An incidentally discovered paraganglioma that caused sinus arrest after resection.

Paragangliomas are neural-crest-derived nonepithelial neuroendocrine tumors distributed along the parasympathetic and sympathetic nerves. To our knowledge, no studies were reported regarding sinus arrest on day 4 after paraganglioma resection. A 66-year-old female patient with a history of pulmonary vein isolation visited our department for sigmoid colon cancer treatment. Enhanced computed tomography revealed an enhanced small nodule-like lymph node near the root of the inferior mesenteric artery. The patient underwent laparoscopic colectomy with regional lymph node dissection. Postoperatively, paroxysmal atrial fibrillation attacks developed, and the patient resumed oral medication. Additionally, sinus arrest after tachycardia developed. Changing the oral medication could maintain her circulatory dynamics. Pathological examination revealed that differentiated tubular adenocarcinoma infiltrated the submucosa. Immunohistochemically, the excised nodule as a lymph node was considered a functional paraganglioma. Our case indicates that paraganglioma resection and oral medication resumption may contribute to sinus arrest. When arrhythmias affecting the circulation occur perioperatively, the presence of a catecholamine-producing tumor should be considered in addition to cardiac disease. J. Med. Invest. 70 : 503-507, August, 2023.

Intra-familial transmission of Streptococcus dysgalactiae subsp. equisimilis (SDSE): A first case report and review of the literature.

BACKGROUND: Concern about Streptococcus dysgalactiae infections has been increasing worldwide, and many cases of invasive infections have been reported. Streptococcus dysgalactiae has two main subspecies: S. dysgalactiae subsp. equisimilis (SDSE) and S. dysgalactiae subsp. dysgalactiae (SDSD). The epidemiology of invasive SDSE infections is not well understood, and the exact numbers of human SDSE infections are not known because standard laboratories are not able to identify Lancefield group C streptococci (GCS) or group G streptococci (GGS) to the species level. SDSE is often present in skin lesions, and sites of SDSE colonization and focal SDSE infections serve as the principal reservoirs for the transmission of skin and soft-tissue infections. Although the person-to-person transmission of S. pyogenes infections has been reported, the intra-familial transmission of SDSE has not been reported. CASE PRESENTATION: We report two cases of cellulitis with bacteremia in a family. A 72-year-old female with cellulitis in her right lower extremity was hospitalized, and a 104-year-old male relative was hospitalized with cellulitis 2 days later. Two strains of Streptococcus dysgalactiae subsp. equisimilis were isolated from the blood of the patients. Single nucleotide polymorphism analysis of the bacterial genomes suggested that the two strains had the same origin. This is the first case report about the intra-familial transmission of Streptococcus dysgalactiae subsp. equisimilis. CONCLUSIONS: This is the first case report about the intra-familial transmission of Streptococcus dysgalactiae subsp. equisimilis.

Morphological and genetic identification of fungal genus/species in formalin-fixed, paraffin-embedded specimens obtained from patients with histologically proven fungal infection.

BACKGROUND: Although fungi are found relatively easily by microscopic examination of pathological samples, identification of fungal genus and species in pathological samples is not easy because the morphological features of fungi are similar among genera and species. OBJECTIVES: A multiple real-time PCR was developed for identification of fungal genus/species, and morphological characterizations of fungi were analysed in pathological samples. PATIENTS/METHODS: Seventy-five formalin-fixed paraffin-embedded (FFPE) samples morphologically proven to contain any fungus were examined. A multiple real-time PCR system was developed to identify 25 fungal genus/species in pathological samples. Morphology of fungus in the specimens was re-reviewed retrospectively based on the results of real-time PCR. RESULTS: Real-time PCR identified fungal genus/species in 56 of 75 (74.6%) specimens with histologically proven fungal infection. In 53 specimens of filamentous fungi, Aspergillus spp. (22 specimens), Cladosporium (8), Scedosporium apiospermum (4), Malassezia sympodialis (1) and Candida albicans (1) were identified. Pseudohyphae of Candida were confused with filamentous fungus in a case. Morphological observation suggested differences in the presence of septated or non-septated hyphae, the filament size, and the branch angle among genus/species of filamentous fungi; however, genus/species was not able to be determined by their morphological features. In 22 specimens of yeasts, real-time PCR allowed for the identification of Candida albicans (12 specimens), Candida glabrata (2), Cladosporium (2), Scedosporium apiospermum (2), Pichia kudriavzevii (1) and Aspergillus sydowii (1). CONCLUSIONS: These data suggest that it is difficult to identify fungal genus/species by morphological features alone. Real-time PCR is useful to identify fungal genus/species in pathological samples.

