An autopsy study of hematolymphoid malignancies

Background: Hematolymphoid malignancies (HLM) are primary cancers of blood, bone marrow and lymphoid organs associated with high mortality. Clinically undetected cases, diagnosed primarily at autopsy, are common.Methods: Cases of HLMs at autopsy performed in our hospital from 1st January 2009 to 30th June 2017 were analysed. Gross and microscopic findings at autopsy along with clinical parameters were studied. Special stains and immunohistochemistry (IHC) were performed wherever possible.Results: There were 49 cases of HLMs (0.98 %) among 4971 autopsies conducted during the study period, occurring predominantly in males (70 %). Fifteen (30.61 %) were primarily diagnosed at autopsy. There were 26 lymphomas and 19 leukemias; three patients had multiple myeloma and one patient had Langherhan cell histiocytosis. Non-Hodgkin’s lymphoma was the commonest (24 patients). Nine were diffuse large B-cell lymphoma; rare sub-types included angiotrophic lymphoma, post-renal lymphoproliferative disorder and hepatosplenic gamma-delta T-cell lymphoma. Among the 19 leukemias, majority were acute (13 cases) with seven cases of myeloid and six of lymphoid types. IHC was performed in 21 cases which aided the diagnosis. The commonest symptoms were fever (43%) and dyspnoea (37%). Splenomegaly (67%), hepatomegaly (61.2%) and lymphadenopathy (57.1%) were the most common autopsy findings. Infiltrations were seen in various organs; an acute myeloid leukemia (M6) had infiltration of the atrio-ventricular node.Conclusions: One-third cases in the present study were diagnosed at autopsy without any prior clinical suspicion thereby emphasizing on the relevance of autopsy in the current practice of pathology and further studies to improve accuracy of ante-mortem diagnosis.

Cardiac sarcomas: A gist

In large cities, cancer management has moved to highly specialized centers that provide holistic care. Hence, freelance consultants very occasionally encounter cancer patients. Sometimes due to a variety of reasons, one may not have much to offer to a patient in terms of medical management. However, empathy and small acts of kindness can have a major impact on patients going through tough times and must be always remembered. A glass of water is a true narrative of how seemingly benign words can make a patient aloof when one least expects and how small acts of kindness can change their equation with the doctor. It reinforces the fact that the art of medicine is still the better half of the science of medicine and must be practiced at all times.

A child with neurological deficits, electrolyte imbalance, and arrhythmia.

A child presented with an acute febrile illness associated with neurological symptoms. The differential diagnoses of such a presentation with effects of prolonged hospitalization is discussed.

Pulmonary arterial intimal sarcoma with retrograde extension: Report of a case and review of literature.

Most of the pulmonary arterial sarcomas arise from multi-potential mesenchymal intimal cells and are designated as intimal sarcomas. These tumors grow in the direction of blood flow into peripheral arteries producing clinical features mimicking pulmonary thromboembolism. Retrograde extension is rare. We report one such case of intimal sarcoma that had a retrograde extension into the right ventricular outflow tract, and review such a presentation in the last ten years.

Wilms' tumor with right heart extension: Report of a post-chemotherapeutic fatality.

Wilms' tumor (WT) has a strong propensity to invade the vasculature in the form of tumor-thrombus, into the renal veins, and inferior vena cava and even into the right atrium. This cavo-atrial propagation does not alter the prognosis and pre-operative chemotherapy produces shrinkage to the extent of even disappearance of caval or atrial extensions. We present a case of sudden death due to hemorrhagic expansion of the intra-atrial component of WT, immediately after commencement of chemotherapy, an uncommon incident.

Microscopic thymoma: A report of four cases with review of literature.

Context: Thymectomy is an effective therapy for patients with myasthenia gravis, and the lesions usually identified are lymphoid hyperplasia and thymomas. Nodular epithelial proliferations, termed as microscopic thymoma, are rare. Aims: Our aim was to assess the incidence and clinicopathological features of microscopic thymomas. Setting and Design: Retrospective analyses. Materials and Methods: Thymectomies performed in 105 patients with myasthenia gravis were reviewed, and patients with microscopic thymomas were studied with respect to their clinicopathological features. These were compared with those in other reports in literature. Results: Four thymuses showed features of microscopic thymomas, an incidence of 3.8%. There were 2 males and 2 females, all myasthenic; the mean age was 31 years. The resected thymic tissues were seen as soft, capsulated adipose tissue. They showed variable degrees of involution or were atrophic. All had multifocal proliferation of ovoid-to-polygonal cells with tongue-like insinuations into the fat. After thymectomy, there was improvement in the symptoms. Eight patients reported in literature had similar clinicopathological features. Conclusions: Thorough examination of the thymectomies is required to increase the detection of these lesions for assessment of their incidence, causes, pathogenesis and functional significance.

