1/51. Cutaneous granulocytic sarcoma mimicking immunoblastic large cell lymphoma. A peculiar case of cutaneous granulocytic sarcoma without leukemic manifestation (so-called aleukemic leukemia cutis) that developed in the skin of the back of a 69-year-old man is reported. A skin biopsy specimen showed atypical cells with a prominent nucleolus proliferating around dermal blood vessels and along adnexa without epidermotropism. Atypical cells similar to those of the skin had infiltrated diffusely into the interfollicular area of an inguinal lymph node. Flow cytometric and immunohistochemical studies with a panel of monoclonal antibodies revealed neoplastic cells that had a biphasic phenotype of myeloid and T cell precursors. They expressed CD13, CD15, CD33, lysozyme, CD3epsilon, CD4, CD7 and terminal deoxynucleotidyl transferase (TdT). Gene analysis showed no rearrangement of the immunoglobulin heavy chain or T cell receptor beta and gamma genes. Ultrastructurally, the tumor cells exhibited a few intracytoplasmic electron-dense granules and well-developed rough endoplasmic reticulum with an occasional whorling arrangement. The initial diagnosis was immunoblastic large cell lymphoma, and the patient was treated with six courses of ProMACE-CytaBOM. In spite of the high-grade cytological characteristics of this tumor, the patient has been free of disease for 5 years. ( info) |
2/51. Perianal lymphoma in a heterosexual and nonimmunocompromised patient: report of a case and review of the literature. PURPOSE: This study was conducted to report a rare case of anorectal pathology. methods: We report a case of perianal lymphoma in a nonimmunocompromised, heterosexual patient and review the literature. RESULTS: A 67-year-old white male was found to have an ulcerated posterior anal mass. biopsy revealed large cell lymphoma, B cell type, immunoblastic. There was no disseminated disease. The patient denied any homosexual activity and was HIV negative. There was complete resolution with chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), and he remained disease free for six years. CONCLUSIONS: Rare anorectal pathology may mimic common conditions. Biopsies should be taken to rule out malignancy. ( info) |
3/51. A patient with bilateral primary adrenal lymphoma, presenting with fever of unknown origin and achieving long-term disease-free survival after resection and chemotherapy. Primary adrenal lymphoma is extremely rare. We describe a 64-year-old female patient who presented with fever of unknown origin. Imaging studies demonstrated bilateral bulky adrenal masses. She underwent bilateral adrenalectomy and the pathological diagnosis was large cell immunoblastic (B-cell) lymphoma. She received adjuvant combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone in the following 6 months. She has been relapse free for 52 months. To the best of our knowledge, this case has the longest disease-free survival among those reported. The present case indicated that primary adrenal lymphoma should be included in the differential diagnosis of fever of unknown origin and/or suprarenal mass. Chemotherapy following surgical resection may be considered the treatment of choice. ( info) |
4/51. Carboxypeptidase G2 rescue in a 79 year-old patient with cranial lymphoma after high-dose methotrexate induced acute renal failure. A 79 year-old female patient presented with immunoblastic B-cell lymphoma of the ethmoidal sinuses and destruction of the anterior cranial fossa. After 3 cycles of high-dose methorexate (HD-MTX) MTX serum level remained elevated and creatinine serum levels raised. The patient received Carboxypeptidase G2 (CPG2) intravenously. Within one hour the MTX serum level decreased to <1 micromol/l as determined by high pressure liquid chromatography (HPLC). The patient recovered without significant toxicity and attained a long lasting ongoing (>14 months) complete remission. In this case we were able to demonstrate that rescue from HD-MT nephrotoxicity by CPG2 is also safe and effective in patients with advanced age with impaired renal function. With the help of CPG2, sufficient and potentially curative therapy with HD-MTX may also be provided to patients with a high risk of renal failure. ( info) |
5/51. Primary malignant lymphoma of the ovary: an unusual presentation of a rare disease. Because the outcome of patients with primary ovarian non-Hodgkin's lymphoma (NHL) is controversial, we present the incidental finding of a primary malignant lymphoma of the ovary in a 50-year-old patient. Three and a half years following ablative surgery and adjuvant chemotherapy, the patient is alive and disease free. Ovarian lymphoma is a disease of reportedly poor prognosis. However, many previously reported cases of ovarian lymphoma actually represented ovarian involvement by a more diffuse lymphomatous process. If stringent criteria are used for case selection, true primary ovarian lymphoma is a very rare disease and usually carries a favorable prognosis. ( info) |