A case of protein-losing gastroenteropathy caused by systemic AA amyloidosis secondary to undifferentiated carcinoma of unknown primary origin.

We report the case of a 61-year-old woman with Kartagener syndrome who presented with a 3-month history of chronic watery diarrhoea and severe hypoalbuminaemia. Histopathological examination of duodenum and large intestine biopsies showed amyloid A (AA) amyloid deposition. Scintigraphy and alpha-1 anti-trypsin clearance evaluations revealed protein-losing gastroenteropathy. Computed tomography with contrast and positron emission tomography showed a pelvic mass with multiple para-aortic lymph node enlargement. We suspected protein-losing gastroenteropathy secondary to AA amyloid produced related to malignant tumours. Following tumour resection, histopathological examination of the lesion revealed undifferentiated carcinoma of unknown origin. Postoperatively, the patient's nutritional condition improved. There has been no recurrence of protein-losing gastroenteropathy 6 months postoperatively. This is the first report of protein-losing gastroenteropathy and AA amyloidosis secondary to undifferentiated carcinoma. Early recognition and intervention could increase the likelihood of amyloidosis remission.

Calcified Amorphous Tumor-Induced Acute Cerebral Infarction.

We report the case of a 38-year-old woman who was admitted for acute cerebral infarction linked to a cardiac calcified amorphous tumor (CAT) and related mitral annular calcification (MAC). The cardiac mass was removed, and mitral valve replacement surgery was performed. Pathological examination revealed an amorphous accumulation of degenerating material within both lesions, indicating that build-up of calcium along the mitral annulus and subsequent rupture of the fibrotic tissue may be involved in the initiation and progression of CAT.

Mycotic pseudoaneurysm of a pulmonary artery branch caused by Cladosporium.

We report the case of a 53-year-old male with a history of acute myelogenous leukemia, who suffered the rupturing of a right-sided pulmonary artery pseudoaneurysm combined with pneumonia. He underwent a right-sided lower lobectomy. The resected lung tissue demonstrated a mycotic pseudoaneurysm of a pulmonary artery branch together with a filamentous fungal infection. Pseudoaneurysms are caused by the breaching of all layers of a blood vessel wall. The extravasated blood is trapped by the surrounding extravascular tissue or clots. Cladosporium was detected during a polymerase chain reaction-based analysis followed by DNA sequencing of formalin-fixed paraffin-embedded lung tissue samples. Although previous cases of pulmonary artery pseudoaneurysms caused by fungal infections, e.g., Candida or Aspergillus sp., have been reported, to the best of our knowledge this is the first case to involve cladosporiosis.

Conserved sequences of BART and BHRF regions encoding viral microRNAs in Epstein-Barr virus-associated lymphoma.

OBJECTIVE: Epstein-Barr virus (EBV) encodes at least 25 pri-microRNAs (miRNAs) in two regions of its DNA genome, BART and BHRF. B95-8, an EBV reference strain, has a deletion in the BART region. However, no information is available on the deletions or mutations in the BART and BHRF regions in clinical samples of EBV-associated lymphoma. RESULTS: Nine DNA fragments encoding miR-BARTs and two coding miR-BHRF1s were amplified by PCR from DNA samples extracted from 16 cases of EBV-associated lymphoma. All the PCR products were sequenced directly. DNA fragments encoding miR-BARTs and miR-BHRF1-1 were successfully amplified from all samples. An adenine-to-guanine mutation in the DNA fragment encoding miR-BART2-3p was detected in four of the 16 cases, and a cytosine-to-thymidine mutation in the DNA fragment encoding miR-BART11-3p was detected in one of the 16 samples. These mutations were not associated with any histological categories of lymphoma. In conclusion, mutations were rarely observed in the DNA encoding viral miRNAs in cases of lymphoma. This suggests that the DNA sequences of EBV-encoded miR-BARTs and miR-BHRF1-1 are conserved in EBV-associated lymphoma.

Post-mortem detection of bacteremia using pairs of blood culture samples.

AIM: The objective of this study was to assess the utility of examining pairs of blood culture samples obtained from separate sites (both ventricles or the aorta and vena cava) for detecting bacteremia in the post-mortem setting. METHODS: Autopsy cases in which bacterial species were isolated from blood cultures were identified over a 4-year period. Ante-mortem and post-mortem records and the findings of pathological examinations were reviewed. RESULTS: Overall, 23 bacterial species were detected in 18 autopsy cases. E. coli was the most commonly detected species (5 cases, 27.8%), followed by S. aureus and K. pneumoniae, respectively. Seven of the detected bacterial species (3 cases, 16.7%) were obligate anaerobes (Clostridium spp. and Bacteroides spp.). Among the 3 cases involving obligate anaerobes, multiple bacterial species were detected in 2 cases. Clinically, 2 of the 18 patients in which bacteria were detected were treated for significant infections (urosepsis, pneumonia, and catheter-related bloodstream infection) before their deaths. Seven cases exhibited evidence of significant infection during the post-mortem pathological examination. The differences between the aerobic and anaerobic bacteria positivity rates of the single and paired blood culture samples were significant (aerobic: p=0.013 and anaerobic: p=0.018). CONCLUSION: Analyzing pairs of blood culture samples obtained from separate sites is useful for detecting bacteremia during post-mortem examinations.

