A case of diaphragmatic hemangioma completely resected by partial diaphragmatic resection.

Diaphragmatic hemangiomas are rare tumors and the preferred resection range in surgical procedures is considered on a case-by-case basis. We report a case of diaphragmatic hemangioma that was completely resected by partial diaphragmatic resection. An 81-year-old man was referred for the examination of right diaphragmatic mass. Computed tomography revealed two contrast-enhanced nodules (diameter: 17 and 10 mm, respectively) on the right diaphragm. The nodules were completely resected by partial resection of the diaphragm via video-assisted thoracic surgery using an ultrasonic coagulation and incision device. Resection was performed leaving part of the muscular layer of the diaphragm. Histopathology confirmed the nodule to be hemangioma originating from the diaphragm and no hemangiomatous lesions were noted in the normal connective tissue in the resected stump. Partial diaphragmatic resection is a less invasive treatment method and may be a useful surgical procedure for diaphragmatic hemangioma.

Intra-abdominal bleeding caused by amyloid transthyretin amyloidosis in the gastrointestinal tract: a case report.

Diagnosis of gastrointestinal (GI) amyloidosis is often very difficult because of its nonspecific symptoms. However, a few reports have indicated that serious symptoms such as fatal GI bleeding and obstruction or perforation sometimes lead to a diagnosis of GI amyloidosis. A 79-year-old man was transported to our emergency department with a 1-week history of worsening abdominal pain. Abdominal contrast-enhanced computed tomography showed extravasation from part of the transverse colon wall and moderate ascites. Because intra-abdominal bleeding was suspected, the patient urgently underwent partial resection of the transverse colon, which was the source of the bleeding. Postoperative pathological examination of the tissue specimens led to a diagnosis of amyloid transthyretin amyloidosis. This is the first reported case in which intra-abdominal bleeding led to a diagnosis of GI amyloidosis. We should consider the possibility of GI amyloidosis when intraperitoneal bleeding is observed in elderly patients.

Endobronchial Mucosa-associated Lymphoid Tissue Lymphoma: A Report of Two Cases and a Review of the Literature.

Primary endobronchial mucosa-associated lymphoid tissue lymphoma (EML) is rare. We reviewed 20 cases of EML, including ours and case reports. We found that the location of tumor in 70% of these cases was limited to the trachea and main bronchus, and the form of tumor in 61% of these cases was several nodular protrusions. If a patient exhibits these characteristics, adequate specimen collection on bronchoscopy is important. Because the prognosis for patients with EML is good, tumors on the trachea and main bronchus should be treated, while those on the peripheral airway can be watched carefully.

Complete response to second-line chemotherapy with sunitinib of a gastrointestinal stromal tumor: A case report.

Gastrointestinal stromal tumors (GISTs) are a type of sarcoma, and the most common mesenchymal tumor of the gastrointestinal tract. Systemic chemotherapy is recommended for unresectable or metastatic GISTs. Imatinib is an oral multitargeted receptor tyrosine kinase inhibitor that is effective as adjuvant chemotherapy for primary high-risk cases, and as palliative chemotherapy for unresectable or metastatic cases. For imatinib-resistant cases, second-line chemotherapy with sunitinib is recommended due to significantly longer median progression-free survival and higher response rates compared with a placebo. A 54-year-old woman presented with persistent upper abdominal pain and anorexia. An upper gastrointestinal endoscopy and computed tomography revealed a submucosal tumor of the stomach with no apparent metastases. The patient underwent total radical gastrectomy, and was diagnosed histologically with high-risk GIST for recurrence, therefore, the patient received adjuvant chemotherapy with imatinib. However, multiple liver and lymph node metastases were detected, and the patient received sunitinib therapy. After four cycles of sunitinib, the liver and lymph node metastases disappeared, and a complete response (CR) was achieved. To date, there have been no cases of CR in the prospective clinical trials examining the effects of sunitinib, or in case reports worldwide. Therefore, this is a very rare case report of a patient with metastatic GISTs who achieved CR with sunitinib as second-line chemotherapy.

