrointestinal tract, and bleeding is among the primary symptoms. If the tumor is located in the small intestine, diagnosis might be difficult because of its inaccessibility by endoscope. This report presents a case of duodenal GIST diagnosed with the aid of diffusion-weighted (DW) magnetic resonance imaging (MRI). A 66-year-old male was admitted to the hospital because of shortness of breath and melena. The patient demonstrated marked anemia. Esophagogastroduodenoscopy and colonoscopy revealed no involvement of the mucosa of the gastrointestinal tract in regard to the observed bleeding. Thereafter, computed tomography was done and showed a tumorous lesion 4.2 cm in diameter located around the ligament of Treitz. The area showed significant high intensity by DW-MRI, strongly suggesting malignant character. Examination with a long endoscope demonstrated a large submucosal tumor with wide ulceration located at the third portion of the duodenum. The biopsy sample contained spindle-shaped cells that were positive for CD34 and c-kit on histochemistry, and the tumor was diagnosed as GIST. The patient underwent surgery and survived for 16 months without any recurrence.