Pulmonary lymphangitic carcinomatosis-An experience at autopsy.

BACKGROUND: Pulmonary lymphangitic carcinomatosis is an uncommon mode of metastases, characterized by diffuse infiltration of the lymphatics malignant cells, and is mainly the subject of case reports or a few case series. AIMS: The aim of this study is to study the clinicopathological features of this entity at autopsy. MATERIALS AND METHODS: This is a retrospective study of data collected over an 11-year period at a tertiary-care hospital. Clinicopathological data of all autopsies with lymphangitic carcinomatosis were analyzed with reference to their demographics, clinical presentation, investigations, sites of primary cancer, and other relevant findings at autopsy. RESULTS: Among 255 cases of malignancies encountered in 7521 autopsies during the study period, 43 patients (16.9%) showed pulmonary metastasis with 22 (13 females and 9 males, mean age of 52.04 years) histologically proven lymphangitic carcinomatosis of the lungs. An antemortem diagnosis was made in only 1 of the 22 cases. The most common sites of carcinoma had been the stomach or the lungs (40.9%) and dyspnea had been the main symptom. The lungs, in addition, showed metastatic deposits and arterial thrombi and/or tumor emboli. The most common nonpulmonary site for metastasis was the liver and lymph nodes. CONCLUSIONS: Pulmonary lymphangitic carcinomatosis is often an underdiagnosed clinical entity and the presence of acute progressive respiratory failure in patients with epithelial cancers should raise clinical suspicion. This is one of the largest series to be reported at autopsy.

Aortopathy - A surgical pathology experience.

BACKGROUND: Aortopathy is a non-inflammatory and non-atherosclerotic disease of aorta that results from significant 'degenerative' changes in the media. This often leads to thoracic aortic aneurysms and/or dissections in young individuals. AIMS: This study aimed to analyze the clinical and pathological features of aortic resections performed for aortopathy. MATERIALS AND METHODS: Over the span of 15 years (2008-2022), all surgically resected specimens of aorta showing aortopathy in all age groups were studied. Particular attention was paid to the changes in the media to assess the extent, grade and severity of aortopathy. RESULTS: During the 15-year study period, 73 surgically resected specimens of the ascending aorta showed features of aortopathy. There were 48 males and 25 females, who had chief complaints of dyspnea, chest pain and palpitation. The aortopathic manifestations observed were ascending aortic aneurysms (36 patients, 49.3%), aortic dissections (21 patients, 28.8%) and aneurysms with dissections (16 patients, 21.9%). Bicuspid aortic valve (24 cases), Marfan syndrome (13 cases) and hypertension (12 cases) were commonly identified. There was one case each of Loeys-Dietz syndrome, and aortopathy possibly related to blunt chest trauma. In a significant proportion of patients (22 cases), the cause remained elusive. Moderate to severe aortopathy was observed in 60 cases (82.2%). CONCLUSION: This study helps in standardizing the histological parameters of aortopathy. In patients where the cause is uncertain despite of detailed clinico-radiological assessment, there is a need for molecular and genetic studies.

Unusual cardiac aneurysms: a surgical pathology experience.

Among the cardiac outpouchings, left ventricular aneurysms or pseudo-aneurysms that develop secondary to myocardial infarctions are the most common. On the other hand, atrial appendageal and valvular aneurysms are uncommon occurrences. The appendageal aneurysms develop possibly due to congenital dysplasia of atrial pectinate muscles, while valvular aneurysms result from infective endocarditis, mechanical injury or degenerative changes. Despite their unusual locations, they are prone to life-threatening complications. We present our experience of unusual cardiac aneurysms in surgical cardiovascular material.

Pediatric pneumonia - A clinico-pathological study.

CONTEXT: Numerous pathogens (bacteria, viruses, or fungi) can cause childhood pneumonia. The clinical presentations of viral and bacterial pneumonia can be similar. Though viruses are a more common cause as compared to bacteria, antibiotics remain the first line of treatment for pneumonia. AIMS: This study was planned to describe the pulmonary histopathological patterns in cases of pediatric pneumonia (age <12 years) at autopsy and aimed to identify the probable etiology and correlate with clinical presentations. MATERIAL AND METHODS: This is a single-center 3-year retrospective descriptive autopsy study. Relevant clinical data was correlated with the postmortem findings. The cases were assigned to one of the following categories based on probable etiology: viral, bacterial, mixed, or others. RESULTS: There were 89 cases with a postmortem diagnosis of pneumonia among 262 autopsied children (34%). Most patients had histological patterns that suggested viral and bacterial etiology in 46 (51.7%) and 27 (30.3%), respectively. A total of 35 out of 46 patients received antibiotics. Twelve cases had mixed viral and bacterial patterns. Antibiotics were also given in the remaining four children (4.5%) with a similar clinical presentation, where a diagnosis of tuberculosis (03 cases) and invasive aspergillosis (01) was made at autopsy. CONCLUSION: Neither clinical features nor investigations reliably differentiate between viral and bacterial pneumonia. Autopsy has an important role in providing insights into the pathogenesis of pneumonia and suggests inappropriate antibiotic exposure. No prior Indian studies have been performed to compare the clinical and postmortem findings of pneumonia in children.

