The multiple brain abscesses associated with congenital pulmonary arteriovenous malformations: a case report

In this report, we describe a case of multiple brain abscesses associated with diffuse congenital pulmonary arteriovenous malformations (PAVM). Although the cases of brain abscesses associated with congenital PAVM are very rare, the brain abscess could be an initial clinical manifestation in asymptomatic PAVM as in the case presented in this report. PAVM may contribute to the development of a brain abscess by allowing easy bacterial access to systemic circulation through the right-to-left pulmonary vascular shunt, bypassing the filtering effect of the pulmonary capillaries. Hence, this association should be considered in cases with brain abscesses of undetermined etiologic factors.     

105例脑动静脉畸形并出血的外科治疗

目的探讨脑动静脉畸形及合并出血后外科治疗方法的选择。方法105例病人根据脑动静脉畸形的分级、出血的部位、出血量的大小及病人的状况等选择手术、栓塞或栓塞后手术等方法治疗。结果手术组60例,全切除55例,占92%;部分切除5例,占8%。血管内治疗40例,一次全部栓塞的15例,占37%;分次全部栓塞的18例,占45%;部分栓塞的7例,占18%。血管内治疗后手术5例,全切除3例,占60%;部分切除2例,占40%。治愈91例,治愈率为86%;好转6例,占6%;死亡8例,占8%。结论手术治疗和血管内治疗是目前治疗脑动静脉畸形并出血的有效方法。     

Spindle cell/sclerosing rhabdomyosarcoma: case series from a single institution emphasizing morphology,immunohistochemistry and follow-up

Spindle cell/sclerosing rhabdomyosarcoma is a rare skeletal-muscle tumor with distinctive clinicopathologic characteristics. 10 cases (6 cases of spindle cell rhabdomyosarcoma and 4 cases of scleroisng rhabdomyosarcoma) were composed of 6 males and 4 females aging from 5 months to 57 years, with median age 33 years, most of who represented a painless solid mass. Histologically, the tumors were composed of fascicles of spindle cells or primitive round cells embed in sclerotic matrix with presence of rhabdomyoblasts in varying proportion. Immunohistochemically, the tumor cells expressed MyoD1 (10/10), Desmin (10/10), myogenin (6/10), AE1/AE3 (2/10), EMA (2/10), but were negative for SMA, caldesmon, S-100. All of the patients underwent a complete surgical resection without or with chemotherapy (2/10) or radiotherapy (1/10). During the follow-up period (1 to 24 months), 1 patient was succumbed, and 2 cases showed in situ recurrence with 1 of them adopting metastasis. Our cases further demonstrate there do present some clincopathologic relations between spindle cells rhabdomyosarcoma and sclerosing rhabdomyosarcoma, but the latter seems to have a better prognosis. Exact grading and staging contribute to predict the outcome.     

Tissue reactions induced by different embolising agents in cerebral arteriovenous malformations: a histopathological follow-up

AIMS: Comparative histopathological analysis was performed in 47 incompletely embolised and resected cerebral arteriovenous malformations (AVMs). METHODS: Thirty-three AVMs were embolised with n-butyl-cyanoacrylate (NBCA), four with iso-butyl-cyanoacrylate (IBCA), seven with polyvinyl alcohol particles (PVA), one with a fibrin mixture, one with silicon pellets, and one with microcatheter balloons. Maximum exposure time (MET) of the embolising agent (interval between embolisation and surgery) ranged from <24 hours to 80 months. All AVMs were investigated regarding angionecrosis, angiofibrosis, acute inflammation, chronic inflammation, foreign-body reactions, vascular calcification, blood admixture to embolising cast, and capillary recanalisation within the AVMs. These parameters were correlated with MET, comparing different embolising agents, age, and sex. RESULTS: A typical sequence of events depending on MET is observed in all embolised AVMs: acute inflammation with mural angionecrosis is soon replaced by prominent chronic granulomatous vasculitis, which remains stable and is detectable for a very long time, even in AVMs with a MET of more than 6 years. CONCLUSION: Capillary recanalisation is always present in incompletely embolised AVMs, detectable after 3 months of MET, irrespective of the embolising agent used. Age and sex does not influence pattern and time course of tissue lesions and recanalisation in incompletely embolised AVMs.     

