Congenital cystic adenomatoid malformations (CCAM) also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology. It is a rare disease with an incidence of 1 in 25,000 to 1 in 35,000. It is a disease of infancy with most of the cases diagnosed within first 2 years of life. We report autopsy findings of two fetuses with congenital cystic adenomatoid malformation (Stocker Type II and I) with brief review of literature.
Introduction:Peripheral nerve sheath tumors (PNSTs) are neuroectodermal in origin. Now these tumors are classified under World Health Organization (WHO) classification of tumors of soft tissue and bone 2013.Objective:To study the morphological spectrum of PNST and to study the secondary degenerative changes associated with it.Materials and Methods:This study was conducted from January 2010 to June 2015. The gross details of tumor and patient's demographic profiles were reviewed. The hematoxylin and eosin stained slides were reassessed and the lesions were categorized and classified as per the WHO 2013 classification. The tumors were also assessed for secondary degenerative changes.Results:Our study comprised 143 cases of PNST. Age of the patients ranged from 5 to 75 years. 21–30 years is the most common age of occurrence with head and neck being the most common site. The PNSTs observed in the present study were neurofibroma (NF) (61.5%), schwannoma (36%), malignant PNST (2%), and granular cell tumor (0.5%). Nearly 10% of NF fulfilled the criteria for neurofibromatosis 1 (NF1). Rare tumors such as plexiform schwannoma and granular cell tumor were also observed. Malignant tumors were larger in dimension than benign. Myxoid, cystic, and hyaline changes were commonly associated with benign tumors while necrosis, hemorrhage, and mitotic activity were seen with malignant tumors.Conclusion:This series highlights the pathological variants of PNST along with their morphological changes and NF1 association. It is essential to be familiar with all these variants of PNST for accurate diagnosis as they have varied biological behavior.
We present two unusual cases of carcinoma of palatine tonsil in elderly patients. Both the cases were initially diagnosed as squamous cell carcinoma of the tonsil, second case, in addition, showed lung metastasis. On subsequent follow-up, the first case developed cutaneous metastasis in the right frontoparietal region while second case showed granulocytosis as paraneoplastic manifestation. The association of cutaneous metastasis and paraneoplastic granulocytosis with carcinoma of tonsil is rare. Cutaneous metastasis has been described only once in the literature while paraneoplastic granulocytosis is being reported for the first time. Both the conditions need long-term follow-up as they manifest at the extreme edges of the neoplastic process.
The routinely used urinary reagent strip can be utilized for the rapid analysis of CSF. If implemented, this technique will be useful in rural areas. It would also decrease the turnaround time in centers where the facility is available.
Clear cell sarcoma is a deep-seated, exceedingly rare aggressive tumor, typically involving the tendons and aponeuroses with melanocytic differentiation and a distinct genetic background. A primary dermal location is rarer. It exhibits histological, immunohistochemical, and ultrastructural similarities with the more common primary (or metastatic) malignant melanoma causing major diagnostic confusion. We describe a case of primary cutaneous clear cell sarcoma arising in the right lower extremity of a 40-year-old male patient.
Background
Early and accurate diagnosis followed by timely treatment are the key prerequisites to fight tuberculosis (TB) and reduce its global burden. Despite scientific advances, the rapid and correct diagnosis of both pulmonary and extrapulmonary tuberculosis remains a challenge due to traditional reliance on detection of the elusive bacilli. Mycobacterium tuberculosis (Mtb)-specific host immune activation and cytokine production have shown significant promise as alternative means of detecting and distinguishing active disease from latent infection. We queried the diagnostic ability of phenotypic markers on Mtb-specific cytokine-producing immune cell subsets for identifying active tuberculosis.
Methods
Subjects belonging to the following groups were recruited – pulmonary and extrapulmonary TB, latent TB, cured TB, sick controls and healthy controls. Polychromatic flow cytometry was used to identify host immune biomarkers in an exploratory cohort comprising 56 subjects using peripheral blood mononuclear cells. Clinical performance of the identified biomarker was evaluated using whole blood in a blinded validation cohort comprising 165 individuals.
Results
Cytokine secreting frequencies of Mtb-specific CD4 + T cells with CD38 +CD27 – phenotype clearly distinguished infected individuals with active tuberculosis from those without disease. TNF-α secretion from CD38 +CD27 –CD4 + T cells upon stimulation with ESAT6/CFP10 peptides had the best diagnostic accuracy at a cut-off of 9.91% [exploratory: 96.67% specificity, 88.46% sensitivity; validation: 96.15% specificity, 90.16% sensitivity]. Additionally, this subset differentiated treatment-naive TB patients from individuals cured of TB following completion of anti-tuberculosis therapy.
Conclusions
Mtb-specific CD38 +CD27 –TNF-α +CD4 + T cell subset is a robust biomarker both for diagnosing tuberculosis and assessing cure.
We present autopsy findings of a case of limb body wall complex (LBWC). The fetus had encephalocele, genitourinary agenesis, skeletal anomalies and body wall defects. The rare finding in our case is the occurrence of both cranial and urogenital anomalies. The presence of complex anomalies in this fetus, supports embryonal dysplasia theory of pathogenesis for LBWC.
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