Primary anaplastic glio-neuronal tumor of the pineal gland: a new type of pineal neoplasm?

BACKGROUND: Tumors of the pineal gland are relatively rare. Most are solid, infiltrative lesions characterized by either germ cell, glial, or pineal parenchymal origin. To our knowledge, none have been described that contain an admixture of both anaplastic glial and neuronal elements. CASE REPORT: The authors present a case of a primary anaplastic glio-neuronal tumor of the pineal gland in a 78-year-old gentleman who presented with hydrocephalus. The patient underwent an endoscopic third ventriculostomy for treatment of his hydrocephalus as well as tumor biopsy under endoscopic visualization. RESULTS: Sections of the tumor displayed a modestly to moderately cellular neoplasm infiltrating the residual pineal gland. The majority of the nuclei appeared hyperchromatic, spindle-shaped, and pleomorphic, with fibrillary cytoplasmic processes. Rare nuclei were round with vesicular chromatin and red nucleoli. Mitotic figures were easily identified, despite small sample size. GFAP immunostaining highlighted the fibrillar background and cytoplasmic processes while synaptophysin immunostains revealed intense, dot-like positivity in the rounder nuclei with vesicular chromatin. Although the different nuclear morphologies seen on the hematoxylin-eosin stained slides suggested both glial and neuronal origin, immunostains were required to confirm the presence of dual differentiation. CONCLUSION: We present the first case of a primary anaplastic glio-neuronal neoplasm of the pineal gland. Increased use of immunostaining may unveil additional cases in the future. Furthermore, additional research will be required to determine whether the dual differentiation has prognostic significance.

Adenomatoid tumor of the pancreas: a case report with comparison of histology and aspiration cytology.

We present a 58-year-old woman who presented with a 1.5-cm, hypodense lesion in the head of the pancreas. Endoscopic ultrasound-guided fine-needle aspiration yielded bland, monotonous cells with wispy cytoplasm, slightly granular chromatin, and small nucleoli. A presumptive diagnosis of a neuroendocrine lesion was rendered. Whipple procedure yielded a well-circumscribed, encapsulated lesion with dense, hyalinized stroma and a peripheral rim of lymphocytes. Spindled and epithelioid cells formed short tubules, cords, and nests. The neoplasm stained for CK 5/6, calretinin, vimentin, CD 99, pancytokeratin, and EMA, consistent with mesothelial origin. This characteristic histology and immunohistochemistry is consistent with an adenomatoid tumor. We believe we are the first to report this benign neoplasm in such an unusual location. Herein we address the diagnosis of adenomatoid tumor by histology, immunohistochemistry, and aspiration cytology. Our case is particularly unique in that the histology and cytology are compared and correlated.

Secondary amyloidosis and gastrointestinal stromal tumors. A case report and discussion of pathogenesis.

A 69-year-old man presented with a malignant gastrointestinal stromal tumor associated with secondary amyloidosis. The tumor had classic features of a malignant gastrointestinal stromal tumor with interlacing fascicles and whorls of spindled cells, numerous and conspicuous mitotic figures, and extensive coagulative necrosis. The cells stained diffusely for CD117 (c-Kit), confirming the diagnosis of gastrointestinal stromal tumor. The spleen, 1 adrenal gland, and part of the pancreas were removed en block with the stomach. By microscopy, the spleen and adrenal gland were partially replaced with amyloid deposits confirmed by Congo red staining, electron microscopy, and immunohistochemistry. In contrast, neither the tumor nor the surrounding vasculature showed amyloid deposition. To our knowledge, this represents only the second case of systemic amyloidosis associated with a gastrointestinal stromal tumor. This case is unique in that extensive, diffuse amyloid deposits were observed in the spleen, adrenal gland, and liver.

Fine needle aspiration of splenic extramedullary hematopoiesis presenting as a solitary mass. A case report.