Shaggy aorta-An autopsy analysis.

Aims: Shaggy aorta is defined as "very extensive atheromatous disease with diffuse ulcers associated with soft, loosely held debris and a paucity of actual thrombus" and often results in visceral or peripheral arterial embolization (shaggy aorta syndrome). Most of the studies are clinico-radiological with hardly any assessment of the pathological features. We present an autopsy analysis of shaggy aorta. Materials and Methods: A retrospective study of autopsied cases of shaggy aorta over 15 years was conducted. The involvement of the various segments of the aorta (ascending, transverse, thoracic, and abdominal) was correlated with the clinical manifestations and cardiac/extra-cardiac findings at autopsy. The mortality was categorized as those related to shaggy aorta (Group I), related to cardiac diseases (Group II), and those unrelated to cardiovascular diseases (Group III). Statistical Analysis: Nil. Results: In a span of 15 years, there were 76 cases of shaggy aorta affecting predominantly males (85.5%) and patients in the sixth decades of life (mean age of 64.5 years). The important associated cardiovascular risk factors included hypertension, tobacco use, and diabetes mellitus. Predominant involvement of the entire aorta and arch + descending aorta was seen in 39.5% and 35.5% of the cases, respectively. Regardless of extreme severity, only half of the patients (37 cases, 48. 7%) had clinical presentation due to shaggy aorta. Conclusions: The occurrence of shaggy aorta may be more common than expected, and it would be important to keep this possibility in mind even in asymptomatic elderly patients with cardiovascular risk factors since aorto-arterial manipulations and anti-coagulant therapy can prove detrimental in such patients.

Septic pulmonary embolism: Is it an underestimated diagnosis?

Background: Non-thrombotic pulmonary embolism, an uncommon entity, is defined as the embolization of tissues, microorganisms, air, or foreign material. One subset in this non-thrombotic category is septic pulmonary embolism (SPE) that refers to embolism of microorganisms with or without a thrombotic mantle into the pulmonary vasculature. This condition is often recognized on the basis of imaging with a clinical correlation. Unfortunately, data regarding the pathological features are meager. This has prompted to review such cases at autopsy. Aims: To study the pathological features of SPE at autopsy. Materials and Methods: Ten-year (2012 to 2021) autopsy records of the hospital were retrospectively reviewed. The diagnosis was based on the identification of acute necrotizing pulmonary arteritis with peri-bronchoarterial consolidation. These cases were analyzed with reference to the demographics, clinical characteristics, and pulmonary/extrapulmonary findings at autopsy. Statistical Analysis: Nil. Results: According to the inclusion criterion, 19 cases demonstrated the presence of SPE. There were 11 men and 8 women with a mean age of 32.1 years. The major source of infection included infection arising from skin and musculo-skeletal system (11 patients, 59.7%). The common clinical presentation included fever, dyspnea, chest pain, hemoptysis, and altered sensorium. The cause of death was mainly due to septicemia and/or confluent lung consolidations. A large number of bacterial colonies were seen in all; Candida species were also identified in two cases. Other lung findings included diffuse alveolar damage, fresh arterial thrombosis, infarction, arterial pseudo-aneurysms, abscess formation, and pyogenic pleuritis. Conclusion: Presence of an extrapulmonary infection with persistent fever, bacteremia, and pulmonary complaints should raise suspicion for this entity, particularly in resource-poor settings, to prevent grave pulmonary complications.

A rare case of metastatic malignant phyllodes tumor with osteosarcomatous differentiation presenting with intestinal obstruction.