Pelvic recurrence of an ovarian seromucinous borderline tumor detected by vaginal cytology: A case report and review of the literature.

Seromucinous borderline tumors are typically confined to the ovaries and rarely relapse after surgery. We report the case of a woman with a seromucinous borderline tumor with peritoneal implant at the Douglas pouch, who was affected by a recurrent tumor at the vaginal stump 2 years and 6 months after the primary surgery. The recurrent lesion was detected by vaginal cytology. Histology of the recurrent lesion showed perineural infiltration, and progression to low-grade adenocarcinoma was suggested. After the second surgery, vaginal cytology showed that the tumor cells remained positive. At postoperative follow-ups of ovarian borderline tumors, an examination of the specific region where recurrence is likely to occur can contribute to the early detection of tumor relapse. Diagn. Cytopathol. 2016;44:912-916. © 2016 Wiley Periodicals, Inc.

p16/CDKN2A FISH in Differentiation of Diffuse Malignant Peritoneal Mesothelioma From Mesothelial Hyperplasia and Epithelial Ovarian Cancer.

OBJECTIVES: It can be difficult to differentiate diffuse malignant peritoneal mesothelioma (DMPM) from reactive mesothelial hyperplasia (RMH) or peritoneal dissemination of gynecologic malignancies, such as epithelial ovarian cancer (EOC), which cause a large amount of ascites. Detection of the homozygous deletion of p16/CDKN2A (p16) by fluorescence in situ hybridization (FISH) is an effective adjunct in the diagnosis of malignant pleural mesothelioma. The aim of this study was to investigate the ability of the p16 FISH assay to differentiate DMPM from RMH and EOC. METHODS: p16 FISH was performed in 28 DMPMs (successful in 19), 30 RMHs, and 40 EOC cases. The cutoff values of p16 FISH were more than 10% for homozygous deletion and more than 40% for heterozygous deletion. RESULTS: According to the above criteria, nine (47.4%) of 19 successful DMPM cases were homozygous deletion positive, and three (15.8%) of 19 were heterozygous deletion positive, whereas all RMH cases were negative for the p16 deletion. In all four major histologic subtypes of EOC, neither p16 homozygous nor heterozygous deletions were detected. To differentiate DMPM from RMH or EOC, the sensitivity of the p16 homozygous deletion was 32% (9/28), and the specificity was 100%. CONCLUSIONS: Our study suggests that p16 FISH analysis is useful in differentiating DMPM from RMH and EOC when homozygous deletion is detected.

Clinical features and outcomes of 9 patients with immunodeficiency-associated lymphoproliferative disorders treated at a single institution.

Immunodeficiency-associated lymphoproliferative disorders (LPD) represent a rare life-threatening clinical entity characterized by heterogeneous histological findings that range from polymorphic to monomorphic proliferated abnormal lymphocytes. Currently, there is no standard treatment for LPD. To elucidate the clinical features and treatment outcomes of immunodeficiency-associated LPD patients with rheumatoid arthritis (RA), we retrospectively evaluated 9 cases observed over a 5-year period. The diagnoses of these patients included 5 diffuse large B-cell lymphomas, 3 LPD, and 1 mucosa-associated lymphoid tissue lymphoma. At initial diagnosis, 6 patients had advanced-stage RA, and half of these underwent total knee arthroplasty. All patients with RA received methotrexate (MTX) and low-dose prednisolone. Biologics were administered to 4 of 9 patients. After the development of immunodeficiency-associated LPD, MTX discontinuation resulted in 5 complete remissions (CR), 1 partial remission, and 3 cases of stable disease. Relapse was observed in 3 of 5 CR patients in the MTX-withdrawal remission group. Subsequently, conventional chemotherapy, rituximab, and radiation were administered to 4, 3, and 1 patient, respectively. These treatments induced a second CR. In the chemotherapy group, 1 patient developed acute myocardial infarction and another experienced ileus and pulmonary abscess. In the rituximab group, no severe complications were observed. Consequently, all patients remained disease-free during the median 23-month follow-up period. Our results indicate that, depending on the RA disease stage, performance status, and extent of treatment response, less intensive treatments than those commonly indicated for non-Hodgkin lymphoma, involving MTX discontinuation and subsequent therapy containing rituximab, might be an efficient therapeutic strategy for immunodeficiency-associated LPD.

