Methamphetamine Injection Into Face Causing Fatal Cavernous Sinus Thrombosis.

ABSTRACT: Cavernous sinus thrombosis is a rare and often fatal condition, usually associated with infections in the head and neck region. Clinical presentation includes fever, headache, and periorbital swelling. Mortality can be high; however, prompt treatment can reduce the probability of death. We present a case of a cavernous sinus thrombosis associated with methamphetamine injection into the face.

A Case Series of Acute Hemorrhagic Leukoencephalitis.

ABSTRACT: Acute hemorrhagic leukoencephalitis (AHL) is an acute, hemorrhagic demyelinating disease thought to be caused by an immune-mediated process. Acute hemorrhagic leukoencephalitis is both diagnostically challenging and fatal in the majority of cases. We present two cases of AHL unexpectedly diagnosed at autopsy. These cases demonstrate the often nonspecific and challenging nature of AHL clinical presentation, review neuropathological mimics, and emphasize the importance of considering this diagnosis in the forensic setting.

Fatal Hemoperitoneum Due to Isolated Splenic Peliosis.

ABSTRACT: Isolated splenic peliosis is an exceedingly rare condition of unclear etiology characterized by blood filled cyst-like cavities in the primary mononuclear phagocytic organ. People with splenic peliosis are typically asymptomatic. However, splenic peliosis may present as an incidental mass on imaging mimicking a neoplasm during life, or present as sudden death after hemoperitoenum when ruptured, mimicking traumatic injury. Awareness of this condition is important to forensic pathologists so as to provide an accurate cause and manner of death.

Maternal Death Due to Clostridium novyi in an Injection Drug User.

BACKGROUND: Soft-tissue infections in women with subcutaneous injection drug use are often polymicrobial. CASE: A 21-year-old nulliparous woman presented at 14-15 weeks of gestation with several gluteal abscesses related to subcutaneous injection of heroin. She was well appearing, afebrile, and without findings of systemic illness. After empiric broad-spectrum intravenous antibiotics, same-day incision and drainage was performed. Immediately after the procedure, she developed refractory hypotension, severe tachycardia, pulmonary edema, and leukemoid reaction (white blood cell count 80×10/L). Cardiac arrest ensued, and she died within 4 hours of onset of sepsis and within 24 hours of presentation. Wound cultures identified Clostridium novyi. CONCLUSION: Local, soft-tissue infections that includes C novyi can result in rapidly fatal, toxin-mediated septic shock in pregnant women with high-risk social behavior.

Mullerian adenosarcoma of the cervix with heterologous elements and sarcomatous overgrowth.

Cervical adenosarcomas are exceedingly infrequent tumors that occur most often in women of reproductive age. Adenosarcomas comprise benign epithelial elements and malignant stromal elements. The malignant stromal elements can either be homologous, such as fibroblasts or smooth muscle, or heterologous, like cartilage, striated muscle, or bone. We report a case of adenosarcoma of the cervix with heterologous elements and sarcomatous overgrowth in a 38-year-old woman.

Anaplastic large-cell lymphoma in AIDS.

AIDS-related malignancies may alter clinical courses and result in death in critically ill patients. We present a case of a newly diagnosed AIDS patient with Pneumocystis jiroveci pneumonia, Epstein-Barr virus, and cytomegalovirus infections who was found to have widely metastatic kinase-negative anaplastic large-cell lymphoma. This case demonstrates the diversity in the malignant presentation of HIV-infected patients, outside of the more commonly observed non-Hodgkin lymphomas.

Tubulocystic carcinoma of the kidney.

Tubulocystic carcinoma (TCC) of the kidney is a unique, rare, and recently recognized neoplasm. Although originally considered a low-grade collecting duct carcinoma, TCC is now considered to be a distinct entity. TCC should be considered in the differential diagnosis of cystic renal neoplasms. We report a case of TCC arising in the left kidney.

Undifferentiated Intimal Sarcoma of the Inferior Vena Cava with Extension to the Right Atrium and Renal Vasculature.