Concordance of second opinion diagnosis in salivary gland cytopathology: experience from a single academic institution

Interpretation of fine needle aspiration (FNA) material from salivary gland lesions has high interobserver variability due to the heterogenous and overlapping cytological features of various lesions. For this reason, second opinion consultation may play an important role in guiding appropriate clinical management for challenging cases. We aimed to report our experience with salivary gland cytology consultation cases at our academic center. Consecutive salivary gland FNA cases received from outside institutions for second opinion consultation between 2013 and 2022 were reviewed. Cases were divided into true consults (diagnostic assistance sought) or confirming consults (reviewed by in-house cytopathologists for patients being transferred to our institution for treatment). All diagnoses were re-classified using the Milan System for Reporting Salivary Gland Cytopathology (MSRSGC). Discordance between reclassified consult diagnostic categories and preliminary diagnostic categories was recorded. Consultation resulted in a change in the diagnostic category in 15% of confirming and 18% of true consult cases. The overall distribution of diagnostic categories provided by outside pathologists was similar to consult diagnoses. Only 4 (5.4%) confirming and 3 (5.5%) true consult cases had major diagnostic discrepancies, which may impact the clinical treatment. Moderate interobserver variability is often expected with salivary gland FNA. However, in our consultation practice, we found a relatively high degree of concordance between submitting and consult diagnoses utilizing the MSRSGC.     

Early rerupture of cerebral arteriovenous malformations: beware the progressive hemispheric swelling

While early rerupture of cerebral arteriovenous malformations (AVMs) may not be as rare as previously thought, its determinants and risk factors remain unknown. Impairment of the venous drainage of AVMs is a well known risk factor for rupture and has been linked with the development of perinidal cerebral edema. We propose that a significant proportion of early AVM reruptures are the result of post-hemorrhagic venous drainage impairment, which may manifest as refractory perihematomal edema. To support this hypothesis, an illustrative case of early AVM rerupture occurring 3 weeks following intracranial hemorrhage and heralded by progressive perinidal and perihematomal edema is presented. This finding should be viewed as a marker for unstable lesions with a high risk of imminent rerupture and should thus prompt a rapid definitive treatment for the AVM.     

The academic medical center: a stressed American institution.

In recent years, relations between academic medical centers and the government have become increasingly adversarial. Although the centers and the government were formerly partners in a number of health ventures, to the mutual benefit of both, the climate is now too often confrontational. It is a confrontation in which there will be no winners, but the larger society may be the loser. A number of pressures on academic centers have helped to contribute to this situation. The federal sector and academic medical institutions must understand one another better and rediscover effective ways to preserve the special strengths of academic medicine in this country.     

Factors that impact turnaround time of surgical pathology specimens in an academic institution

Turnaround time of laboratory results is important for customer satisfaction. The College of American Pathologists' checklist requires an analytic turnaround time of 2 days or less for most routine cases and lets every hospital define what a routine specimen is. The objective of this study was to analyze which factors impact turnaround time of nonbiopsy surgical pathology specimens. We calculated the turnaround time from receipt to verification of results (adjusted for weekends and holidays) for all nonbiopsy surgical specimens during a 2-week period. Factors studied included tissue type, number of slides per case, decalcification, immunohistochemistry, consultations with other pathologists, and diagnosis. Univariate and multivariate analyses were performed. A total of 713 specimens were analyzed, 551 (77%) were verified within 2 days and 162 (23%) in 3 days or more. Lung, gastrointestinal, breast, and genitourinary specimens showed the highest percentage of cases being signed out in over 3 days. Diagnosis of malignancy (including staging of the neoplasia), consultation with other pathologists, having had a frozen section, and use of immunohistochemical stains were significantly associated with increased turnaround time in univariate analysis. Decalcification was not associated with increased turnaround time. In multivariate analysis, consultation with other pathologists, use of immunohistochemistry, diagnosis of malignancy, and the number of slides studied continued to be significantly associated with prolonged turnaround time. Our findings suggest that diagnosis of malignancy is central to significantly prolonging the turnaround time for surgical pathology specimens, thus institutions that serve cancer centers will have longer turnaround time than those that do not.     

