A Rare Case of a Primary Spinal Solitary Fibrous Tumor/Hemangiopericytoma in a 9-Month-Old Patient.

BACKGROUND Solitary fibrous tumors (SFTs)/hemangiopericytomas (HPCs) are mesenchymal tumors commonly found in middle-aged patients, usually localized to thoracic pleurae. Spinal tumor involvement is rarely seen, and its imaging findings are largely inconsistent because of the rarity of these cases. We present a case report of a 9-month-old girl with a rare intraspinal tumor with histologic evidence of SFT/HPC, but no STAT6 nuclear immunoreactivity. CASE REPORT A 9-month-old girl, born at term with good prenatal care, presented to the emergency room with regression of developmental milestones. The patient was in good health until 2 months, when she developed decreased spontaneous leg movements. Physical exam revealed diffuse muscular atrophy, with no deep tendon reflexes, sensation, or spontaneous movements of the lower extremities. Computed tomography and magnetic resonance imaging showed a heterogeneous irregular mass filling the lumbosacral spinal canal, extending through the neural foramina to the prevertebral/perivertebral and presacral regions. The tumor was biopsied and referred to the National Institutes of Health for consultation and the diagnosis of SFT/HPC was confirmed on the basis of its histologic features, despite the fact that the tumor was negative for STAT6 immunoreactivity. CONCLUSIONS Although the tumor histology was consistent with SFT/HPC, it was negative for STAT6 nuclear immunoreactivity, which is unusual and may exclude the diagnosis. To our knowledge, this is the youngest patient to present with a spinal SFT with these features.

Leptomeningeal Carcinomatosis as the Initial Manifestation of Metastatic Disease diagnosed in Postmortem Examination: A Case Series.

Leptomeningeal carcinomatosis (LMC) refers to the infiltration of malignant cells in the pia-arachnoids. LMC is undiagnosed until autopsy in about 20% of cases. A nonspecific neurologic symptomatology makes diagnosis challenging; especially in the scenario of unknown malignancy. Diagnosis is made by the identification of malignant cells in CSF; though studies have shown that serial examination may be required for acceptable accuracy. We report 3 cases with distinct neurological presentations, negative cerebrospinal fluid (CSF) examinations and neurological imaging. A 52 year old woman with history of breast cancer on remission, a 2 year old male with left ear rhabdomyosarcoma status post resection, and a 59 year old woman with communicating hydrocephalus of unknown etiology. LMC was diagnosed at autopsy and confirmed by immunohistochemistry. LMC is a complication requiring a high level of clinical suspicion. Postmortem examination is an invaluable tool to confirm LMC as part of the multidisciplinary approach aiming towards the improvement of clinical diagnosis.

Intravitreal rituximab for the treatment of a secondary intraocular relapse of a large B-cell lymphoma.

PURPOSE: To report a rare case of secondary intraocular lymphoma treated with intravitreal rituximab, following pars plana vitrectomy. OBSERVATIONS: A 74-year-old female with history of parotid gland large B-cell lymphoma presented bilateral intraocular recurrence 10 years after the onset of the primary malignancy. Systemic work-up including PET/CT Scan, bone marrow biopsy, brain MRI and CSF analysis were unremarkable, and the patient declined to undergo systemic chemotherapy. Vision loss in her left eye was severe due to significant sub-retinal pigment epithelium (RPE) infiltration involving the macula; this eye was treated with external beam radiation therapy. On the right eye, the relapse manifested with vitreous involvement and fovea-sparing multifocal, sub-RPE infiltration for which the patient received monthly intravitreal rituximab injections, following pars plana vitrectomy. Through the course of therapy, the patient achieved good local control and maintained 20/20 visual acuity on her right eye. Brain magnetic resonance imaging (MRI) surveillance, every 3 months, was performed and revealed a cerebellar recurrence 24 months into the course of therapy. CONCLUSIONS AND IMPORTANCE: Our case illustrates how intravitreal immunotherapy with rituximab may provide local control of CD-20 positive secondary intraocular lymphoma; particularly in cases where systemic therapy is not amenable. In our case, a prior vitrectomy, did not appear to interfere with the therapeutic effect of intravitreal rituximab. Close quarterly surveillance with Brain MRI may help disclose central nervous system recurrences in such cases.

A hematologic condition expressed as a lung disease.