Primary sarcomas of the great vessels (aorta, pulmonary artery, and inferior vena cava (IVC)) are exceedingly rare. We report a rare case of an undifferentiated intimal sarcoma of the IVC with extension to the right atrium, adrenal, and renal veins. The patient underwent extensive resection, reconstruction of the IVC, and subsequent adjuvant chemotherapy. Patient has tolerated chemotherapy and, at 17 months after resection, the patient remains free of tumor recurrence. Undifferentiated intimal sarcomas remain a rare entity with only five cases of venous undifferentiated intimal sarcomas reported in the literature, two of which occurred in the IVC. Intimal sarcomas tend to carry a poor prognosis with the limited literature available on treatment approaches. Our objective is to highlight this rare entity and possible treatment approach which we utilized. Primary sarcomas of IVC need to be included as part of a complete differential diagnosis in patients with atrial masses or recurrent pulmonary emboli.

Microcystic Stromal Tumor of the Ovary: A Case Report of a Newly Described Ovarian Neoplasm With a ß-catenin (CTNNB1) G34E Mutation.

Microcystic stromal tumor of the ovary (MSTO) is an exceedingly rare, unusual, and recently described entity with unique genetic alterations that assist in its diagnosis. We describe the case of a 50-year-old woman who presented with a complex right ovarian mass. A hysterectomy with bilateral salpingo-oophorectomy was performed and revealed an ovarian mass consistent with MSTO by histomorphology and immunohistochemical studies. Tumor cells were immunohistochemically reactive for vimentin, CD10, ß-catenin, and Wilms tumor 1. In addition, we detected a missense mutation c.101 G>A, p.G34E in exon 3 of the ß-catenin (CTNNB1) gene, which leads to an amino acid substitution of glycine at codon 34 by glutamic acid. The utility of genetic testing of this tumor and additional reporting of alterations detected is needed to verify pathogenicity of variants detected, as well as their potential roles with prognosis, behavior, and therapeutic targets. The overall clinical course of MSTO appears to be nonaggressive, although the number of reported cases are limited thus far.

A case series of clinically undiagnosed hematopoietic neoplasms discovered at autopsy.

OBJECTIVES: In the United States, autopsy rates have diminished to less than 5% during the last half of the 20th century and the beginning of the 21st century for a multitude of reasons. Many believe this results in unrecognized malignancies that could have explained a patient's death. METHODS: We describe six deaths in which hematopoietic neoplasms were identified at autopsy but were not diagnosed clinically. RESULTS: The six undiagnosed hematopoietic malignancy cases discovered at autopsy include four men and two women ranging from 50 to 78 years of age. One patient was African American and five patients were white, all with multiple comorbidities. The tumors included diffuse large B-cell lymphoma, activated B-cell type, intravascular large B-cell lymphoma, ALK-negative anaplastic large cell lymphoma arising in a setting of human immunodeficiency virus, and a myeloid sarcoma. CONCLUSIONS: These cases illustrate the importance of the traditional postmortem examination in not only confirming clinical diagnoses but also identifying previously unknown diagnoses. Hematologic malignancies may present with nonspecific clinical manifestations, and this series of cases also emphasizes the necessity for widening the differential diagnosis in patients with unexplained lactic acidosis and hepatic failure to include hematopoietic malignancies since prompt treatment may be lifesaving.

Mixed epithelial and stromal tumors of the kidney discovered incidentally at autopsy.

Mixed epithelial and stromal tumors (MEST) of the kidney are uncommon neoplasms that were added to the World Health Organization's renal tumor classification in 2004. These entities are biphasic and contain both epithelial and mesenchymal components. MEST most commonly occur in women. Presented are two cases of MEST incidentally discovered at autopsy.

Sickle cell trait as a contributory cause of death in natural disease.

Sickle cell trait (SCT) affects 300 million people globally, and awareness is growing that SCT is not an entirely benign condition; however, most reported cases have been non-natural deaths. Autopsy records from the Baylor University Medical Center (BUMC) in Dallas, Texas, contained seven natural deaths from January 2007 to October 2013 in which micro-occlusive sickling was identified at autopsy and SCT confirmed by postmortem hemoglobin fractionation. Sickle crisis was never diagnosed clinically. These cases illustrate the importance of red cell morphology in autopsy material. When sickling is suspected, hemoglobin fractionation should be performed. If confirmed, SCT should be listed as an autopsy finding and the severity and distribution of sickling documented. Extensive micro-occlusive sickling should be considered contributory to death; however, its relative importance depends on all facts of the case. Accurate reporting should facilitate further research and the development of evidence-based preventative and supportive strategies for these patients.