BACKGROUND: Extramedullary hematopoiesis (EMH) generally occurs in patients with deficient bone marrow hematopoiesis secondary to either peripheral red cell destruction or marrow replacement. EMH is most commonly seen in the liver and spleen as a diffuse lesion. Rarely EMH presents as a solitary mass, posing a diagnostic dilemma. In asymptomatic patients without obvious evidence of hematopathology, the differential diagnosis is even more complex. Despite the several articles detailing the radiographic findings in EMH, fewer promote the utility of fine needle aspiration (FNA) in making this diagnosis. CASE: EMH presented as a discrete, 6-cm, tumorlike splenic mass, first identified on computed tomography in a patient with a history of prostate carcinoma. FNA findings suggested a diagnosis of EMH, which was confirmed by splenectomy. In searching for the cause of EMH, no evidence of either metastatic or hematologic disease was identified. The cause of EMH in this patient remains unknown. To our knowledge, this is the first case of solitary splenic EMH reported to occur in an asymptomatic patient. CONCLUSION: FNA is a useful adjunct in the evaluation of isolated splenic masses, particularly in the diagnosis of EMH, where hematopoietic precursors are readily identified cytologically. We advocate the use of FNA in the management of splenic masses. In addition, our case also suggests that EMH be included in the differential diagnosis of isolated splenic lesions, even in patients without obvious hematologic disorders.

The role of placental pathology in the evaluation of interpersonal violence: a case of abdominal gunshot wound in a 27-week gravid uterus.

We present a 17-year-old G1P0 Asian American woman with a previously undiagnosed pregnancy who sustained an intra-abdominal gunshot wound at 27 weeks' gestation. Within 2 hours of the traumatic event, the victim was taken emergently to the operating room for exploratory laparotomy. Findings included a gravid uterus with two entrance wounds and two small exit wounds with active bleeding from the right broad ligament. The fetus was bradycardic but viable, having suffered a gunshot wound to the left shoulder. Evaluation of the placenta revealed no sequelae from the acute event. Unexpectedly, two older, green, 7.0 cm retromembranous hematomas were present, both ringed by hemosiderin-laden macrophages. These hemorrhages clearly preceded the acute event. Although these findings seemed suspicious for a history of prior abuse or trauma, corroborative clinical data were unavailable at the time of initial placental evaluation. However, days later, the victim admitted to a history of interpersonal violence, with previous abuse from her boyfriend, a fatal victim of the same attack. The old retroplacental hemorrhages proved to be the only physical documentation of her previous abuse.

Fatal progressive hepatic necrosis associated with lamotrigine treatment: a case report and literature review.

We present a case of fatal, progressive hepatotoxicity in a patient treated with lamotrigine. After presenting with a rash and fever, she developed elevated liver function tests and clinical sequelae of hepatic failure. The subacute course of her progressive liver damage is documented in serial liver biopsies. While her initial biopsy showed approximately 50% hepatocyte necrosis, her post mortem examination performed three weeks later displayed massive hepatic necrosis with extensive bile duct proliferation. Although she was taking other antipsychotics at the time, her clinical course best parallels other reports of lamotrigine-associated hepatotoxicity. Here we discuss not only the clinicopathologic findings of this case but also review the pertinent literature.

Congenital nephrosis of the Finnish type: overview of placental pathology and literature review.

Congenital nephrosis of the Finnish type (CNF) is a rare, autosomal recessive disorder of glomerular filtration that results in massive proteinuria, edema, and ascites. Although previous studies describe the classic renal lesions characterizing this disorder, there are few documenting in detail the associated placental alterations. In this context, we present a case of CNF with emphasis on the placental pathology and compare our findings to what has been previously reported in the literature. A 36-year-old G2P1 with no significant medical history developed persistently elevated amniotic fluid alpha-fetoprotein in the absence of neural tube defects. Because of a clinical suspicion of CNF, she electively terminated the pregnancy at 19 weeks. Postmortem examination revealed characteristic renal changes, confirmed by electron microscopy, as well as significant placental villous edema. Although the placenta was not enlarged, the villi appeared profoundly hydropic. Extensive cystic vacuolar change was documented in both stem villi and tertiary villi, affecting 95% of the villi present. Since the fetus was not grossly edematous, the placental findings may represent the first sign of systemic hypoproteinemia.