Pericardial Extramedullary Hematopoiesis Associated with Metastatic Adenocarcinoma of Gastrointestinal or Pancreaticobiliary Origin: A Case Report.

Extramedullary hematopoiesis (EMH) is a rare complication of solid tumor malignancies. We describe the first case of a patient who developed EMH in the pericardium secondary to metastatic gastrointestinal or pancreaticobiliary cancer. A 58-year-old man presented with recurrent episodes of fatigue and shortness of breath and was treated with thoracocentesis and pericardiocentesis for pleural and pericardial effusions, respectively. Owing to a markedly elevated alkaline phosphatase, a bone scan was performed and demonstrated diffuse sclerotic lesions. Evaluation of pleural effusion diagnosed metastatic adenocarcinoma, and cytospin morphology of the pericardial fluid demonstrated EMH. While EMH secondary to solid tumors is commonly suggested to be due to cytokine signaling, we propose the mechanism of EMH in this patient was due to extensive disruption of bone marrow hematopoiesis, similar to what is seen in myeloproliferative neoplasms.

Modulation of T cell activation by stomatin-like protein 2.

T cell activation through the Ag receptor (TCR) requires sustained signaling from signalosomes within lipid raft microdomains in the plasma membrane. In a proteomic analysis of lipid rafts from human T cells, we identified stomatin-like protein (SLP)-2 as a candidate molecule involved in T cell activation through the Ag receptor. In this study, we show that SLP-2 expression in human primary lymphocytes is up-regulated following in vivo and ex vivo activation. In activated T cells, SLP-2 interacts with components of TCR signalosomes and with polymerized actin. More importantly, up-regulation of SLP-2 expression in human T cell lines and primary peripheral blood T cells increases effector responses, whereas down-regulation of SLP-2 expression correlates with loss of sustained TCR signaling and decreased T cell activation. Our data suggest that SLP-2 is an important player in T cell activation by ensuring sustained TCR signaling, which is required for full effector T cell differentiation, and point to SLP-2 as a potential target for immunomodulation.

Hodgkin lymphoma with cutaneous involvement.

We report a case of a 54-year-old previously healthy man with Hodgkin lymphoma who presented initially with a solitary cutaneous ulcer. Unlike non-Hodgkin lymphoma subtypes, skin involvement of Hodgkin lymphoma is extremely rare. Furthermore, the prognosis of Hodgkin lymphoma with skin infiltration is felt to be extremely poor. Contrary to other reports, this case demonstrates that a good response with standard therapy is possible.

Unexpected Gallbladder Metastasis of Clear Cell Renal Carcinoma.

Renal cell carcinoma (RCC) commonly metastasizes to areas such as the lungs, liver, bone, brain, adrenals, and lymph nodes. We present a rare case of a 59-year-old female patient with RCC metastasis to the gallbladder. The patient had undergone right nephrectomy for RCC more than 6 years prior to the metastasis. During routine follow-up, a polypoid lesion of the gallbladder was identified. Laparoscopic cholecystectomy was performed, and pathologic examination of the specimen revealed metastatic RCC. The patient was completely asymptomatic, which reinforces the importance of postoperative follow-up. Renal cell carcinoma is one of the few common malignancies for which there is good evidence of survival benefit from surgical resection of the metastatic tumours.

Splenic Angiosarcoma with Bone Marrow Involvement Initially Diagnosed as Systemic Mastocytosis: A Case Report.

We describe the case of a 67-year-old female patient presenting with constitutional symptoms and rapid decline. Two bone marrow core biopsies were performed, with spindled cells identified and thought to represent marrow involvement by systemic mastocytosis on the first biopsy. A diagnosis of metastatic vascular malignancy with sarcomatoid features was favored on the second core biopsy. The patient rapidly deteriorated and passed away. The post-mortem examination revealed a splenic angiosarcoma with metastasis to the liver and bone marrow. Splenic angiosarcoma is a rare, aggressive entity, with bone marrow metastasis even more uncommon. This report perceives this as a diagnostic consideration on bone marrow biopsies with spindled cells and explores the diagnostic dilemma and overlapping features of systemic mastocytosis and angiosarcoma.

