Paleopathological study on malignant bone tumor in Japan. Differential diagnosis on osteolytic lesions in the skull

This report concerns a probable case of metastatic malignant bone tumor in the skull of a prehistoric skeleton from Honshu Island in Japan. In gross observation, a fragmental skull of an adult showed many osteolytic lesions without any healing processes which could be also ascertained by roentgenological studies. Besides this case, four cases with a diagnosis of malignant bone tumor have so far been reported among archeological skeletal remains in Japan. This case from the prehistoric "Jomon" period is certainly the oldest case showing such malignant tumorous change in the skeleton. In this report, the osteolytic changes in the "Jomon" skull are described in detail and compared with two other cases showing the same osteolytic changes. The morphology and distribution of the lesions as well as the sex and age of the individual are discussed to make an adequate differential diagnosis for malignant osteolytic lesions in the skeleton.     

Pseudotumoral Neuroparacoccidioidomycosis: One Case Report

Neuroparacoccidioidomycosis is the central nervous system involvement by Paracoccidioides brasiliensis, a condition that may be underdiagnosed and has been scarcely reported. We describe a case of neuroparacoccidioidomycosis in a diabetic male with antecedent of heavy cigarette smoking and alcohol abuse. Thirty years before, he lived in the Amazon area and exerted rural activities in more recent years. The patient's main complaints were headache, visual deficit, hemiparesis, and weight loss. Imaging studies detected changes in the lungs and right adrenal gland, in addition to brain lesions. Paracoccidioides brasiliensis was found in tissue samples collected by the lung and brain biopsies. The patient is under ambulatory surveillance and in use of trimethoprim-sulfamethoxazole.     

T-cell leukemias of adulthood]

9 adult patients suffering from different forms of T-cell-malignancies were investigated: 4 patients with T-ALL; 1-T-ALL-CLL mixed form (prolymphocytic); 2 T-CLL; 2 Sézary-syndrome. The clinical peculiarities of the different forms of leukemias were compared: involvement of lymph nodes and spleen, of the central nervous system and the skin was frequent; in contrast to the findings in Sézary-syndrome, bone marrow infiltration was prominent. Light and electron microscopic morphology of the malignant cells are described. In all cases a strong activity of acid phosphatase was demonstrated, in one patient prominent deposits of glycogen. The T-cell-quality of the respective malignant cell population as well as the B-T-cell distribution of the remaining "normal" lymphocytes were shown by the following cell markers: demonstration of T-cell-antigen, resp. membrane immunoglobulins with the aid of specific heterologous antisera conjugated with peroxidase, 125iodine or fluoresceine; complement consumtion or cytotoxicity with such antisera; spontaneous rosette formation with sheep red cells or with acrylic acid beads. Usually, there was a good coincidence in results obtained with the different markers. In two patients, however, T-cells demonstrated by anti-T-globulin were not able to form T-rosettes. Responsiveness of the malignant T-cells and also of the remaining "normal" blood lymphocytes to different mitogens usually was depressed, immunoglobulin levels in the blood mostly were normal. Taking all findings into consideration, T-cell-leukemias of the adult represent a special group of hematological malignancies; the different subgroups show similarities; transitional forms occur.     

Synchronous tumors of the female genital tract: triple malignant and one benign tumor

Synchronous tumors of the female genital tract are rare, accounting for 0.7-1.8% of all cases. Double synchronous tumors are most often mentioned in the literature. Reviewing the English literature on this topic, we have found only one case report of a triple synchronous tumor. The 55-year-old patient mentioned in our case has had advanced diabetes mellitus, and has been treated with corticosteroid therapy for a long time because of chronic obstructive pulmonary disease (COPD). She was examined because of her vulvar tumor. During the diagnostic procedure, cervical and endometrial malignant tumors and a benign ovarian cyst have also been found. This event brings to our attention the fact that we should be prepared to manage synchronous even triple malignant gynecological tumors.     