Phyllodes tumors (PTs) are uncommon biphasic breast neoplasms constituting 0.5 to 1.0% of all breast tumors. Malignant PTs form a very small proportion of these and may metastasize, especially to the lungs and bones. Aggression and metastatic potential are accentuated in tumors exhibiting heterologous differentiation. Metastases to the gastrointestinal tract (GIT) have seldom been reported and are often confined to a segment of the digestive tract. In the absence of relevant clinical history, such patients presenting with gastrointestinal symptoms can lead to diagnostic perplexities. We report a unique case of a malignant PT with extensive osteosarcomatous differentiation and widespread metastases to the GIT.

The occult rheumatic scourge: A clinicopathological analysis of missed rheumatic heart disease.

Aims: To study the clinical and pathological manifestations of missed cases of rheumatic heart disease (RHD) and postulate possible reasons behind a missed diagnosis. Materials and Methods: Retrospective 20-year (2000-2019) autopsy data of chronic RHD were reviewed and patients, in whom the valvular deformities had been incidental autopsy findings, were selected. The clinical details of these patients were correlated with the morphology of the affected valves. On this pathological analysis, the patients were assigned to a category of subtle or significant valvular deformity. By clinically correlating, the latter group was subdivided into clinically misdiagnosed, clinically undiagnosed, and sudden cardiac death. Statistical Analysis: Nil. Results: Among the 475 cases of chronic RHD identified at autopsy in the study period, the disease was diagnosed incidentally in 69 patients (14.5%). Significant valvular deformity was noted in 61 cases while the other 8 cases had subtle valvular deformity. The most common cause of death was cardiac failure in 39 out of 69 patients (56%). Eleven (16%) patients had experienced sudden cardiac death. Among the undiagnosed cases, 5 (7%) of them had a diagnosis of non-rheumatic cardiac disease, while the other 14 (20.5%) patients had overwhelming non-cardiac diseases. Conclusions: Our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies.

Peri-adventitial smooth muscle - inheritance of the iliac arterial system?

The histo-morphology of the arterial walls is typically made of 3 distinct layers or tunics designated as intima, media and adventitia. Based on the composition of the media, the arteries are classified into elastic and muscular types. The common iliac artery is an elastic artery, whereas its branches, the external and internal iliac arteries are muscular arteries. In this study, the presence of smooth muscle bundles outside the adventitia was noted in 93 samples taken from the iliac arterial system and the reasons for their presence have been hypothesized.

Pathology of pulmonary tuberculosis: has the tiger changed it's stripes?

Background: India accounts for the highest number of TB cases globally (almost one-fifth of the global burden and almost two-thirds of the cases in South East Asia. Furthermore, the development of drug resistance of varying levels such as multi-drug resistant TB (MDR-TB), extensively-drug resistance TB (XDR-TB) and total-drug resistant TB (TDR-TB) has been on the increase, and now India also features in the 27 high-MDRTB-burden countries. Almost parallel to these developments, in the last few years, we have been encountering less common morphological forms of pulmonary TB (PTB) at autopsies. With these less common manifestations of the disease, we undertook this study to examine the changing trends in the morphological pattern of pulmonary TB over the recent years. Methods: In this 3-year retrospective study, adult autopsy cases of PTB (that significantly contributed to the final cause of death) were studied in detail. HIV-positive cases were excluded from the study. The clinical details, gross appearances of the pulmonary lesions, microscopic pattern and Ziehl-Neelsen (ZN) staining were studied. Extrapulmonary involvement and causes of death were documented. Results: Pulmonary tuberculosis as a cause of death at autopsy was seen in 130 adult patients over 3 years. The age range was between 12 to 70 years. Anti-tuberculous therapy had been administered in 33 of them, but only one patient had taken complete therapy. Dyspnea was the commonest respiratory symptom seen in 51 cases (39.2%). Tuberculous bronchopneumonia was the commonest lesion (45.3%), miliary lesions (including localized miliary) accounted for 26% while fibrocavitary lesions (including the ones not involving apex) were seen in 13% cases. Other morphologies included nodular forms of TB (13%), localized miliary lesions (11.9%), and fibrocavitary lesions, not necessarily involving the apex (11.7% of all fibrocavitary cases), and predominant pleuritis with underlying lung involvement by TB in 1 case. Many cases of TB bronchopneumonia had a bronchocentric pattern of distribution (14.7%). On microscopy, caseating granulomas were seen in 93% cases, only caseation necrosis was seen in 4.6% cases, and necrotizing granulomas with abscess-like reaction in 11.5% cases. ZN staining was positive in 92 cases (70.7%). All the extrapulmonary lesions showed caseating granulomas histologically. The final cause of death was found to be primarily tuberculous in 106 cases (81.5%), whereas in 24 cases (19.5%) pulmonary TB was attributed to the secondary cause of death. Conclusion: The typical apical involvement of secondary TB was not seen in most of our cases. This could indicate a difference in the morphology and the pattern of lung involvement in recent years. The difference in gross morphology does not affect the pattern of involvement of the lung. In our study, we have observed both; a change in morphology, i.e., more cases of TB bronchopneumonia, and a change in the pattern of involvement like nodular forms, localized miliary forms, and fibrocavitary lesions not necessarily involving the apex. We postulate that this less common manifestation of pulmonary TB is closely related to the development of multi-drug and microbial resistance posing serious medical challenges.