Renal abscess involving mucormycosis by immunohistochemical detection in a patient with acute lymphocytic leukemia: a case report and literature review.

A 14-year-old girl with acute lymphocytic leukemia complained of right flank pain and fever. As her fever was prolonged, she underwent renal biopsy and was diagnosed with mucormycosis. We performed right nephrectomy, and subsequent pathological examination of her tissue specimen also detected mucormycosis. Here, we report a rare case of renal mucormycotic abscess.

An autopsy case of disseminated strongyloidiasis combined with cytomegalovirus infection.

We report a rare autopsy case of disseminated strongyloidiasis combined with cytomegalovirus co-infection involving a 68-year-old man, who was originally from Okinawa Prefecture in southern Japan, where strongyloidiasis occurs sporadically among the elderly. This patient was admitted with a diagnosis of drug eruption and hypereosinophilic syndrome. He was administered steroid therapy, but suffered complications of fever, respiratory distress, and pulmonary hemorrhaging. The autopsy findings showed disseminated strongyloidiasis in the alveolar spaces and the intestine and cytomegalovirus inclusion body foci in the lungs.

Ubiquitin-positive pneumocytes and inclusion bodies are present in secondary organizing pneumonia.

OBJECTIVE: We previously reported that various types of interstitial pneumonia (IP) patterns contain intracytoplasmic eosinophilic inclusions or Mallory bodies (inclusions) that are ubiquitin positive (Ub+). In the present study, we examined tissues with the organizing pneumonia pattern (OP) to determine if they contain inclusions and Ub+ pneumocytes using lobectomized specimens. METHODS: Tissues from 34 patients with secondary OP, which appeared in 33 carcinomas and 1 pulmonary abscess, were histologically evaluated for the type of intraluminal granulation tissue and the presence of erosions and inclusions. Granulation tissues were classified into polypoid, mural and occluded subtypes according to Basset's criteria and scored. RESULTS: Inclusions were noted in 5.9% of the secondary OP cases with light microscope. Ub+ pneumocytes were detected after immunostaining and all inclusions were Ub+. Ub+ pneumocytes (inclusions) were noted in 14.7% of the secondary OP cases. OP contained pneumocyte erosions and inflammatory cell infiltration without a significant difference in the Ub+ and Ub- subgroups. Although there was no significant difference in the polypoid type of granulation tissue between the Ub+ and Ub- negative (Ub-) subgroups, the Ub+ subgroup had significant increases (p<0.05) in the mural-occluded type of granulation tissue (Ub+: 1.76±0.64, n=5 vs. Ub-: 0.72±0.87, n=29) as compared to the Ub- subgroup. CONCLUSION: Some patients with secondary OP had Ub+ inclusions as pneumocyte injury.

[Autopsy case of corticosteroid-induced invasive pulmonary aspergillosis with cavity-formation].

Invasive pulmonary aspergillosis (IPA) is an important and fatal complication in the patients with neutropenic or immunosuppressed condition. In spite of intensive treatment with anti-fungal drugs, the prognosis of the patients who have been suffered from IPA is extremely poor. This case was an 85-year-old Japanese man who was diagnosed as idiopathic thrombocytopenic purpura (ITP). He underwent high-dose corticosteroids and gamma-globulin therapy. During the hospitalization, he complained respiratory symptoms, and the abnormal shadow suggesting pulmonary infiltration and cavitation was pointed out on chest imaging. He was diagnosed as IPA because of high level of serum beta-D-glucan and positive for aspergillus antigen. Although he underwent intensive care with anti-fungal drug administration and artificial respiration, he died of respiratory distress on the 68th hospitalization. Postmortem examination disclosed severe bronchopneumonia, cavitation and pulmonary arterial thrombi in both lungs. Histological examination showed widely distributed epitheloid cell granulomata with central necrosis containing aspergillus hyphae, and vasodestructive growth of fungus with occasional organized thrombi.

Extraskeletal myxoid chondrosarcoma: a study using a quick-freezing and deep-etching method.