Amyloid deposition in extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue: A clinicopathologic study of 5 cases.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is the most common subtype of marginal zone lymphoma (MZL), with stomach being the most frequent primary site, followed by salivary gland, lung and ocular adnexa. Although clinically indolent, MALT lymphoma has the potential of local recurrence and systemic spread. Amyloid deposition is a very unusual complication of MALT lymphoma. In this study, we report clinicopathologic features of 5 cases of MALT lymphomas with associated amyloid deposits. One case showed amyloid deposits in the primary lesion; the other four cases showed amyloid deposits only in recurrences. Previous studies suggest that the amyloid deposits do not implicate worse prognosis. In our study, although amyloid deposits were focal and organ confined, one patient had extensive deposits of amyloid in the large bowel wall leading to bowel perforation and another patient developed significant peripheral neuropathy due to amyloid deposits in the brachial plexus. In conclusion, amyloid deposits in MALT lymphomas are rare and organ/tumour confined. However, complications can be critical and cause considerable morbidity. Therefore, pathologists should be aware of the association between MALT lymphoma and amyloid deposition, and clinical follow up is warranted.

Primary intraocular lymphoma arising during methotrexate treatment of temporal arteritis.

CASE REPORT: Primary intraocular lymphoma arose over a period of 9 months in the left eye of an 81-year-old woman who was blind in both eyes from temporal arteritis. During this period, she was treated with prednisone and methotrexate. Following a sudden total hyphema, the eye was enucleated. Examination revealed that, in addition to iris neovascularisation and central retinal artery occlusion, the neurosensory retina was replaced by atypical lymphocytes. COMMENTS: Histological and immunohistochemical studies confirmed the presence of a lymphoma with features indicative of an immunosuppression-related disorder. The relationship of the lymphoma to the vascular changes within the eye is discussed.

Extensive Bone Marrow Necrosis in a Case of Acute Myeloid Leukemia Transformed from a Myeloproliferative Neoplasm.

Extensive necrosis affecting more than 50%percnt; of the bone marrow is an extremely rare histopathological finding. Relatively little is known about its clinical significance because it is most commonly identified at autopsy - whether it is an independent prognostic marker or whether it is a surrogate marker of underlying disease burden remains unclear. We describe herein a case of a 66-year-old patient with acute myeloid leukemia who presented with acute bone marrow failure and was found to have extensive necrosis. We include presenting clinical features, pathology attained at biopsy, and the challenge of treatment. Bone marrow necrosis is a rare but important clinicopathological entity whose recognition may herald the way for more effective prognostication of underlying disease.

Epstein-Barr virus seronegativity is a risk factor for late-onset posttransplant lymphoroliferative disorder in adult renal allograft recipients.

BACKGROUND: Posttransplant lymphoproliferative disorder (PTLD) remains a difficult management issue; therefore, many studies focus on the identification of risk factors to allow for preventive strategies. We investigated risk factors for PTLD in the adult renal transplant setting. METHODS: A single-center, matched case-control study design was used. Cases were identified from patients who underwent a first renal transplant between January 1, 1985, and December 1, 2001. Two controls were chosen per case, matched (+/-1 year) by date of transplant and graft survival. Clinical and demographic data were ascertained from medical records. Pretransplant serology for Epstein-Barr virus (EBV) and cytomegalovirus was confirmed on frozen, stored sera. Statistical analysis included univariate and multivariable examination of putative risk factors using conditional logistic regression. RESULTS: Twenty cases of PTLD were identified, an incidence of 2.4%. Median time from transplant to diagnosis was 55 months (range, 3-168 months), with 16 cases of late-onset PTLD (>1 year posttransplant). The only significant risk in univariate analysis was EBV-negative status at transplant (risk ratio 6.0, P=0.03). In multivariable analysis, EBV-negative status remained significant (adjusted risk ratio 8.9, P=0.01). The risk related to EBV status held true when late cases were analyzed separately (adjusted risk ratio 7.1, P=0.03). CONCLUSIONS: Pretransplant EBV-seronegative status is a strong risk for development of PTLD in adult renal allograft recipients, even in late disease. These results indicate that primary infection with EBV may have a pathogenic role in some cases of late PTLD.

Comprehensive cytogenetic analysis including multicolor spectral karyotyping and interphase fluorescence in situ hybridization in lymphoma diagnosis. a summary of 154 cases.