Hepatic angiosarcoma associated with disseminated intravascular coagulation.

We present a case of hepatic angiosarcoma that presented with disseminated intravascular coagulopathy to highlight the difficulty in diagnosing this disease due its aggressive clinical course, the overlapping features of various coagulopathies, and the nonspecific appearance of angiosarcomas on imaging.

Eosinophilic variant of chromophobe renal cell carcinoma.

Chromophobe renal cell carcinoma is a distinct subtype of renal cell carcinoma that accounts for 5% of all renal tumors. This subtype is further subdivided into two variants, classic and eosinophilic, with the latter variant being less frequent. We report two cases of the eosinophilic variant of chromophobe renal cell carcinoma diagnosed at our institution between January 2008 and December 2012.

Clear cell carcinoma of the ovary associated with increased risk of venous thrombosis.

Clear cell carcinoma of the ovary (CCCO) is an uncommon, clinically aggressive neoplasm that has a propensity for the development of venous thrombosis and embolization, especially when compared to other subtypes of ovarian malignancies. We present a fatal case of a 59-year-old woman with a clinical course complicated by venous thrombosis and pulmonary thromboembolism that was attributed to CCCO discovered initially at autopsy.

Malignant rhabdoid tumor of the kidney arising in an adult patient.

Malignant rhabdoid tumors (MRT) of the kidney are rare in children and even less common in adults, with only six previously reported adult cases. We present the case of a 60-year-old man with an MRT arising in the left kidney with extensive pulmonary micrometastases and thromboembolism resulting in thrombotic pulmonary microangiopathy (pulmonary tumor embolism syndrome). MRT is an extremely aggressive neoplasm with a short survival time.

Sudden Death by Pulmonary Thromboembolism due to a Large Uterine Leiomyoma with a Parasitic Vein to the Mesentery.

The pathophysiology of venous thrombosis is classically attributed to alterations in one or more components of Virchow's triad: hypercoagulability, stasis, and damage to the vascular endothelium. Deep vein thrombosis (DVT) may lead to pulmonary thromboembolism (PE), and the latter is culpable for many deaths annually in the United States; however, DVT as a complication of uterine leiomyoma has rarely been reported. We report a case of a 57-year-old woman whose death was due to a large pedunculated subserosal leiomyoma externally compressing the pelvic veins resulting in stasis and venous thrombosis leading to fatal PE. The association of large pelvic masses with venous thrombosis has clinical implications, since prophylactic surgery could be life-saving.

Isolated atrial amyloidosis and the importance of molecular classification.

Amyloid is identified microscopically as an amorphous extracellular hyaline material that exhibits "apple-green" birefringence with Congo red stains. Amyloid is not a chemically distinct entity, and currently available molecular methods are capable of identifying over 20 amyloidogenic precursor proteins. Some of the more common diseases associated with amyloidosis include plasma cell dyscrasias, chronic inflammatory disorders, hereditary-familial mutations involving transthyretin, Alzheimer's disease, and so-called "senile" or age-related amyloidosis. The amyloid deposits in these various diseases may be isolated to a single organ such as the heart or brain, or the amyloidosis may be systemic. The senile types of cardiac amyloidosis can result from overproduction of atrial natriuretic factor or from accumulation of otherwise normal or wild-type transthyretin. We present the case of an 83-year-old hospitalized woman with known atrial fibrillation and previous pacemaker implantation who had cardiac arrest unresponsive to attempted resuscitation. Autopsy disclosed prominent amyloidosis involving the left atrium, and subsequent molecular studies identified the amyloidogenic material as alpha atrial natriuretic factor. Since the clinical management and genetic implications of the various diseases associated with amyloidosis are markedly different, we stress the importance of molecular classification whenever possible.