Pleural involvement secondary to Multiple Myeloma is considered a very rare complication. According to the literature only 1% of these patients develop a myelomatous pleural effusion. We present a case of a 39 year old man with multiple myeloma diagnosed six years prior to our evaluation, which developed progressive dyspnea, dry cough and right pleuritic chest pain two weeks prior to admission. On physical examination the patient had decreased breath sounds over the right posterior hemithorax accompanied by dullness to percussion. The chest radiogram was consistent with a right sided pleural effusion. Pleural fluid analysis revealed the presence of abundant abnormal plasma cells. The patient died four weeks after hospitalization. The presence of myelomatous pleural effusion is considered to be a poor prognostic finding, no matter at what disease stage it develops. So far no definite treatment has been shown to improve survival.

Primary endobronchial anaplastic large cell lymphoma in a pediatric patient.

The authors describe a pediatric patient who presented with a 3-month history of dry cough, chest pain, progressive breathlessness, fever and recurrent pneumonia with atelectasis. A fiberoptic bronchoscopy revealed a whitish lesion at the left bronchus. A biopsy of the lesion demonstrated an anaplastic large cell lymphoma (ALCL). Evaluation for disseminated disease was negative. After the patient completed chemotherapy the lesion abated and she has been in complete remission for almost 4 years. Although extranodal involvement of ALCL is frequent at some stage of the disease, endobronchial involvement is extremely rare even in the presence of advanced disease. To our knowledge, this is the first primary isolated endobronchial ALCL described in a pediatric patient.

The contribution of immunohistochemistry in the diagnosis of pediatric neoplastic lesions in the University of Puerto Rico, School of Medicine

Immunohistochemistry has revolutionized the field of diagnostic pathology in the past fifteen years. Since then, it has been increasingly used as an adjunct to morphological diagnosis. The purpose of this retrospective analysis is to examine the value of this technique in the diagnosis of pediatric neoplasias in our laboratory. Forty eight pediatric cases, collected from January 1998 until May 1999, were reviewed, and classified in one of four categories: confirmed the morphological diagnosis, provided the definite diagnosis from a list of probable diagnoses, contributed by excluding other entities, and non-contributory. Immunohistochemistry confirmed the morphological diagnosis in 29 cases (60.4), provided the definite diagnosis from a list of probable diagnoses in 13 cases (27.1), was contributory by exclusion of other entities in 2 cases (4.2), and was non-contributory in 4 cases (8.3). In this preliminary study, we conclude that immunohistochemistry is being used in our laboratory mostly as a confirmatory tool for the definitive diagnosis of the lesions and once more exalts the utility of this technology in the field of diagnostic pathology.

Immediate transfusion reactions

Immediate transfusion reactions were characterized in recipients of 15,990 RBCs, 18,013 platelets, 409 single donor platelets, 3,451 FFP and 1,507 units of cryoprecipitate. The overall incidence of immediate reactions was 0.2. Allergic reactions occurred in 40 patients, febrile non-hemolytic in 36 patients, bacterial contamination in 2 patients, circulatory overload in 3 patients and mechanical hemolysis in one patient. Three patients had nonspecific reactions. The incidence of immediate transfusion reactions was low when compared with similar studies. Possible causes are under-reporting transfusion reactions and the use of leukoreduced components.

Evaluation of serologic markers for transfusion trasmitted infectious diseases for allogeneic blood donors in Puerto Rico

The purpose of this project is to assess the prevalence of serologic markers for transfusion transmitted infectious disease in allogeneic blood donors of the American Red Cross Blood Services (ARCBS) in Puerto Rico. Four hundred records were randomly selected from a population of 7718 first time volunteer donors from the ARCBS in P.R. covering the period from Jan. 1st to Jun. 30th, 1991. Variables obtained were: age, sex, presence of hepatitis B surface antigen (HBsAg), hepatitis B anti-core antibody (anti-HBc), hepatitis C virus antibody (anti-HCV), HIV 1/2 antibody, HTLV I/II antibody, RPR reactivity and ALT. The prevalence of serologic markers in our study is consistently higher than that found in similar studies in the U.S. population, except for HBsAg and HCV. This could be explained with the use of data from only first time volunteer donors since the prevalence is higher in this group than in repeat donors. None of the donors in this sample were positive for HBsAg probably due to the small sample. The prevalence of anti-HCV in this study is within the values found for the U.S. population.