Cytogenetic analysis including multicolor spectral karyotyping (SKY) and interphase fluorescence in situ hybridization (FISH) was performed on 154 consecutive cases with suspected lymphoma. The cytogenetic results were reviewed in correlation with the final pathologic diagnosis. A diagnosis of lymphoma was established in 94 cases, with 16 Hodgkin lymphomas and 78 non-Hodgkin lymphomas (NHL). Cytogenetic results were obtained in 63 NHLs (81%); 61 of those showed abnormal karyotypes (97%). The t(14;18) or IGH-BCL2 fusion was detected in 83% (20/24) of follicular lymphomas and in 57% (12/21) of diffuse large B-cell lymphomas (DLBCL). The application of interphase FISH and SKY has contributed to a high detection rate of t(14;18) in DLBCLs. This study showed that genes at 1q25, 3p21, 3q21, 5q31, 6p23, 7q22, 8q11 approximately q12, 9q34, 11q23, 12q13, and 19q13.1 may have been involved as the less common changes in follicular lymphoma and DLBCL. Comparison of the recurrent secondary aberrations in the groups of follicular lymphoma and DLBCL revealed a pattern of clonal evolution from the changes rea(1)(p36), del(6q), +7, +12 or dup or trp(12)(q13q22), +der(18)t(14;18), and +21 in follicular lymphoma to the changes rea(1)(p36), del(6q), +6, +7, +9, rea(11)(q23), +12, -13 or del(13(q12q14), +18, +21, and +X in DLBCL. The clonal evolution of the secondary aberrations is thought to contribute to the progression of the disease. About 90% (16/18) of other types of NHL had abnormal karyotypes showing specific translocations or gene rearrangements consistent with the pathologic diagnosis. A comprehensive cytogenetics approach including SKY and interphase FISH using probes for specific genes, such as IGH, BCL2, CCND1, and ALK, is a very useful ancillary diagnostic tool for lymphomas. The combined approach also led to the identification of t(2;19)(p23;q13.1) as a new variant of t(2;5)(p23;q35) in a case of Ki-1-positive anaplastic large cell lymphoma with a null cell phenotype.

Hodgkin lymphoma of the oral mucosa.

In this report, we describe a rare case of relapsed nodular sclerosing Hodgkin lymphoma presenting as a lesion of the oral mucosa. Although this is an uncommon clinical scenario, health care professionals should be aware of this possibility. A brief differential diagnosis and review of Hodgkin lymphoma is discussed.

Mediastinal choriocarcinoma masquerading as relapsed hodgkin lymphoma.

Primary mediastinal choriocarcinoma is a rare extragonadal germ cell malignancy. We describe the first case of a patient who developed mediastinal choriocarcinoma after treatment for Hodgkin lymphoma (HL). A 25-year-old man with classic HL, nodular sclerosis subtype, underwent treatment with splenectomy followed by radiation therapy. Unfortunately, his disease relapsed with a paraspinal mass, and he was subsequently treated with MOPP (mechlorethamine, Oncovin, procarbazine, and prednisone) alternating with ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine). He achieved a complete remission after 6 cycles. Ten years after treatment, the patient presented with a persistent cough, haemoptysis, right supraclavicular lymphadenopathy, and weight loss. His chest X-ray showed opacification of the lower right hemithorax with a widened mediastinum. Given unresponsiveness to several antibiotics and lack of evidence for lung volume loss, there were concerns over lung infiltration with relapsed lymphoma. Transbronchial fine needle aspiration biopsy suggested recurrence of his HL. MOPP alternating with ABVD was again given. Due to disease progression, brachytherapy as well as a cocktail of dexamethasone, cytarabine, and cisplatin were also tried. However, on a subsequent excisional lymph node biopsy, it turned out that the tumour was in fact choriocarcinoma and not relapsed HL. Unfortunately, despite aggressive therapy, the patient's disease rapidly progressed, and he died within 2 weeks.

Castleman's disease in children: report of 2 cases and clinicopathologic review.

Castleman's disease (CD), a disorder of lymphoid hyperplasia with an unknown etiology, is rare in children. It usually presents as a localized mass and is often cured by surgery. The authors report two unusual cases of CD in children. One patient had localized CD in the left supraclavicular region, a rare site of unicentric disease. She was ultimately treated by excisional biopsy, and the mass did not recur. Another patient presented with airway compression due to localized infiltrating mediastinal disease, not amenable to surgery. She refused prednisone and was treated with radiotherapy, with slow remission and no relapse for 4 years.