Long-term, real world experience of ventricular tachycardia and granulomatous cardiomyopathy.

BACKGROUND: Granulomatous cardiomyopathy (GCM) is relatively uncommon in patients presenting with ventricular tachycardia (VT). Sarcoidosis and tuberculosis are the most common causes of GCM with VT. The aim of study was to evaluate their clinical characteristics and the long-term outcomes. METHODS: We retrospectively analyzed patients from March 2004 to January 2020, presenting with VT and subsequently diagnosed to have GCM. Patients were divided into three groups (sarcoid, tuberculosis and indeterminate) based on serologic tests, imaging and histopathology. The response to anti-arrhythmic and disease specific therapy on long-term follow-up were analyzed. RESULTS: There were 52 patients, comprising 27 males and 25 females, age 40 ± 10 years. The follow-up period was 5.9 ± 3.9 years. Sarcoidosis was diagnosed in 20 (38%); tuberculosis (TB) in 15(29%) and 17(33%) patients were indeterminate. Left ventricular ejection fraction (LVEF) of the entire cohort was 0.45 ± 0.14. Erythrocyte Sedimentation Rate(ESR) was found to be significantly higher in TB(43.6 ± 18.4) patients vs sarcoid(18.9 ± 6.7)p < 0.0001, but not the indeterminate group (36.2 ± 21.1), p = 0.3. Implantable Cardioverter Defibrillator (ICD) implantation was performed in 12/20(60%) patients in the sarcoid group, in 4/15(27%) patients in the TB group and in 10/17(59%) patients in the indeterminate group. At a mean follow-up of six years, VT recurrences were noted in 6, 2, and 7 patients in the sarcoid, TB and indeterminate groups respectively. CONCLUSION: Despite the advances in diagnostic modalities for tuberculosis and sarcoidosis, in real-world practice, almost one-third of the patients with VT and GCM have uncertain etiology. Long term outcomes of patients presenting with GCM and VT with mild left ventricle dysfunction treated appropriately seems favorable.

Disseminated epithelial cancers-An autopsy analysis.

BACKGROUND: Cancer is one of the leading causes of death due to noncommunicable diseases worldwide. Despite increasing public awareness and availability of sophisticated imaging techniques, some cancers evade clinical diagnosis and/or are incidentally encountered at autopsies, often with dissemination. AIMS: The present study evaluated the disseminated epithelial cancers at autopsy. MATERIALS AND METHODS: This is a retrospective observational 5-year autopsy analysis of disseminated epithelial cancers performed at a tertiary-care hospital. The cases were categorized as (1) clinically diagnosed malignancy, known primary; (2) clinically diagnosed malignancy, unknown primary; and (3) clinically undiagnosed malignancy. STATISTICAL ANALYSIS: Nil. RESULTS: Dissemination was identified in 66 (57.9%) of the 114 patients with epithelial malignancies. There were 29 patients (43.9%) in category 1, 26 patients (39.4%) in category 2, and 11 patients (16.7%) in category 3, majority of whom were women (38 patients, 57.6%). When all categories were considered together, lung and colorectal carcinomas were the commonest cancers seen in 13 (19.7%) and 8 (12.1%) patients, respectively, in both men and women. Majority of the patients (43 cases, 65.2%) had symptoms produced by metastases, which were the sole manifestations in 13 patients (19.7%). Lungs and liver were the common metastatic sites. CONCLUSIONS: Cancerous dissemination continues to be a major cause of morbidity and mortality even after considerable improvements in the surgical or nonsurgical treatment modalities. An autopsy study can provide important clinical insights in retrospect.

Thymectomy for myasthenia gravis: A pathological analysis.

BACKGROUND: Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology. AIMS: This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia. MATERIALS AND METHODS: This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG. STATISTICAL ANALYSIS: Nil. RESULTS: There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively. CONCLUSION: The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.