6/51. Concomitant progressive multifocal leucoencephalopathy and primary central nervous system lymphoma expressing jc virus oncogenic protein, large T antigen. This report describes the concomitant occurrence of the jc virus (JCV) induced demyelinating disease progressive multifocal leucoencephalopathy (PML) and a primary central nervous system lymphoma (PCNS-L) in a patient with AIDS. Postmortem neuropathological examination revealed characteristic features of PML including multiple lesions of demyelination, enlarged oligodendrocytes with hyperchromatic nuclei (many containing eosinophilic intranuclear inclusions), and enlarged astrocytes with bizarre hyperchromatic nuclei. Immunohistochemical analysis demonstrated the expression of the JCV capsid protein VP-1 in the nuclei of infected oligodendrocytes and astrocytes. The PCNS-L lesion located in the basal ganglia was highly cellular, distributed perivascularly, and consisted of large atypical plasmacytoid lymphocytes. Immunohistochemical examination of this neoplasm identified it to be of B cell origin. Moreover, expression of the JCV oncogenic protein, T antigen, was detected in the nuclei of the neoplastic lymphocytes. This study provides the first evidence for a possible association between JCV and PCNS-L. ( info) |
7/51. A man with hereditary exostoses and high-grade non-Hodgkin's lymphoma of the bone. Multiple cartilaginous exostoses (MCE) is an autosomal dominant disorder that can lead to malignant transformation from exostoses to a secondary chondrosarcoma. We present a case report of a 52-year-old man with MCE who had a palpable mass at the left shoulder. At the site of the left proximal humerus, a cartilaginous exostosis was localized, suggesting that the tumor developed by a malignant transformation of an exostosis into a secondary chondrosarcoma. Interestingly, a biopsy showed a diffuse large B-cell lymphoma with Burkitt-like features. To our knowledge, the association of high-grade lymphoma and hereditary exostoses has not been described previously. This case demonstrates that a malignant tumor at the location of a cartilaginous exostosis is not necessarily a chondrosarcoma and that a biopsy is an essential part of the diagnostic work-up. ( info) |
8/51. Primary B cell lymphoma of the rectum in a patient coinfected with hiv-1 and HTLV-I. This report describes a clinical case of a large cell, immunoblastic plasmacytoid malignant B-cell lymphoma of the rectum in an AIDS patient coinfected with HTLV-I. The malignant cells showed clonal genetic rearrangement of the HC (JH) and LCK genes. infection by EBV was demonstrated serologically and with slot blots using genomic dna of the cancer cells. Southern blot analysis with dna extracted from the lymphoma cells were negative for HTLV-I. The patient received seven cycles of VACO-B which induced complete but transient clinical remission of the tumor. The final outcome of the patient is unknown. ( info) |
9/51. Primary lacrimal sac B-cell immunoblastic lymphoma simulating an acute dacryocystitis. The case of a 72-year-old woman with diffuse large B-cell lymphoma of the lacrimal sac is reported. The patient was evaluated for the first time in our department for tearing of the right eye. One month later, a slightly aching mass appeared over the right lacrimal sac. An acute infectious etiology was suspected and antibiotic therapy was given. When she finally presented with a rapidly growing lesion, she underwent echography and computed tomography followed by incisional biopsy. Results of histopathologic and immunohistochemical evaluation showed a primary, diffuse, large B-cell non-Hodgkin lymphoma of the lacrimal sac. This case demonstrates how difficult the clinical diagnosis of tumors of the lacrimal sac may be in the early stages. The clinical signs, usually aspecific, may be misleading and the diagnosis delayed. ( info) |
| Progressive polyradiculopathy is a rare, well-documented complication of the acquired immunodeficiency syndrome in man. It has been commonly attributed to a cytomegalovirus (CMV) infection. We report two HIV-infected patients with clinical and electrophysiological features of a unique, subacute, progressive polyradiculopathy. Post-mortem examination in case 1 disclosed an infiltration of the leptomeninges, the lumbar spinal cord, and the anterior and posterior roots by a B-cell immunoblastic lymphoma. immunochemistry for HIV1 and CMV was negative in the peripheral and the central nervous system. Case 2 showed bone-marrow involvement by a Burkitt type lymphoma. Specific chemotherapy was followed by both clinical improvement of the polyradiculopathy and complete remission on a second bone-marrow biopsy. These findings may indicate that a lymphoma must also be considered a possible cause of polyradiculopathy in AIDS. ( info) |