Paraneoplastic Neuromyelitis Optica Spectrum Disorder: A Rare Case of Advanced Breast Cancer with Intractable Nausea and Vomiting.

BACKGROUND Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disease of the central nervous system that includes the triad of transverse myelitis, optic neuritis, and area postrema syndrome (APS), characterized by intractable nausea and vomiting. NMOSD can be part of a paraneoplastic syndrome and is associated with seropositivity to aquaporin-4 (AQP-4). We present a patient with uncontrollable nausea and vomiting who developed herpes zoster and acute myelitis and was finally diagnosed with paraneoplastic NMOSD due to breast cancer. CASE REPORT A 51-year-old woman was hospitalized due to 2 weeks of intractable nausea and vomiting. Although contrast-enhanced thoracoabdominal computed tomography (CT) on day 4 suggested breast cancer in her left breast, the etiology of her symptoms remained unknown. On day 13, she developed herpes zoster, followed by acute myelitis on day 25. Magnetic resonance imaging (MRI) showing longitudinal extensive transverse myelitis and an elevated serum AQP-4 antibody level led to the diagnosis of NMOSD. Brain MRI detected a small lesion in the dorsal medulla oblongata, which explained the preceding APS. After starting intravenous methylprednisolone pulse therapy, her nausea and vomiting rapidly subsided. Breast cancer was resected on day 63, and immunohistochemical staining revealed overexpression of AQP-4 in the tumor cells, suggesting paraneoplastic NMOSD. CONCLUSIONS This report has highlighted the presentation and diagnosis of NMOSD and supports the possibility that this can present as part of a paraneoplastic syndrome. In addition, diagnosis of NMOSD preceded by APS requires meticulous history taking and careful interpretation of MRI in the dorsal medulla oblongata.

Ravulizumab can effectively treat ischemic enteritis caused by paroxysmal nocturnal hemoglobinuria.

Ischemic colitis is a common disease with a good prognosis; however, complications can occur in the presence of a serious underlying disease. Herein, we present a case report in which characteristic findings on lower gastrointestinal endoscopy led to a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). An 82-year-old woman visited our hospital for chronic heart and renal failure. She had a history of breast cancer, myocardial infarction, and hemorrhoidal fistula and was initially hospitalized for ischemic colitis. Subsequent lower gastrointestinal endoscopy revealed extensive ulcerative lesions in the ascending, transverse, and descending colon. Histopathologically, small vessels exhibited multiple fibrin thrombus formations. Based on histopathological and endoscopic results, the presence of an underlying disease was suspected. Flow cytometric analysis showed that erythrocytes and granulocytes had 5.5 and 86.4% CD55- and CD59-negative cells, respectively. The patient was ultimately diagnosed with PNH and considered severely ill, given the ischemic colitis-induced abdominal pain and the need for red blood cell transfusions (4-6 units per month). Accordingly, the patient was administered ravulizumab. Ischemic enteritis did not relapse following ravulizumab administration, and transfusion dependence improved. If a patient with ischemic colitis presents atypical lower gastrointestinal endoscopic findings, it is important to explore the presence of an underlying disease.

Periosteal Myxoid Leiomyosarcoma Histologically Mimicking Extraskeletal Myxoid Chondrosarcoma: Report of a Case with Histopathological and Cytopathological Comparison with Extraskeletal Myxoid Chondrosarcoma.

Myxoid leiomyosarcoma (MLS) is a rare variant of leiomyosarcoma, with most cases occurring in the uterus. A case of MLS arising in the periosteal region of the tibia, mimicking extraskeletal myxoid chondrosarcoma (EMC), is described. The evaluation included histological and cytological comparison with EMC. The patient was a 77-year-old man with a palpable mass at the anterior aspect of the right lower leg. After diagnosis by cytopathology and biopsy examination, a wide resection was performed. The resulting cytological smears were composed primarily of spindle-shaped tumor cells in a myxoid and hemorrhagic background. Histologically, the tumor showed abundant myxoid matrix and tumor cells proliferating in a cord-like to reticular pattern, exhibiting a lace-like arrangement that mimicked EMC. Although immunohistochemical findings suggested leiomyosarcoma, a diagnosis of EMC eventually was excluded by the lack of a split signal when assessed for a rearrangement of NR4A3 by chromogenic in situ hybridization. Despite histological similarity to EMC, characteristic cytological findings of EMC such as epithelioid structures with a cord-like pattern and chondroblast-like lacunar structures were not observed in the smears of this patient's MLS. We propose that cytopathological examination of bone and soft tissue lesions is useful as a diagnostic tool in similar cases. A total diagnostic workup, including clinical, radiographic, cytopathological, histopathological, and molecular findings, is needed to ensure an accurate final diagnosis and to reduce diagnostic error.

[A Case of Intussusception Due to Ileal Malignant Lymphoma of AYA Generation].

