Autopsy and Case Reports最新文献

A 31-year-old man presented to the hospital after suffering a sudden cardiac arrest. Despite optimal therapy, the patient passed away. His medical history included febrile rash at age 2. At autopsy, there was aneurysmal dilation and severe coronary artery stenosis by atherosclerotic plaques and myocardial fibrosis. These findings were presumed to be due to complications of Kawasaki disease, given the remote history of severe febrile rash as a toddler and the presence of chronic coronary artery injury, recanalization, and thrombosis with ischemic heart disease leading to sudden cardiac collapse and death.

Obesity is a common chronic disorder and has detrimental long-term consequences if left untreated. Herein, we report a case of a young lady who suffered from morbid obesity and many of its consequences, and we present a literature review of these complications. While the cause of obesity is multifactorial, the genetic component is particularly important in the pathophysiology of marked obesity. Resistance to Leptin is considered one of the main causes of obesity. There is a unique relationship between polycystic ovary syndrome and obesity, as observed in our case. Obesity is associated with cardiovascular and lung diseases such as heart failure, thromboembolic disease, sleep apnea, and pulmonary hypertension. Our patient had cardiomegaly (730 gm) with eccentric hypertrophy of left and right ventricles. The coronary arteries and aorta were free of atherosclerosis, which is a surprising finding that relates to the mysterious phenomenon of obesity paradox. The terminal event in our young woman was multiple segmental and subsegmental pulmonary arterial thrombi/thromboemboli superimposed on chronic cardiopulmonary stress due to massive obesity.

Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

Calcifying fibrous tumor is a rare benign mesenchymal neoplasm. The etiology and pathogenesis of this tumor are uncertain. It has wide anatomical distribution. The tumor is most commonly found in the soft tissues of the extremities in younger individuals. However, in middle-aged patients, it tends to affect the visceral locations more commonly. In visceral location, it can mimic aggressive lesions clinically. The purpose of this report is to describe a case of calcifying fibrous tumor in a 71-year-old female with a history of breast carcinoma who was found to have an incidental small bowel mass on her follow-up. Clinically and radiologically, the mass was suspicious for either metastatic disease or gastrointestinal stromal tumor. The patient underwent open small bowel resection, and a 6.5 cm segment of the small bowel was sent to pathology. Grossly, a 2.0 cm tan-pink smooth round submucosal polyploid mass protruding into the lumen, mimicking a gastrointestinal stromal tumor, was identified. The tumor was hard and serially sectioned to reveal a white, calcified cut surface. Microscopically, the tumor appeared hypocellular and composed of scant spindle cells embedded in a dense, hyalinized and calcified collagenous stroma. Immunohistochemical stains for pan-cytokeratin, DOG1, desmin, S100, CD34, and MUC4 were negative, and a diagnosis of the calcifying fibrous tumor was rendered. This case provides a rare gross specimen image of calcifying fibrous tumor and highlights the importance of knowledge of rare entities in providing an accurate diagnosis for entities that can mimic other lesions.

We report a case of a benign glandular schwannoma in a 63-year-old male who presented with a solitary subcutaneous mass on the left knee, with no previous history of neurofibromatosis type 1. This histological subtype is rare, with only 38 cases reported in the literature. Some of the glands found in this patient resembled sweat glands. These lining stromal spindle cells were positive for S-100 but negative for EMA. S100 was faintly staining the glandular elements. All the glands in the tumor were positive for EMA, particularly at the luminal borders. They were also positive for pancytokeratin. The cystic areas variably show intraluminal, foamy, and hemosiderin-laden macrophages. The different glands expressed two patterns. Some of these were reactive for CK7 and low molecular weight keratin. Immunohistochemical workup is mandatory to assess the neoplastic nature of this glandular component.

Sialolithiasis is a common nonneoplastic disease of the major salivary glands that often affects the submandibular glands. Minor salivary gland involvement by sialolithiasis is uncommon, with only 273 cases reported. A long clinical history, acute symptoms, and mucopurulent discharge are unusual features of these cases. Herein, we report the case of a 63-year-old woman who complained of symptomatic nodular swelling of the buccal mucosa associated with purulent discharge for several days. The clinical history lasted 15 years, with episodes of asymptomatic non-suppurative swelling in the same area. The patient underwent surgical excision. The microscopic examination revealed chronic nonspecific sialadenitis associated with psammomatous calcifications, confirming minor salivary gland sialolithiasis. After 3 years of follow-up, the patient was free of symptoms. Patients with sialolithiasis are usually asymptomatic; however, swelling, pain, and fistula may be present in rare cases. The presence of purulent exudate should lead to the differential diagnosis of stomatitis glandularis, a rare inflammatory condition affecting the minor salivary glands. Sialolithiasis and stomatitis glandularis should be considered in the clinical differential diagnosis of symptomatic suppurative nodular swelling affecting the oral mucosa, and histopathological analysis is necessary for the diagnosis.

Intracranial lipomas are rare benign tumors considered exceptional when localized in the cerebellopontine angle (CPA), with an incidence of 0.1% of the total number of expansive processes located in this area. We present a case of the sudden death of a 26-year-old young woman in which an unencapsulated neoformation of 0.8 cm was documented at the right cerebellopontine angle and was histologically characterized as intracranial lipoma. The cause of death was then identified as a cardiocirculatory failure secondary to supratentorial (uncal right) herniation caused by the lipoma of the pontocerebellar angle with high-grade diffuse cerebral edema.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive hyperinflammatory syndrome in which an inciting event triggers massive, uninhibited activation of T lymphocytes and macrophages. Although viral infections are the most common trigger of HLH, cases of HSV-1 induced HLH are rare in adults. We present the case and postmortem findings of a 27-year-old woman diagnosed with HLH in the setting of immunosuppression for the treatment of granulomatosis with polyangiitis (GPA). Autopsy revealed evidence of herpes simplex virus-1 (HSV-1) infection and no findings suggestive of GPA recurrence.

Lymphoplasmacyte-rich meningioma (LPRM) is one of the rarest variants of grade I meningiomas. It can be clinically associated with prominent peripheral blood abnormalities, anemia, and/or various gammopathy, which usually disappear after surgical removal of the tumor. We document a case of right frontal LPRM in a 72-year-old male who presented general cognitive decadence. The patient suffered from mild anemia. The LPRM is a rare variant of meningioma, with only a few cases globally reported in the literature. It has been categorized as a grade I tumor in the 2021 World Health Organization (WHO) classification central nervous system. Due to the rarity, this meningioma variant origin and biological behavior are still not clear. Immunohistochemistry profile showed prominent PD-L1 expression, leading to additional interrogation on LPRM immunomorphological characteristics, the significance of the inflammatory tumoral microenvironment and its correlation with the immune-checkpoints.