Cancer as a marker of genetic medical disease: an unusual case of medullary carcinoma of the kidney.

Medullary carcinoma is a recently recognized tumor of the kidney with distinctive microscopic features; the most notable are diffuse and glandular growth patterns, inflammatory infiltrates, and rhabdoid/plasmacytoid cells. It is a clinically aggressive tumor that occurs in relatively young patients. Moreover, this tumor shows a peculiar clinical association: it occurs in patients with sickle cell hemoglobinopathy. The case presented is that of a 37-year-old black woman with a history of bronchial asthma who died suddenly. Autopsy showed a 4-cm renal mass with extension to the inferior vena cava and metastases to the liver. Histologic evaluation showed the characteristic findings of medullary carcinoma of the kidney. This diagnosis prompted the investigation and subsequent detection of sickle cell trait in the deceased, alerting the family to the genetic nature of her illness. This case is the first report of this entity since the original described series of patients and shows the unique nature of this cancer as a marker of a genetic medical disease.

Papillary mucinous adenoma arising in adenomyomatous hyperplasia of the gall bladder.

A case of papillary mucinous adenoma arising in adenomyomatous hyperplasia (AMH) of the gall bladder is reported. The lesion was unsuspected and discovered by routine palpation of the gall bladder during laparotomy. The adenoma developed within fundal AMH and showed cytological atypia. This case illustrates that neoplastic proliferation is indeed possible in AMH and challenges the classical opinion that AMH is devoid of neoplastic potential.

Disseminated pneumocystosis without pulmonary involvement during prophylactic aerosolized pentamidine therapy in a patient with the acquired immunodeficiency syndrome.

Pneumocystosis, the most common opportunistic infection associated with the acquired immunodeficiency syndrome, is usually restricted to the lungs and results in severe bilateral pneumonia, which is fatal unless vigorously treated. Rare cases have been reported in which involvement of other organs or disseminated disease occurred in addition to the pulmonary lesions. Pentamidine, an efficient drug used intravenously for the treatment of pulmonary pneumocystosis, has also recently been used in aerosolized form for the prevention of Pneumocystis infection in patients with the acquired immunodeficiency syndrome. In the present case, widely disseminated, though symptomless, pneumocystosis developed in a human immunodeficiency virus-positive individual treated prophylactically with aerosolized pentamidine. Despite heavy multiorgan infection with Pneumocystis carinii, the lungs revealed no microorganisms or characteristic inflammatory lesions. This case indicates that aerosolized pentamidine, while efficient against the pulmonary infection, may not produce fungicidal blood levels sufficient for the prevention of disseminated pneumocystosis.

Lacerations of the hepatoduodenal ligament, pancreas and duodenum in a child due to blunt impact.

Descriptions of the nature of pediatric injury as reported by parents and caretakers is frequently tainted, even fraudulent. We present here such a case of trauma incurred in the presence of a parent which resulted in hepatoduodenal ligament laceration with associated pancreatic and duodenal injuries, the certain result of severe blunt force abdominal trauma. These findings were at variance with the father's description of the events leading up to the child's untimely demise.

Large sub-pleural air cysts: an extreme form of pulmonary interstitial emphysema.

Pulmonary interstitial emphysema is a well-documented complication of positive-pressure ventilation. However, the occurrence of large sub-pleural air cysts is a less well-known, extreme manifestation of this entity. We present here an infant who developed this complication of pulmonary barotrauma during cardiopulmonary resuscitative efforts.

Adrenal lacerations in child abuse: a marker of severe trauma.

There are few reports of adrenal injuries complicating inflicted pediatric trauma. All child-abuse cases at the New York City Medical Examiner's Office over a 7-year period were reviewed. Of these 121 homicides, 50 sustained severe injuries to the torso. Five infants (10.0%) had adrenal laceration, a marker of major blunt-force injury.

Spontaneous uterine rupture with fatal hemoperitoneum due to placenta accreta percreta: a case report and review of the literature.

Placenta accreta percreta is an unusual cause of hemoperitoneum. Less than 50 such cases have been reported in the English-language literature over the past 100 years. A 22-year-old pregnant multigravida woman who had spontaneous uterine rupture with exsanguination caused by this entity is described. She had abdominal pain and cardiovascular collapse and died without a premortem diagnosis.

Non-accidental pediatric pelvic fracture: a case report.

Few reports have documented pelvic fracture as a result of child abuse. We present a case with radiologic findings and pathologic correlation. Although documented patterns of osseous injury have been extensively described, a thorough skeletal evaluation must include a frontal view of the pelvis as well.