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Ganglioneuroma in the Adrenal Gland of a Rat.

Necropsy was performed on a 22-month-old male Fischer 344 rat (Rattus norvegicus) that had weight loss and was icteric. Examination revealed a large, smooth, circumscribed, tan mass located at the cranial border of the right kidney. Microscopically, the mass consisted almost entirely of well-differentiated ganglion cells mixed with satellite and Schwann cells within a neurofibrillar matrix. The cells displaced the adrenal medulla and were partially surrounded by a thin rim of compressed adrenal cortex. Focal areas of hemorrhage and infiltrates of leukemic mononuclear cells were evident throughout the mass, but pheochromocytes were not detected. On the basis of findings during histologic examination, a diagnosis of adrenal medullary ganglioneuroma was made. Mononuclear cell leukemia was also diagnosed in the adrenal gland and was evident in lymphoid organs such as the spleen and mesenteric lymph nodes. Ganglioneuroma is rarely seen in rats and can be differentiated from more common complex pheochromocytomas, which consist of fewer neural components (< 80% of the mass). In rats, ganglioneuromas can develop in the central nervous system, pituitary gland, and thyroid gland. Differential diagnoses for a primary neoplasm of the adrenal gland should include carcinomas and adenomas of the adrenal cortex, and pheochromocytomas, neuroblastomas, and ganglioneuromas of the adrenal medulla.

Journal Article↗

Leukemic dissemination within a glioblastoma in a patient with chronic lymphoid leukemia.

Metastasis from an extracranial tumor to a primary central nervous system tumor is a rare event, and most reported cases concern metastases to meningiomas. The authors describe the first case of leukemic cell dissemination within a glioblastoma. The patient likely presented a genetic predisposition to multiple neoplasms, and the unusual localization of leukemic cells might be partly related to the characteristic microvascular proliferation in glioblastoma.

Brain↗

MR imaging of autopsy-proved paraneoplastic limbic encephalitis in non-Hodgkin lymphoma.

We report the case of a 26-year-old man with precursor T-cell acute lymphoblastic leukemia who developed paraneoplastic limbic encephalitis that was diagnosed on the basis of MR imaging findings and was proved post mortem. In our MR imaging studies, fluid-attenuated inversion recovery images and diffusion-weighted echo-planar images clearly depicted bilateral involvement of the medial temporal lobes and multifocal involvement of the brain, whereas T2-weighted turbo spin-echo images failed to show the changes.

Adult↗

[Portal hypertension caused by intra-hepatic block during chronic lymphoid leukemia].

Portal hypertension in chronic lymphocytic leukemia is rare. A 66 year-old man was admitted for splenomegaly, thrombopenia and cholestasis. Endoscopy showed esophageal varices. The hepatic venous pressure gradient was 15 mmHg. The liver biopsy showed dense leukemia cells in sinusoidal and portal sites. After splenectomy, the hepatic venous pressure gradient normalized, but esophageal varices and cholestasis persisted. The authors discuss the mechanisms of portal hypertension in chronic lymphocytic leukemia. Previously reported cases are summarized.

Aged↗

[Improvement of diabetes insipidus after allogeneic bone marrow transplantation in a patient with myelodysplastic syndrome].

A 45-year-old male was diagnosed as having myelodysplastic syndrome (RAEB) in March 2001. He was admitted to our hospital because of cellulitis in his left lower limb in May. The blood cell count showed pancytopenia and immature myeloid cells were seen in the peripheral blood. Bone marrow aspiration showed the proliferation of myeloblasts (11.2%) and complex karyotypic abnormalities were detected including the long arm deletion of chromosome 7. The patient developed polyuria and polydipsia after admission and was diagnosed as having central diabetes insipidus. He was treated with DDAVP and the polyuria disappeared. In November, he underwent unrelated allogeneic bone marrow transplantation after conditioning with total body irradiation and cytarabine. After transplantation DDAVP was no longer required. Central diabetes insipidus has been reported as a rare complication of leukemia or myelodysplastic syndrome, but the underlying mechanism remains unclear. The complete remission of diabetes insipidus after bone marrow transplantation suggests that the infiltration of leukemic cells into the pituitary gland caused the diabetes insipidus in this case.

Bone Marrow Transplantation↗

[Blastic natural killer cell leukemia--one case report and review of literature].

OBJECTIVE: To report a case of blastic natural killer cell leukemia with an aggressive clinical course. METHODS: The characteristics of blastic NK cell leukemia and its treatment were discussed with review of literatures. RESULTS: After combination chemotherapy and spinal cord segmental radiotherapy, the patient entered hematological remission, but the extramedullary lesion remained unchanged. CONCLUSION: Blastic NK cell leukemia has an aggressive clinical course with poor response to treatment and unfavorable prognosis.

Adult↗

Acute leukostasis pulmonary distress syndrome.

A 75-year-old woman in accelerated-phase chronic myeloid leukemia with hyperleukocytosis presented with acute respiratory distress syndrome. Despite early and aggressive pulmonary support and cytoreductive chemotherapy, the patient died. Autopsy confirmed the presence of the leukostasis syndrome. The clinical, radiologic, pathophysiologic, and therapeutic aspects of this entity are reviewed.

Acute Disease↗

[Renal and electrolyte disturbances in chronic myelogenous leukemia].