Viral ventilator-associated pneumonia: Uncovering tip of the iceberg.

Context: Hospital-acquired infections are frequently encountered by the physicians for ailments demanding prolonged hospitalization, especially in intensive care units, where patients are often mechanically ventilated. The organisms most often implicated are bacteria; viral etiology is infrequent. Aims: The study aims at reviewing lung pathology at autopsy in mechanically ventilated children admitted in pediatric intensive care unit (PICU) to assess the incidence of viral ventilator-associated pneumonias (VAP). Setting and Design: Retrospective analysis. Materials and Methods: Among the 275 children who had been autopsied, 13 who had been admitted in the PICU satisfied the criteria for VAP. These cases were analyzed on the basis of clinical data and pulmonary pathology. Depending on the overall histology, the cases were classified as being viral or bacterial in etiology. Immunohistochemistry (IHC) for detection of viral antigens was also performed. Results: Of the 13 children, nine (five males and four females) had shown the histomorphologic features, suggesting viral inflammation. The mean age was 33 months. Falling oxygen saturation and increasing respiratory distress had necessitated ventilator support. Acute lymphocytic bronchiolitis, interstitial pneumonitis, diffuse alveolar damage, and necrotizing pneumonia were the histological features. The viruses identified in five patients were adenovirus, respiratory syncytial virus and cytomegalovirus. Conclusion: This communication, though not representing the true incidence, emphasizes that a proportion of nosocomial infections is due to viral infections. This should alert the treating intensivists to actively pursue investigations to confirm viral etiology.

Intracardiac leiomyomatosis: Report of two cases.

Intravenous leiomyomatosis is an example of unusual growth patterns that is sometimes exhibited by uterine leiomyomas, characterized by smooth muscle proliferations within the uterine or pelvic veins. Extension into the heart via the inferior vena cava is even rarer and is designated as intracardiac leiomyomatosis. This is a report of two such cases that had preoperative diagnoses of a thrombus and right atrial myxoma, respectively.

Syphilitic aortitis: Rearing of the ugly head.

Context: Syphilitic aortitis has been relegated to the category of rare cardiovascular disease or a "medical curiosity" in the west. The same situation may not exist in developing countries due to the stigmata that continue to remain attached to sexually-transmitted diseases in general. Aims: To study the prevalence of syphilitic aortitis among autopsied non-atherosclerotic aortic diseases encountered in a span of 15 years. Settings and Design: Retrospective, autopsy-based study. Materials and Methods: Among 187 cases of non-atherosclerotic diseases of the aorta, 44 had been diagnosed as syphilitic aortitis on the basis of the pathological features and serology. The demographic details and modes of clinical presentation were retrieved from the health records. Depending on the presence of complicating lesions, the cases were classified as uncomplicated or complicated aortitis. Results: The 44 cases of syphilitic aortitis formed 23.5 % of the non-atherosclerotic aortic diseases. They were predominantly seen in males in the fifth decade, who often presented with valvular regurgitation, aneurysmal disease or myocardial ischemia; 13.6 % of patients were asymptomatic. Blood VDRL results were available in 19 patients; 84.2 % were positive. Concomitant involvement of the ascending, transverse and descending thoracic was seen in 45.5 % of cases. None had uncomplicated aortitis. Complications in the form of aortic regurgitation (72.7 %), coronary ostial stenosis (59 %) and aneurysms (59 %) frequently coexisted. Thirty-five aneurysms were present in 59 %, chiefly involving the aorta. Conclusions: We found syphilitic aortitis to be a common cause of aortitis at autopsy. Diagnosis should be made with the help of characteristic pathological features correlated to the clinical context and appropriate serological tests.

Nitric oxide synthase 3 and endothelin 1 immunoreactivity in pulmonary hypertension.