Successful treatment of bortezomib-refractory multiple myeloma derived from monoclonal gammopathy of undetermined significance with dose-adjusted lenalidomide therapy in a patient with concomitant end-stage renal disease due to diabetic nephropathy requiring haemodialysis.

Malignancy is a fatal complication of end-stage renal disease (ESRD) requiring haemodialysis. However, the successful treatment of haematological malignancies has been rarely reported. We describe the case of a 63-year-old man who presented with IgA-type multiple myeloma (MM; Durie-Salmon stage IIIB) derived from monoclonal gammopathy of undetermined significance concomitant with ESRD due to diabetic nephropathy. First, haemodialysis was initiated before chemotherapy, and bortezomib and dexamethasone were found to be ineffective. Subsequently, 8 courses of dose-adjusted lenalidomide therapy were administered according to the degree of haematological and renal functions. The patient remained in partial remission without disease progression for 21 months. Thus, lenalidomide therapy is effective for bortezomib-refractory MM concomitant with ESRD.

Successful surgical treatment for pulmonary crystal-storing histiocytosis following the onset of gastric non-hodgkin lymphoma.

Crystal-storing histiocytosis is a rare clinical entity characterized by an increase in the number of abnormal histiocytes accompanied by accumulation of crystallized immunoglobulins. We describe the case of an 80-year-old man who presented with crystal-storing histiocytosis of the lung 13 years after receiving a diagnosis of gastric non-Hodgkin lymphoma (NHL ; clinical stage, Lugano IA). After wedge resection of the left upper lobe, the histological findings showed crystal-storing histiocytosis with CD68(+), some small to medium lymphoid cells with CD79a(+) with κ(+(weekly)) and λ(-), and some plasma cells with CD138(+), and rearrangement of the immunoglobulin heavy chain. Based on the nonrecurrent gastric NHL, small B-cell population, and failure to detect the same clone by polymerase chain reaction analysis, our case was classified as pulmonary localized crystal-storing histiocytosis without underlying lymphoproliferative or plasma cell disorder. The findings of minor B-cell populations harboring a heavy chain rearrangement with slight light-chain restriction (κ > λ) may be related to the pathogenesis of crystallogenesis and crystal-storing histiocytosis. Moreover, surgical treatment may be an effective therapeutic option for solitary crystal-storing histiocytosis.

Clinical characteristics and outcomes of 11 patients with pure red cell aplasia at a single institution over a 13-year period.

OBJECTIVE: Pure red cell aplasia (PRCA) is a rare clinical entity characterized by anemia due to severe suppression of erythroid precursors, where the other cell lineages in the bone marrow remain morphologically normal. A standard treatment has not yet been established for PRCA due to the rarity of this condition. Recently, however, the administration of either cyclosporine (CSP) or prednisolone (PSL) has been reported to be an effective treatment for PRCA. METHODS: To clarify the clinical characteristics of PRCA, 11 PRCA cases were retrospectively analyzed over a 13-year period at our institution. Since acute PRCA was found to be self-limiting, we administered the immunosuppressive treatment of CSP or PSL after providing supportive care for 4 weeks. RESULTS: The causes of PRCA were as follows: idiopathic (3), acute parvovirus infection (1), chronic parvovirus infection (3), thymic tumor (3), and end-stage renal disease with hemodialysis (1). Complete remission (CR) was achieved for 4 of the 5 patients treated with CSP, for 2 of the 3 patients with chronic parvovirus infection treated by immunoglobulin (Ig), and for all 3 patients treated with PSL. During the follow-up periods, 4 of the 11 patients relapsed. Complete remission was achieved a second time in all 4 cases by therapies that were more intensive and had longer administration periods than those provided during initial treatment. Consequently, 9 of the 11 patients were still alive (80%) after 5 years. CONCLUSION: Depending on the cause of the PRCA, treatment with CSP, PSL, or Ig was found to be effective in most PRCA cases.

Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011.

OBJECTIVE: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. METHODS: Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. RESULTS: These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. CONCLUSION: We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.

Validation of a newly proposed histopathological classification in Japanese patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis.

BACKGROUND: A new histopathological classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis was recently proposed. We evaluated the predictive value of this classification for renal outcome in Japanese patients. METHODS: We enrolled 122 patients with ANCA-associated glomerulonephritis diagnosed at several institutions in Japan between January 2000 and March 2010. Twenty patients were excluded because of observation durations of <1 year, and/or because their biopsy specimens contained <10 glomeruli. Renal biopsy specimens were categorized into four classes according to the proposed classification. We evaluated the predictive value of immunohistochemical staining for α-smooth muscle actin (SMA), Wilm's tumor 1 (WT1), CD68, and cytokeratin for end-stage renal disease (ESRD). RESULTS: The study population included 54 men and 48 women. Age, estimated glomerular filtration rate (eGFR), and proteinuria were 66.3 ± 11.3 years, 21.6 ml/min. and 1.10 g/24 h, respectively. Eighty-six patients were positive for myeloperoxidase-ANCA, five were positive for proteinase 3-ANCA, and 11 were negative for both antibodies. Median follow-up time was 41.0 months. Twenty-three patients (22.5%) developed ESRD during the follow-up period. Twelve patients died during follow up; 7/12 patients developed ESRD before death, and 5/12 patients died without ESRD. The incidence of ESRD increased with sequential categories: focal, 2/46 (4.3%); crescentic, 9/32 (28%); mixed, 8/18 (44%); and sclerotic, 4/6 (67%). The focal class had the best renal survival and the sclerotic class had the worst renal survival (p < 0.001). Kaplan-Meier renal survival analysis was similar to that of the new classification system proposal. In the multivariate analysis, the classification system tended to be a prognostic factor for ESRD (p = 0.0686, crescentic, mixed and sclerotic vs. focal, hazard ratio (HR) [95% confidence interval, CI]; 2.99 [0.61-22.7], 5.04 [1.11-36.4] and 9.93 [1.53-85.7], respectively). α-SMA-positivity also tended to be associated with ESRD (p = 0.1074). CONCLUSION: The new histopathological classification was associated with eGFR at 1 year and tended to be associated with ESRD in our Japanese cohort with ANCA-associated glomerulonephritis. α-SMA positivity might be an additional prognostic factor for ESRD.

Successful treatment of immunodeficiency-associated EBV-negative lymphoproliferative disorders in rheumatoid arthritis by methotrexate withdrawal and prevention of its relapse by rituximab administration.

Immunodeficiency-associated lymphoproliferative disorders (LPD) in rheumatoid arthritis are a rare, aggressive, and life-threatening clinical entity. We describe a 60-year-old man who had rheumatoid arthritis that was treated with methotrexate. Eight months after the treatment, the case was diagnosed as Epstein-Barr virus-negative LPD (diffuse large B-cell lymphoma) with abdominal bulky mass and clinical stage IVB at high risk in the international prognostic index. Immediate withdrawal of methotrexate led the patient to achieve complete remission, and 8 subsequent courses of rituximab treatment for the prevention of relapse kept the patient disease-free for 29 months. Our case suggests that these treatments may be an effective, safe, and feasible strategy for immunodeficiency-associated LPD in rheumatoid arthritis.

Sarcoidosis manifesting as cardiac sarcoidosis and massive splenomegaly.