Acute rheumatic fever - A pathological analysis of clinically missed cases.

BACKGROUND: Acute rheumatic fever (ARF) and its post-inflammatory sequel chronic rheumatic heart disease (RHD) are endemic in the Indian setting. Despite the updated Jones criteria, many cases of ARF remain undiagnosed or are missed. AIMS: This study aims to analyze pathological profiles of such cases and their importance in context of clinical presentation and Jones criteria. MATERIALS AND METHODS: A 22-year retrospective observational study of ARF was conducted in the Department of Pathology in a tertiary care institute. The cases were categorized as 1. Those fulfilling and 2. those partially or not fulfilling the Jones Criteria. Based on the autopsy findings, the lesions were classified as categorized mitral stenosis and/or regurgitation (MS ± MR) and pure mitral regurgitation (MR). STATISTICAL ANALYSIS: Nil. RESULTS: In 22 years, among 697 cases of autopsied cases of RHD, there were 59 cases (8.5%) of ARF. Among them, seven cases fulfilled the Jones criteria; five of them were clinically diagnosed. The remaining 52 cases (88.1%, 34 with MS ± MR and 18 with MR) did not fulfill or partially fulfilled the Jones criteria and were not diagnosed. A total of 18 patients (30.5%) had the first attack of ARF, whereas recurrences were noted in the remaining patients (69.5%). CONCLUSIONS: The study indicates that the typical manifestations of ARF under Jones criteria may not be present, especially in cases with recurrence. Hence, a presumptive or possible diagnosis of ARF can be made with presence of minor criteria or strong clinical suspicion in such cases.

The Ring-and-Sling complex - Does it "Ring" true?

BACKGROUND: The "Ring-and-Sling" complex (RSC) comprises congenital tracheal stenosis and an abnormal origin/course of the left pulmonary artery. Based on clinical and imaging studies, the luminal narrowing is assumed to be as a result of rings cartilage (forming an "O"). AIMS: This is a postmortem based study of tracheal histology in infants after an autopsy encounter of a case of RSC. SUBJECT AND METHODS: RSC was identified in an infant at autopsy. The tracheal histomorphology revealed the presence of cartilaginous plates (instead of rings) and fibro-elastotic proliferation at the site of trachealis muscle. These changes prompted a study on variations in the histology of the trachea (with no known anomaly) in 35 autopsied neonates and infants. The transverse sections of the trachea were taken at one or more levels (Level 1 - at the level of the thyroid, Level 2 - midway between the thyroid and the carina, and Level 3 - just above the carina. STATISTICAL ANALYSIS: Epi-info software (v1.4.3, CD, US). RESULTS: On histology, 83 sections showed the trachealis muscle on the posterior aspect. A single semicircular cartilage was identified in only 17 of the 83 sections studied (20.5%, 6 in level 1, 9 in level 2 and 2 in level 3). In the remaining 66 sections (79.5%), the cartilage was disposed as multiple plates, ranging in number from 2 to 10. No significant association was found between semicircular cartilage rings and age, sex, gestational age, and level of section (P < 0.05). However, 14 cases with sectioning at all three levels were taken into account; all levels showed more cartilaginous plates compared to single rings, which were more common at level 1 (P > 0.05). CONCLUSIONS: The "ring" in RSC and normal infantile tracheas show cartilage plates with intermittent semicircular cartilage rings. These findings may have surgical implications for tracheal anomalies and bode favorable surgical outcomes.

Sudden death due to amyloidosis of intramural coronaries in a patient with amyloidosis of hip joint - An autopsy report with a review of literature.

Amyloidosis is caused by an extracellular accumulation of insoluble fibrillary protein predominantly in the kidneys, spleen, and heart. The deposition of amyloid into the joints, synovia, and osseous tissues (amyloid arthropathy) is an uncommon condition with only a few case reports in the English literature. Similarly, amyloid deposition predominantly limited to the vascular wall is rarely described. In this report, we describe an additional case of amyloidosis of the hip joint along with amyloidosis of intramural coronaries leading to sudden death in a middle-aged male.

Incidental dural-based mass: Surprisingly leiomyoma.

Leiomyoma is a benign smooth muscle cell tumor commonly occurring in the uterine myometrium. Extra-uterine tumors are infrequent and those occurring in an intracranial location are extremely rare. We report a primary intracranial leiomyoma as an incidental autopsy finding in a 60-year-old woman, who died of acute myocardial infarction.