The case is a 17-year-old man. He had complained of right lower abdominal pain for a week. He had no symptoms such as fever, weight loss, or night sweats. He was diagnosed with intussusception by abdominal contrast-enhanced CT and was hospitalized. The day after hospitalization, lower gastrointestinal endoscopy was performed, and a tumor 25 mm in size was found in the invagination of the ileum. Intussusception was recovered by intestinal scope insufflation, and the tumor was found to be a type 1 tumor located approximately 5 cm proximal to the Bauhin's valve. On day 17 of hospitalization, he had intussusception again at the time of surgery, and performed laparoscopic reduction before performing laparoscopy-assisted partial resection of the small intestine and appendectomy. The postoperative course was good and he was discharged on POD12(on day 29 of hospitalization). Histopathological diagnosis was diffuse large B-cell lymphoma(DLBCL), and chemotherapy was to be administered at the referral hospital. In intussusception of the adolescents and young adults(AYA)generation, such as this case outside of childhood, it is necessary to treat the patient with consideration for the presence of neoplastic lesions such as malignant lymphoma. We report our case with some literature considerations.

Glomerulopathy with distinctive fibrillar deposits but lacking glomerular deposition of type III collagen.

A 62-year-old woman with nephrotic syndrome underwent a renal biopsy. Under light microscopy, the biopsy findings included lobulation and enlargement of glomeruli, occasional thickening of glomerular capillary walls, and narrowing of the capillary lumen by swollen endothelial cells. Congo red staining was negative for amyloid. No significant intraglomerular fibrin deposition was found by phosphotungstic acid hematoxylin staining. Immunofluorescence microscopy showed no deposition of immunoglobulin G, A, or M; no κ or λ light chains; and no C3 or C1q. Electron microscopy revealed distinctive subendothelial and mesangial fibrillar deposits, mesangial cell interposition, and swelling and vacuolization of endothelial cells resulting in capillary lumen narrowing. Although some curvilinear fibrillar deposits mimicked the bundles of type III collagen fibers seen in collagenofibrotic glomerulopathy, neither glomerular deposition of type III collagen nor elevation of serum procollagen III peptide was noted. This glomerulopathy does not fulfill any known disease entities with non-amyloid non-immunoglobulin-derived organized glomerular deposits.

[Wide resection of chest wall for intramuscular lipoma in serratus anterior muscle reconstructed by autologous tissue;report of a case].

A 60-years-old man with a right lateral chest wall mass visited our hospital. There was a mass of 50×45 mm on the 5th rib. The mild atypical cells were detected by the percutaneous needle biopsy, and the tumor was resected with the chest wall of which detect was reconstructed with fascia lata and latissimus dorsi muscle cutaneous flap. Pathological diagnosis was lipoma in the serratus anterior muscle. The postoperative course was uneventful without paradoxical respiration and surgical site infection. Since intramuscular lipoma is very rare and is reported to have a risk of recurrence, careful observation is necessary.

[A case of primary effusion lymphoma with elevation of ADA activity in pleural effusion].

A 74-year-old man was referred to our hospital with complaint of dyspnea and left pleural effusion. The pleural effusion was exudative and lymphocytic with elevation of adenosine deaminase (ADA). Antitubercular agents were administered on a diagnosis of tuberculous pleuritis, but the pleural effusion did not improve. After he had been followed up with diuretic agents during about 2 years, he suffered cardiac tamponade and right pleural effusion. We diagnosed primary effusion lymphoma based on the cytology findings of the pleural effusion. The measurement of ADA activity in pleural effusions was useful for diagnosis of tuberculous pleuritis, but not only tuberuculous pleuritis but also lymphoma or other diseases can show elevation of ADA activity in pleural effusions.

[Extrarenal angiomyolipoma with spontaneous rupture: a case report].

We present a very rare case of retroperitoneal extrarenal angiomyolipoma (AML) with spontaneous rupture. A 67-year-old woman without tuberous sclerosis was admitted to our hospital complaining of sudden right flank pain. We suspected right renal AML with rupture by imaging analysis, but the diagnosis was extrarenal AML by surgery and pathological examination.

Sarcoidosis presenting as prepatellar bursitis.

A 61-year-old woman complained of a subcutaneous mass in her right knee. MR images revealed a well-defined subcutaneous mass in the prepatellar region, containing some fluid and foci of short T2. The lesion showed some marginal contrast uptake after an administration of meglumine gadopentetate (Gd-DTPA), consistent with prepatellar bursitis. The pathological specimen, however, revealed subcutaneous sarcoidosis involving the bursa.

A case of Epstein-Barr virus-associated natural killer/T-cell lymphoma presenting as dermatomyositis: extranodal relapse after 7 years in remission.

A 56-year-old Japanese man was admitted to our hospital due to a fever of unknown origin. He had had a history of extranodal natural killer (NK)/T-cell lymphoma, nasal type, and had been in complete remission for 7 years until June 2003, when he developed high fever, eyelid swelling, and muscular weakness. Serum creatine kinase levels were elevated. Histopathological examination of skin and muscle biopsy specimens revealed subcutaneous infiltration of lymphoid cells positive for CD3, CD56, and Epstein-Barr virus-encoded small nuclear RNA-1. We report this unique case of Epstein-Barr virus-associated lymphoma mimicking dermatomyositis.

Sarcoidosis breaching the fascia and mimicking a sarcoma.

A 55-year-old woman complained of a subcutaneous mass in her left buttock. MR images revealed an ill-defined soft tissue mass that crossed the fascia of the gluteus maximus muscle. Some surrounding edema was noted. The lesion showed some contrast uptake after administration of Gd-DTPA. An excisional biopsy revealed sarcoidosis involving both the muscle and subcutaneous tissue across the fascia, mimicking a sarcoma.