Well-differentiated papillary mesothelioma: A 17-year single institution experience with a series of 75 cases

We present our experience with 75 cases of well-differentiated papillary mesothelioma (WDPM) that were diagnosed at our institution between 2000 and 2017. The patients included 58 females and 17 males with age ranging from 18 to 69 years (mean, 42 years). Clinically, the vast majority of WDPMs were incidental findings during laparotomy or laparoscopic surgery for a variety of benign or malignant disease. The lesion manifested as either a small solitary nodule or multiple miliary nodules on the peritoneum or serosal surfaces of internal organs. Histologically, 67 cases were consistent with a classical WDPM, of which 6 cases contained microinvasive foci and 1 case had malignant transformation. Eight cases were hybrid tumors with variable combined component of adenomatoid tumor (n = 4), multicystic mesothelioma (n = 2), and both (n = 2). By immunohistochemistry, besides calretinin, D2-40, CK5/6 and WT1, 94% (29/31) of cases also showed immunostaining for PAX8. In comparison, PAX8 staining was only present in 12% (6/50) of epithelioid malignant mesothelioma selected as control cases. Follow-up information available in 46 cases revealed no signs of tumor progression or local recurrence except for the case that showed transformation to a fully malignant mesothelioma after a period of 15 years. Our comprehensive study further expanded the clinical and histopathological spectrum of WDPM. Compared with epithelioid malignant mesothelioma, PAX8 staining is highly sensitive and specific for WDPM (P < 0.001).     

Long-term pathological follow-up of cerebral arteriovenous malformations treated by embolization with bucrylate

We examined 17 intracranial arteriovenous malformations that were resected after treatment by embolization using bucrylate (isobutyl-2-cyanoacrylate). In nine specimens removed 5 days to 16 months after embolization therapy, a series of pathologic changes was seen, including patchy mural angionecrosis (adjacent to bucrylate fragments) up to six weeks after embolization, the presence of bucrylate in vessel walls and fibromuscular intimal cushions, and the occurrence (after several months) of entirely extravascular bucrylate. Occasional parts of recanalized vascular malformations were identified. Bucrylate was present within arteriovenous malformations as late as 16 months after embolization, although the amount appeared to be diminished. These findings suggest a specific sequence of events in the interaction between bucrylate and mural components within the malformations and may explain some important complications of embolization therapy (e.g., delayed hemorrhage after embolization).     

Dose calculation of 142Pr microspheres as a potential treatment for arteriovenous malformations

Arteriovenous malformation (AVM), especially cryptic AVM, can cause highly variable cerebral neurological defects. Injection of 142Pr microspheres into arteries feeding an AVM in order to simulate radio-embolism has been proposed as a novel treatment method. To investigate optimization of radiation dose to the clinically important arterial wall area, preliminary dosimetric studies have been performed. Monte Carlo calculations were performed for simulated arteries filled with microspheres packed by random packing. Arterial radii from 0.05 mm to 3 mm and microsphere radii from 0.01 mm to 0.7 mm were used in the simulation. For constant arterial size, dose varied significantly with microspheres radius. Inter-arterial effect was also simulated using simplified geometry. For the inter-arterial sites, the dose rate was calculated between two arteries of the same size parallel to each other. The dose increased significantly for large arteries (>1 mm radius) filled with large microspheres (>0.3 mm radius). The dose increase between small arteries (<0.3 mm radius) was not as significant as that between large arteries. Overall results indicate that arterial size and microsphere size significantly affect the dose profile. This factor should be taken into account in future clinical applications.     

Phosphaturic mesenchymal tumor: a report of 6 patients treated at a single institution and comparison with reported series

Osteogenic osteomalacia (OO)–associated phosphaturic mesenchymal tumors (PMTs) might represent a single histopathologic paraneoplastic entity. These tumors are largely misunderstood, ignored, or unknown by pathologists and clinicians. To elucidate the characteristics of OO-associated PMTs, we retrospectively analyzed the clinicopathologic features of PMTs from 6 patients, with either known OO or features suggestive of PMT-mixed connective tissue variant, who were studied and managed at a single center during the period from 1993 to 2011. Histologically, the tumor showed proliferation of spindle cells with focal areas of matrix production that showed distinct calcification, ossification, and osteoid-like matrix. Two patients had no evidence of disease and normal biochemical values; the other 2 patients each had multiple surgeries for multiple recurrences. In conclusion, PMT is histologically a benign lesion, with the malignant and metastatic variant being extremely rare. Infiltration of surrounding tissue is a frequent feature that is best managed with complete surgical removal of all involved tissue, which dramatically resolves the tumor-associated osteomalacia.     

