Spontaneous necrotizing granuloma of the cerebellum: a case report.

BACKGROUND: Intracranial necrotizing granulomatous space-occupying lesions are sparsely reported in literature. Variability in presenting symptomatology and radiographic features makes diagnostic work-up difficult. CASE PRESENTATION: This report presents the case of a 77-year-old female with sinusitis and fatigue who underwent an MRI revealing a posterior fossa lesion compressing the fourth ventricle. Subsequent contrast CT of the chest, abdomen, and pelvis was negative for primary malignancy. Histopathologic examination of the lesion following biopsy showed it to be a necrotizing granuloma in an antineutrophil cytoplasmic antibody (ANCA) negative patient. The most likely diagnosis was determined to be spontaneous necrotizing granuloma, a rare entity with only one previous report noted. CONCLUSIONS: Spontaneous necrotizing granuloma of the CNS is a rare entity that represents an important differential consideration in the work-up of space occupying lesions of the CNS.

Sino-orbital osteochondroma with malignant transformation to osteosarcoma.

Purpose: Osteosarcoma is an aggressive malignant osteogenic tumor that commonly arises in long bones of pediatric populations. Primary osteosarcomas of the head and neck are rare, comprising less than 0.5% of malignancies in this region, usually affecting the mandible or maxilla. Here we present an extraordinary case of a rare benign osteochondroma of the ethmoid sinus and bilateral orbits evolving to an intermediate grade osteosarcoma. Observations: An 80-year-old woman with a history of right orbital tumor resection 20 years ago presented to our clinic with right eye proptosis and palpable bony prominence of the right orbit and nasal bridge. Partial resection demonstrated sino-orbital osteochondroma. Relapse a year later prompted repeat partial resection with unchanged histology. The patient was followed clinically until an abrupt relapse four years after initial presentation. Imaging demonstrated a large bony mass involving the right orbit, ethmoid and frontal sinuses, and anterior cranial fossa. Repeat debulking confirmed transformation to intermediate grade osteosarcoma. Conclusions: Osteochondroma is an extremely rare tumor in the orbit with only three cases previously reported. This patient is the first known case of benign osteochondroma of the orbit undergoing malignant transformation to osteosarcoma. Rapid progression of orbital osteochondroma should raise the suspicion of malignant transformation to osteosarcoma and prompt biopsy. Our patient subsequently underwent palliative radiation treatment and is stable with no gross progression.

Case Report: Metastatic Bronchopulmonary Carcinoid Tumor to the Pineal Region.

Intracranial spread of a systemic malignancy is common in advanced staged cancers; however, metastasis specifically to the pineal gland is a relatively rare occurrence. A number of primary lesions have been reported to metastasize to the pineal gland, the most common of which is lung. However, metastasis of a bronchial neuroendocrine tumor to the pineal gland is a seldom-reported entity. Here, we present a 53-year-old female who presented with worsening headaches and drowsiness. MRI brain revealed a heterogeneously enhancing partially cystic mass in the pineal region. The patient had an extensive oncologic history consisting of remote stage IIA invasive breast ductal carcinoma as well as a more recently diagnosed atypical bronchopulmonary neuroendocrine tumor with lymph node metastases. She underwent microsurgical volumetric resection of the large pineal mass and a gross total removal of the tumor was achieved. Histopathology confirmed a metastatic tumor of neuroendocrine origin and the immunohistochemical profile was identical to the primary bronchopulmonary carcinoid tumor. Eight weeks after surgery, she underwent stereotactic radiosurgical treatment to the resection cavity. At 1-year follow-up, the patient remains clinically stable without any new focal neurological deficits and without any evidence of residual or recurrent disease on postoperative MRI. Metastatic neuroendocrine tumors should be considered in the differential diagnosis of pineal region tumors and aggressive surgical resection should be considered in selected patients. Gross total tumor resection may afford excellent local disease control. We discuss the relevant literature on neuroendocrine tumors and current treatment strategies for intracranial metastases of neuroendocrine origin.

Pineal Gland Metastasis From Poorly Differentiated Carcinoma of Unknown Primary Origin.

Pineal metastasis is an exceedingly rare finding in patients with systemic malignancies. Such lesions are typically the manifestation of a primary lung cancer; nonetheless, a variety of malignancies have been reported to disseminate to the pineal gland including gastrointestinal, endocrine, and skin cancers, among others. However, to our knowledge, pineal gland metastasis without a primary origin has yet to be described. Carcinoma of unknown primary origin is a heterogeneous group of cancers characterized by the presence of metastatic disease without an identifiable primary tumor on metastatic workup. Here, we present a case of a 65-year-old male found to have a heterogeneously enhancing lesion of the pineal gland as well as an enhancing lesion of the left cerebellar hemisphere. Comprehensive metastatic workup demonstrated multifocal metastatic adenopathy without an identifiable primary lesion. Stereotactic biopsy of the pineal lesion revealed poorly differentiated carcinoma with an immunophenotype most consistent with gastrointestinal origin. To our knowledge, this is the first case to describe a pineal gland metastasis without a primary origin. We discuss the relevant literature on pineal gland metastases as well as carcinoma of unknown primary origin.

A plasma cell-based pericardial effusion leading to tamponade in a patient with multiple myeloma - a case report and review of the literature.

A rare case of extramedullary multiple myeloma causing cardiac tamponade secondary to a plasma cell-based pericardial effusion is described. A systematic search using PubMed (National Library of Medicine) was used to identify a further 27 cases dating back to 1970. Case characteristics, treatment strategies, and survival time following tamponade are discussed. Linear regression demonstrated a weak but statistically significant correlation between survival time following tamponade and treatment with systemic chemotherapy and steroids (ß=16.8 weeks, P=.009). However, this manifestation of extramedullary multiple myeloma still conveys a dismal prognosis with a median survival following tamponade of only 6 weeks based on our review.

Improving the pathologic evaluation of lung cancer resection specimens.

Accurate post-operative prognostication and management heavily depend on pathologic nodal stage. Patients with nodal metastasis benefit from post-operative adjuvant chemotherapy, those with mediastinal nodal involvement may also benefit from adjuvant radiation therapy. However, the quality of pathologic nodal staging varies significantly, with major survival implications in large populations of patients. We describe the quality gap in pathologic nodal staging, and provide evidence of its potential reversibility by targeted corrective interventions. One intervention, designed to improve the surgical lymphadenectomy, specimen labeling, and secure transfer between the operating theatre and the pathology laboratory, involves use of pre-labeled specimen collection kits. Another intervention involves application of an improved method of gross dissection of lung resection specimens, to reduce the inadvertent loss of intrapulmonary lymph nodes to histologic examination for metastasis. These corrective interventions are the subject of a regional dissemination and implementation project in diverse healthcare systems in a tri-state region of the United States with some of the highest lung cancer incidence and mortality rates. We discuss the potential of these interventions to significantly improve the accuracy of pathologic nodal staging, risk stratification, and the quality of specimens available for development of stage-independent prognostic markers in lung cancer.