Renal and electrolyte disturbances in 91 patients with chronic myelogenous leukemia (CML) were analyzed over a period of these twenty years. At diagnosis, renal and electrolytes were studied in 72 patients including 65 in chronic cases, 5 in accelerated phase and 2 in blastic crisis. There were 8 cases of hypocalcemia among 62 patients and 5 cases of hyperphosphatemia among 48 patients. The cases of hyperphosphatemia and renal dysfunction had short median survival. There were no significant differences of renal and electrolyte disturbances between before and after chemotherapy. Various electrolyte disturbances, that is, hyponatremia, hypo-, hyperkalemia, hypocalcemia, hypo-, hyperphosphatemia, were found in the blastic crisis of CML. In the last admission, renal dysfunction and various electrolyte disturbances were present in almost half of the cases. Pathological studies were performed in 18 autopsy cases. Acute tubular insufficiency or necrosis, hypercalcemic nephropathy, and renal infiltration of leukemic cells were recognized in patients who had renal dysfunction.

Adolescent↗

A comparison of neuropsychological and psychosocial functioning after prophylactic treatment for childhood leukemia in monozygotic twins.

Outcome findings based on a comprehensive battery of neuropsychological and psychosocial measurements were compared for a set of monozygotic twins. One twin had been diagnosed with acute lymphoblastic leukemia and given prophylactic treatment involving intrathecal methotrexate. Her twin sibling, who developed no signs of the disease, served as a unique control. Remarkably similar profiles were noted for the twins on tests of nonverbal intelligence, visual memory, visual attention, psychomotor speed, and mental flexibility. All performances were in the average to high average range. Significant differences were found on tasks measuring verbal abstract reasoning skills. These differences were postulated to result from prophylactic treatment, leukemia itself, or disruption in normal psychosocial development. As in previous studies, problems with auditory attention were found. However, both children displayed attentional difficulties regardless of treatment status. Emotional assessment indicated that both twins were experiencing a clinically significant level of anxiety that was postulated to play a role in reduced attention skills. Findings argue for the continued need for monitoring the neuropsychological functioning of children given prophylactic treatment and demonstrate the importance of measuring emotional factors in assessment with these children.

Affective Symptoms↗

Treatment options for newly diagnosed patients with adult acute lymphoblastic leukemia.

The treatment of adults with acute lymphoblastic leukemia (ALL) has been modeled on therapy developed for childhood ALL. The similarity between childhood and adult forms of this disease allows for inferences to be drawn from experience in the pediatric population. However, adults with ALL have far poorer outcome when compared to children. Some of this difference can be attributed in ability to tolerate intensive therapy coupled with an increased incidence of unfavorable cytogenetic subgroups and a decreased incidence of favorable cytogenetic subgroups. Treatment of adult ALL is typically divided into four broad categories: induction, consolidation, maintenance, and central nervous system prophylaxis. Despite three decades of clinical investigation for devising improved treatment programs for adults with ALL, no single program has emerged as the standard. Here we review classification, prognostic features, current treatment programs, and new advances as applied to adult patients with newly diagnosed ALL.

Adult↗

Cavitating Pneumocystis pneumonia in an autopsied case of adult T-cell leukemia.

Cavitating necrosis is rare in Pneumocystis carinii pneumonia. In this report, we describe an autopsy patient with adult T-cell leukemia associated with cavitating Pneumocystis carinii pneumonia. The patient, a 61-year-old woman, died of an acute crisis of adult T-cell leukemia associated with diffuse pulmonary infection of Pneumocystis carinii. Postmortem examination revealed necrotic foci in both lungs, one of which, in the left lower lobe, had a central cavitation. Microscopically, leukemic cell infiltration was abundant in the lung parenchyma but not in the necrotic lesions. Pneumocystis carinii organisms were distributed diffusely in the alveoli and also in the cavity wall. Intranuclear and intracytoplasmic inclusion bodies were scattered in the lung indicating cytomegalovirus infection. However, no bacterial or fungal infection was detected in the lungs, even in the necrotic lesions. Cavitating Pneumocystis carinii pneumonia occurs in other immunodeficiency diseases apart from AIDS. To our knowledge, this report is the first case of cavitating Pneumocystis carinii pneumonia in adult T-cell leukemia.

Blast Crisis↗

[CD3-negative natural killer cell leukemia with aggressive clinical course].

The authors report an autopsy case of CD3- large granular cell leukemia with an aggressive clinical course. A 15-year-old male was admitted to our hospital with complaint of high fever. Clinical examination revealed cervical lymphadenopathy and hepatosplenomegaly. His white blood cell count was 7,000/microliters with 45% large granular lymphocytes. A biopsy specimen of the cervical lymph node showed diffuse lymphoma, mixed small and large cells (DM). Surface marker analysis by immunohistochemical technique revealed that neoplastic cells expressed CD2, CD38, CD56 and HLA-DR but lacked CD3, CD4 and CD8. Southern blot analysis of immunoglobulin (Ig) and T cell receptor (TCR) genes showed germ line of Ig and TCR. These findings indicate that this case was a large granular cell leukemia with the natural killer cell phenotype. Despite anti-leukemic therapy, he died of hyperkalemia and acidosis. Autopsy showed a marked swelling of the liver (3,122 g) and spleen (2,434 g) with leukemic cell infiltration.

Adolescent↗