Isobutyl-2-cyanoacrylate pulmonary emboli associated with occlusive embolotherapy of cerebral arteriovenous malformations

Three patients had cerebral arteriovenous malformations (AVM) treated by occlusive embolotherapy using isobutyl-2-cyanoacrylate (IBC). At necropsy two had asymptomatic IBC pulmonary emboli. The numbers of emboli and the size of pulmonary vessels occluded correlated roughly with an increasing frequency and volume of injectate made in the cerebral tissue. The extent of pulmonary vascular occlusion in one case suggests that pulmonary vascular hypertension could develop as a complication of this form of treatment.     

Diagnostic telepathology: long-term experience of a single institution

Objectives The paper reviews the development of the application of telepathology in a department of surgical pathology between 1991 and 2003. The goal of the efforts during this time was to give up the concept of programming a single application, available only between two fixed workstations with sophisticated devices and special software, and to find the virtual largest common denominator for implementing as many different applications as possible with the same basic system.Methods A new telepathology system was designed as a client–server system with a relational database at its centre. The clients interact together by transferring the questions (texts and images) to a record (case) in the database on the server and by transferring the answers to the same record on the database.Results The new open telepathology system iPath () has been very well accepted by many groups around the world. The main application fields are: consultations between pathologists and medical institutions without a pathologist (e.g. for frozen section diagnoses or for surgical diagnoses in hospitals in South Asia or Africa), tumour boards, field studies and distance education ().Conclusions Having observed that with iPath we have succeeded in satisfying all our telepathology needs, we are inclined to put the emphasis on the nature of the tasks being performed, as opposed to the methods or technical means for performing a given task. The three organisation models proposed by Weinstein et al. (2001) [24] can be reduced to only two models: the model of discussion groups and the model of expert groups (virtual institutes).     

The treatment of severe aplastic anemia: outcomes of bone marrow transplantation and immunosuppressive therapy in a single institution of Korea

The present study represents an analysis of 96 patients with severe aplastic anemia (SAA) treated in Seoul National University Hospital, Seoul, Korea between 1990 and 1999. Twenty-two patients were treated by allogeneic bone marrow transplantation (BMT) from HLA identical sibling donors and 74 by immunosuppressive therapy (IS) with antithymocyte globulin (ATG) or antilymphocyte globulin (ALG). There was no statistical difference between the two treatment groups in age, sex, disease duration, and previous transfusion amount. In the BMT group, grade II-IV acute graft versus host disease (GVHD) developed in 10% and chronic GVHD occurred in 33% of patient. Only one patient died from complication of transplantation (veno-occlusive disease). Of 74 patients who received IS treatment, 45% achieved a complete or partial response. Twenty patients died among IS treatment group. Major causes of death were hemorrhage (40%) and infection (55%). In the BMT group, the 5-yr overall survival (OS) was 95% after a median follow-up of 42 months. In the IS group, the 5-yr OS was 70% after a median follow-up of 49 months (p=0.04). In conclusion, the long-term survival rates of SAA in Koreans receiving BMT or IS were excellent compared with the Western data. Further evaluation on the prognosis of aplastic anemia in Asians should be done.     

Analysis of prognostic factors and treatment modality changes in breast cancer: a single institution study in Korea

PURPOSE: To determine the effects of new breast cancer treatments and to provide a baseline for monitoring the development of breast cancer in Korean women, we conducted an analysis at our institution to determine long-term clinicopathological features, survival rates, and prognostic factors. MATERIALS AND METHODS: This study retrospectively analyzed 2,403 patients between Sep 1994 and Dec 2002, who underwent breast cancer surgery at Samsung Medical Center in Korea. Demographic data, pathologic records and surgical records were collected. RESULTS: After a median follow-up duration of 121.9 (range: 2-158.1) months, the 5-year disease free survival (DFS) was 82.8% and the 10-year DFS was 74.7%. The 5-year and 10-year overall survival (OS) rates were 89.4% and 82.9%, respectively. Using multivariate analyses, we determined that the nodal status (p < 0.001), angioinvasion (p < 0.001), positive PR (p < 0.001), and C-erb-B2 (p < 0.001) were independent prognostic factors for OS. The frequency of breast conserving surgery was 33.9% before Dec 1999, and increased up to 44.1% by year Dec 2002. CONCLUSION: Most of the prognostic variables and clinical characteristics of the Korean breast cancer patients were similar to those reported for Western populations. However, the age distribution in Korean patients seemed to be different from that in patients from Western countries.