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.
Primary cutaneous leiomyosarcoma (PCL) is an exceedingly uncommon malignant superficial soft tissue sarcoma with a predilection for middle-aged to elderly male. Morphologic differential diagnosis includes a host of other malignant spindle cell neoplasms, thereby necessitating the use of a panel of immunohistochemical markers to arrive at a definitive diagnosis. We report a case of PCL arising in the right leg of a 70-year-old male.
Meckel-Gruber syndrome (MKS) is an autosomal recessive disorder, characterized by a combination of renal cysts and variably associated with features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia, cysts, and polydactyly. It is a rare syndrome with highest incidence in Gujarati Indians and Finnish population. We report two such cases of MKS in non-Gujarati Indian which were diagnosed by neonatal autopsy.
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.
Kikuchi-Fujimoto disease (KFD; also called Kikuchi disease) is a rare disorder clinically characterized by lymphadenopathy along with constitutional symptoms and several systemic features which may closely mimic infections, malignancies, and autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disorder which may have several clinical manifestations similar to KFD. Association of KFD with SLE is only rarely described, and whether this is a chance incidence or Kikuchi disease is a localized manifestation of SLE per se remains a matter of debate. We report here coexistence of these two diseases in 1 patient.
We report a case of primary pulmonary Hodgkin's disease presenting as an endobronchial mass. Tissue diagnosis was made by microscopic examination following open thoracotomy and excision biopsy of the mass. The patient responded well to the chemotherapy regimen.
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