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. We report an unusual case of sarcoidosis in a woman presenting with cardiac sarcoidosis and massive splenomegaly with a familial history of cardiac sarcoidosis. Cardiac sarcoidosis was diagnosed based on electrocardiogram, echocardiogram, 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG-PET) and skin histological findings. We performed splenectomy to rule out malignant lymphoma, and histological findings confirmed sarcoidosis. After splenectomy, we initiated prednisolone therapy. After 20 months of diagnosis, she was symptom free. Echocardiography and 18F-FDG-PET may be a key diagnostic tool and prednisolone therapy may be safe, effective, and feasible for cardiac sarcoidosis.

Clinical features and treatment outcomes of isolated secondary central nervous system lymphomas in Miyazaki Prefecture.

BACKGROUND: Secondary central nervous system lymphoma (SCNSL) without extra-central nervous system (CNS) involvement is characterized by isolated secondary CNS relapse in malignant lymphoma patients. SCNSL is a rare disease, and no standard treatment has yet been established. PATIENTS AND METHODS: To elucidate the clinical characteristics and outcomes of SCNSL, we retrospectively analyzed 12 patients (median age 67 years) in Miyazaki prefecture for the last 5 years. RESULTS: The initial histological diagnoses of the patients were diffuse large B-cell lymphoma (DLBCL), mantle-cell lymphoma, and adult T-cell lymphoma in 9, 2, and 1 patient, respectively. We focused on analysis of the 9 SCNSL cases originating from DLBCL. The locations of CNS relapse were the cerebral hemisphere, basal ganglia, and cerebellum in 7, 1, and 1 patient, respectively. Three patients were treated with high-dose methotrexate (HD-MTX) therapy; 4 with whole-brain radiation therapy (WBRTX); and 1 with both HD-MTX and WBRTX. The remaining patients were treated with rituximab. Partial remission was achieved in 6 out of 9 patients (67%); the other 3 patients (33%) did not respond to therapy. Median survival of the 9 patients with CNS relapse was 253 days; 6 of the 9 patients survived for more than 6 months. As of March 2011, 2 HD-MTX group patients but none of the WBRTX group patients were alive. CONCLUSIONS: In this retrospective study, 6 of 9 patients with SCNSL originating from DLBCL survived for more than 6 months. Both HD-MTX and WBRTX had clinical benefits in the treatment of SCNSL.

Cerebral phaeohyphomycosis: case report.

Cerebral phaeohyphomycosis is a rare and frequently fatal disease. This disease is often caused by hematogenous spread of pathogens that are inoculated in the skin of the extremities after slight or minor trauma, and its mortality rate is rather high despite aggressive treatment. Our patient presented with headache and pyrexia. She was diagnosed with fungal meningitis and treated by systemic administration of voriconazole (VRCZ). However, after initial improvement, meningitis recurred. MRI of the brain showed multiple small masses in the cerebral hemisphere and she was thus referred to our Department of Neurosurgery. On admission, an examination showed that the masses were deeply located in the brain and were too small to be excised; therefore, treatment with systemic VRCZ and intrathecal amphotericin B was initially selected. However, the intracerebral masses continued to grow; therefore, they were surgically excised. Histological examination of the surgical specimens at that time identified the masses as granuloma caused by infection with Aspergillus niger. After the surgery, her general condition improved; therefore treatment with systemic and intrathecal antifungal agents were continued. However, the intracerebral masses recurred, and despite further aggressive surgical treatment and systemic and intrathecal antifungal administration, she died 43 months after the initial diagnosis. Autopsy examination showed that the cerebral lesions were phaeohyphomycotic granulomas. This paper describes the clinical presentation, histopathological results and treatment for this rare disease.

Successful treatment of non-Hodgkin's lymphoma with rituximab and dose-adjusted CHOP therapy in a patient with concomitant end-stage renal disease requiring haemodialysis.