Context: Pulmonary hypertension (PH) is a serious and sometimes life-threatening event that occurs as a complication of various cardiopulmonary disorders, of which rheumatic heart disease (RHD) is an important example in our country. The pathogenesis of PH is a complex, multistep process in which "pulmonary endothelial dysfunction" (PED) is widely regarded as the central pathogenetic event. PED is, in turn, influenced by several local and systemic factors, of which nitric oxide synthase 3 (NOS3) and endothelin 1 (ET1) are 2 prime candidates, and are the subject of our study. Aims: Our aim was to study the immunoreactivity of NOS3 and ET1 in the pulmonary vasculature of PH patients of various etiologies, with emphasis on RHD cases. Settings and Design: A retrospective, autopsy-based study. Subjects and Methods: A total of 49 autopsy cases (39 patients and 10 controls) were chosen for our study. Of the 39 patients, 20 had PH secondary to RHD, whereas the remaining 19 patients had non-RHD etiologies as the basis of their PH. Lung sections taken from all the 49 cases were subjected to routine H and E, elastic van Gieson, and immunohistochemical staining (with NOS3 and ET1 separately). The intensity of immunostaining in all the cases and controls were then graded as focal/diffuse and weak/strong. Results: Controls showed positivity for both NOS3 (bronchiolar epithelium) and ET1 (endothelium of pulmonary arteries). Characteristic changes of PH on H and E were seen in 14 out of 19 non-RHD cases, which matched with the number of ET1 positivity cases. Similarly, for the RHD cases, 14 out of 20 cases showed changes of PH on H and E, but only 2 cases showed mild, focal positivity for ET1. Surprisingly, NOS3 positivity was largely absent in both the non-RHD and RHD cases. Conclusions: Our study showed NOS3 negativity and ET1 positivity in the lung vasculature of patients with PH, a conclusion more or less in line with the predominant view of the other investigators in this field. But at the same time, our study could not conclude an unequivocal role of NOS3 in PH, whereas it could, in the case of ET1.

Sclerosing hemangioma with lymph nodal metastases.

A case of sclerosing hemangioma of the lung is reported in a young male, who presented with recurrent cough and streaky hemoptysis for three years. The tumor was situated in the right upper lobe and was large (over 9 cm), multicentric and associated with metastases to the regional lymph nodes. To the best of our knowledge, only 14 cases with such metastases have been reported.

Necrotizing adenoviral pneumonia: manifestation of nosocomial infection in pediatric intensive care unit.

A 5-year-old child admitted in the pediatric intensive care unit developed fever and crepitations in the chest on 6(th) day of admission. She succumbed to her illness depite administration of adequate supportive and ventilatory care and anti-microbial therapy. At autopsy, she was diagnosed to have chronic ligneous type of tuberculous meningitis and necrotizing adnoviral pneumonia. There are hardly any reports of nosocomial adenoviral pneumonia from Indian centers. The case serves to remind intensivists to consider this diagnosis so that appropriate therapeutic adjustments and measures to prevent the spread of infection to other critically ill subjects are initiated.

Pathology of pulmonary aspergillomas.

Aspergilloma refers to a fungal ball formed by saprophytic overgrowth of Aspergillus species and is seen secondary to cavitatory/cystic respiratory diseases. Paucity of clinical and pathological data of aspergilloma in India prompted us to analyze cases of aspergilloma over 15 years. The clinical features were recorded in all and correlated with detailed pathological examination. Aspergillomas were identified in 41 surgical excisions or at autopsy. There was male predominance; half the patients were in their fourth decade. Episodic hemoptysis was the commonest mode of presentation (85.4%). Forty aspergillomas were complex, occurring in cavitatory lesions (82.9%) or in bronchiectasis (14.6%). Simple aspergilloma was seen as an incidental finding in only one. Tuberculosis was the etiological factor in 31 patients, producing cavitatory or bronchiectatic lesions; other causes were chronic lung abscess and bronchiectasis (unrelated to tuberculosis). Surgical resections are endorsed in view of high risk of unpredictable, life-threatening hemoptysis.

Pulmonary endarteritis.

Pulmonary arterial endarteritis is a rare event even in patients with congenital heart disease. In this paper, the authors report two cases of pulmonary endarteritis diagnosed at autopsy. The first was a 3-month-old male infant with absence of congenital heart defect (who had had neonatal sepsis). This patient presented with pneumonia and succumbed within 36 hours of hospital stay despite appropriate antimicrobial therapy and supportive management. The second patient was a 13-month-old male child with previously undiagnosed tetralogy of Fallot who had clinical presentation of acute meningitis with convulsions and succumbed within 12 hours of hospital stay despite adequate treatment. The main autopsy findings were chronic arteritis of the pulmonary trunk and right pulmonary artery in the former and rupture of the pulmonary trunk in the latter.

Acute lung injury due to cadmium inhalation--a case report.

Heavy metal inhalation is a rare cause of acute lung injury. Among the various heavy metals, cadmium is more commonly known to cause acute lung injury. A case of accidental inhalation of cadmium fumes in a young male is presented. The incident occurred in local silver jewellery manufacturing unit.

Valvular vegetations? Cardiac amyloidosis is a possibility.

Cardiovascular system is a frequent target for the deposition of amyloid, specially in the primary type. Patients with this condition can present with myocardial dysfunction due to heavy interstitial myocardial deposits. We report a case of primary cardiovascular amyloidosis in a male with sudden cardiac death. The distribution of amyloid was remarkable for exclusive deposition in the pulmonary and systemic arterial adventitia and more strikingly over all valves, specially the mitral valve. These were large enough to simulate vegetations.