A case of extraskeletal myxoid chondrosarcoma (ESMC), which developed in the right thigh of a middle-aged Japanese woman, was studied using immunohistochemistry, conventional electron microscopy, and the quick-freezing and deep-etching (QF-DE) method. In addition to typical light microscopic findings of ESMC, conventional electron microscopy indicated that the tumor cells had features of chondrocytes. Immunohistochemically, the tumor cells showed a positive immunoreaction for S100 protein. A diagnosis of ESMC was made. An interesting observation was the ultrastructural features of collagen fibrils in the myxoid matrix highlighted by the QF-DE method. These collagen fibrils consisted of relatively thin collagen (20-35 nm) with pleated surface structures. The surface striation at 65 nm was obscure. We consider that such a finding of collagen fibrils identified by the QF-DE method is one of the characteristics of the myxoid matrix of ESMC, and this is useful for the differential diagnosis of myxoid soft tissue tumors.

Ruptured giant aneurysm of the left anterior descending coronary artery in Kawasaki disease.

This report describes Kawasaki disease (KD) in a rare autopsy case showing three uncommon phenomena: rapid aneurysmal development, ruptured giant aneurysm, and abscesses such as neutrophil aggregations, all contributing to the unpreventable sudden death of patients with KD. For a 5-year-old boy, KD was diagnosed on day 5 of his illness. His aneurysm was enlarged to a diameter of 18 mm on day 12, and the boy died on day 13. Aggregations of neutrophils containing myeloperoxidase and neutrophil elastase were scattered in chains over the aneurysmal wall of coronary artery, suggesting that destruction of the wall by an enzyme may have caused the rupture of the aneurysm.

Supplemental utility of nested PCR for the pathological diagnosis of disseminated trichosporonosis.

Disseminated trichosporonosis is known to be a severe opportunistic mycosis and has a high mortality rate. In autopsy cases, it is often difficult to diagnose as trichosporonosis because the causative Trichosporon species are pathologically similar to other fungi, especially the Candida species. Immunohistochemical analysis is essential for the differential diagnosis, but an antibody to Trichosporon is not available commercially. In the present study, we investigated the supplemental utility of nested polymerase chain reaction (PCR) for the pathological diagnosis of trichosporonosis from formalin-fixed and paraffin-embedded tissues. Total DNA was purified from 30 major organs in three autopsy cases, and Trichosporon DNA was specifically amplified by nested PCR using three sets of primers. Of 22 organs in which Grocott's stain was positive for fungal infection, 170- and 259-bp PCR products were detected in 20 (91%) and 12 (55%) organs, respectively. In short-term fixation (about 1 day), these bands were highly detected in ten (100%) and nine (90%) organs, whereas the detection efficiency tended to decrease after long-term fixation and decalcification. No PCR product of 412 bp was detected in any organs. These findings suggest that nested PCR from short-term-fixed tissues is useful for supportive pathological diagnosis of disseminated trichosporonosis.

A fatal case of ruptured giant coronary artery aneurysm.

A 5-year-old Japanese boy died because of a ruptured left coronary artery aneurysm (CAA). He was diagnosed as having Kawasaki disease (KD) on the 5th day from onset, with all of the principal signs. On the 7th day of illness, bilateral CAAs were already found via echocardiography, and he was treated with intravenous (IV) gamma globulin and oral ASA. However, the fever persisted and the CAA progressed rapidly. Echocardiography on the 12th illness day showed a giant (18-mm) left anterior descending (LAD) artery aneurysm. Oral propranolol and nifedipine were administered, in conjunction with warfarin/aspirin anti-coagulation therapy. On the 13th day of illness, cardiac arrest developed abruptly, and, despite cardiopulmonary resuscitation (CPR), the patient remained unresponsive and died one hour later. The final pathological diagnosis was a ruptured LAD artery aneurysm and cardiac tamponade. Microscopic investigation of the ruptured vascular wall revealed marked neutrophilic infiltration, with fewer macrophages and lymphocytes. CAA ruptures are a very rare, but fatal, complication of KD. Based on a review of previous reports on CAA ruptures, we consider it useful to distinguish aneurysms which rapidly dilate and continue to expand beyond a diameter of 10 mm with ongoing vasculitis (these CAAs can be termed "super-giant") from the more common giant CAAs limited to a diameter of 8 or 9 mm, because a decision must be made as to whether to start intensive care or to intervene surgically, in order to ensure the survival of patients with such a potentially critical complication.

Serious bradyarrhythmia that was possibly induced by lopinavir-ritonavir in 2 patients with acquired immunodeficiency syndrome.

We describe 2 patients with acquired immunodeficiency syndrome who had potentially fatal bradyarrhythmia that occurred shortly after commencement of antiretroviral therapy. Lopinavir-ritonavir was the only drug that both patients were using.