Although malignancy is a fatal complication of end-stage renal disease (ESRD) requiring haemodialysis, successful treatment of haematological malignancies has been rarely reported. We describe the case of a 64-year-old man who presented with non-Hodgkin's lymphoma (NHL; clinical stage, IVB) concomitant with ESRD. Before chemotherapy, haemodialysis was initiated, and one course of dose-adjusted CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy followed by eight courses of rituximab therapy were administered according to the performance status and degree of organ dysfunction. Consequently, the patient was disease free for 27 months. Thus, rituximab plus CHOP combination therapy was effective for NHL concomitant with ESRD.

Intravascular large B-cell lymphoma involving mainly the uterus: report of a case using liquid-based cytology of the endometrium.

BACKGROUND: Intravascular lymphoma is a rare subtype of extranodal lymphoma. Most instances of the disease are of B-cell lineage. Diagnosis is difficult because of its nonspecific clinical signs, and many cases are diagnosed at autopsy. Uterine involvement is rare, and it is commonly manifested as genital bleeding. In this case, the chief complaint was fever, which is also very rare. CASE REPORT: A 62-year-old woman presented with fever of unknown origin. Computed tomography revealed no localized lesion except for swelling of the right internal iliac nodes. A cytologic smear of the endometrium by liquid-based cytology demonstrated malignant cells. Based on the curettage material, the lesion was diagnosed as an undifferentiated malignant tumor. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic/paraaortic lymphadenectomy revealed widely scattered lymphoma cells of B-cell lineage mainly in the vascular lumina of the uterus, right ovary and lymph nodes. CONCLUSION: The final histologic type was established on the basis of the surgical material of hysterectomy. Diagnosis was difficult because of prominent cellular atypia and rare location of the tumor. Immunocytochemical examination of liquid-based samples can lead to a correct diagnosis of malignant lymphoma, even at the stage of endometrial cytologic examination.

Primary diffuse large B-cell lymphomas of the temporoparietal dura mater and scalp without intervening skull bone invasion.

A 72-year-old man presented with an extremely rare case of primary diffuse large B-cell lymphomas (DLBCLs) of the dura and scalp existing independently without intervening cranial vault invasion. The patient presented with an indolent mass lesion at the left temporal parietal scalp. Magnetic resonance imaging and computed tomography revealed a solid homogeneously enhanced mass in the left temporoparietal scalp, and an extra-axial intracranial mass that existed just below the scalp without intervening skull invasion. The patient underwent gross total resection of these lesions via a left frontotemporoparietal craniotomy. Histological examination of the masses revealed DLBCLs. The patient received whole-brain radiation therapy, and subsequent chemotherapy with cyclophosphamide, adriamycin, vincristine, and prednisolone. He was discharged without neurological deficit. The present case of DLBCLs in the scalp and dura without intervening skull bone invasion indicates that malignant lymphoma should be considered in the differential diagnosis of scalp and dural tumors without intervening skull bone invasion.

Cervical (non-terminal) myelocystocele associated with rapidly progressive hydrocephalus and Chiari type II malformation--case report.

An infant presented with a rare cervical (non-terminal) myelocystocele as a congenital skin-covered mass located in the midline of the posterior aspect of her neck. Magnetic resonance (MR) imaging and computed tomography showed a cystic mass filled with cerebrospinal fluid in the midline of the posterior aspect of the neck, with a fibrous streak extending from the bottom of the sac to the dorsal surface of the cervical cord. Brain MR imaging also showed a dilated ventricular system and Chiari type II malformation. The patient underwent plastic repair of the lesion, which was diagnosed as myelocystocele. After the surgery, the patient experienced respiratory distress. Ultrasound tomography from the anterior fontanel revealed deterioration of hydrocephalus, so a ventriculoperitoneal shunt was inserted, and the respiratory distress improved. The present case illustrates the possibility of rapidly worsening of hydrocephalus and Chari type II malformation after surgical repair of cervical (non-terminal) myelocystocele.