Danger of Hypoglycemia Due to Acute Tramadol Poisoning

ObjectiveTo report a case of prolonged hypoglycemia after acute tramadol poisoning.MethodsWe describe a patient’s clinical presentation and outcome with prolonged hypoglycemia attributable to acute tramadol poisoning. In addition, the possible mechanism for the hypoglycemia is discussed, and a brief review of the pertinent literature is presented.ResultsA 54-year-old woman had previously under- gone a partial hepatectomy because of involvement of her liver by a gastrointestinal stromal tumor. After ingestion of 3,000 mg of tramadol with suicidal intent, she developed prolonged hypoglycemia that necessitated treatment with continuous intravenous glucose infusion for 24 hours. Reports in the literature have described central nervous system depression, nausea, vomiting, tachycardia, seizures, and even death from tramadol overdoses.ConclusionThis report alerts clinicians to the potential danger of severe hypoglycemia in tramadol poisoning. (Endocr Pract. 2012;18:e151-e152)     

Cytology of the cerebrospinal fluid in primary malignant lymphomas of the central nervous system

Two cases of primary malignant lymphoma of the central nervous system are reported. Clinical and pathologic findings did not indicate systemic lymphomatous involvement of lymph nodes and bone marrow. The patients presented with different neurologic deficits. Cytologic examination of the cerebrospinal fluid was the first and most useful technique for establishing the diagnosis.     

Disseminated Breast Cancer Cells Acquire a Highly Malignant and Aggressive Metastatic Phenotype during Metastatic Latency in the Bone

Background

Disseminated tumor cells (DTCs) in the bone marrow may exist in a dormant state for extended periods of time, maintaining the ability to proliferate upon activation, engraft at new sites, and form detectable metastases. However, understanding of the behavior and biology of dormant breast cancer cells in the bone marrow niche remains limited, as well as their potential involvement in tumor recurrence and metastasis. Therefore, the purpose of this study was to investigate the tumorigenicity and metastatic potential of dormant disseminated breast cancer cells (prior to activation) in the bone marrow.

Methodology/Principal Findings

Total bone marrow, isolated from mice previously injected with tumorspheres into the mammary fat pad, was injected into the mammary fat pad of NUDE mice. As a negative control, bone marrow isolated from non-injected mice was injected into the mammary fat pad of NUDE mice. The resultant tumors were analyzed by immunohistochemistry for expression of epithelial and mesenchymal markers. Mouse lungs, livers, and kidneys were analyzed by H+E staining to detect metastases. The injection of bone marrow isolated from mice previously injected with tumorspheres into the mammary fat pad, resulted in large tumor formation in the mammary fat pad 2 months post-injection. However, the injection of bone marrow isolated from non-injected mice did not result in tumor formation in the mammary fat pad. The DTC-derived tumors exhibited accelerated development of metastatic lesions within the lung, liver and kidney. The resultant tumors and the majority of metastatic lesions within the lung and liver exhibited a mesenchymal-like phenotype.

Conclusions/Significance

Dormant DTCs within the bone marrow are highly malignant upon injection into the mammary fat pad, with the accelerated development of metastatic lesions within the lung, liver and kidney. These results suggest the acquisition of a more aggressive phenotype of DTCs during metastatic latency within the bone marrow microenvironment.     

Bronchioloalveolar carcinoma presenting with meningeal carcinomatosis. Cytologic diagnosis in cerebrospinal fluid

The cytologic findings in a 35-year-old patient with bronchioloalveolar carcinoma who initially presented with central nervous system involvement are reported. Following the cytologic diagnosis of carcinomatous meningitis (metastatic adenocarcinoma), an open lung biopsy was performed, which confirmed the presence of a primary pulmonary neoplasm (bronchioloalveolar carcinoma). This case illustrates the importance of the cytologic diagnosis of a clinically unsuspected primary neoplasm. Further, together with three earlier reported cases, it indicates that, in young patients, tumor cells shedding into the cerebrospinal fluid can be the first indication of bronchioloalveolar carcinoma.     

Central neurophysiological processing of joint pain on the basis of studies performed in normal animals and in models of experimental arthritis.

On the basis of anatomical and electrophysiological studies, this review summarizes first, the data dealing with the transmission of joint inputs in the central nervous system of normal animals at the spinal and supraspinal levels. It appears that in these conditions neuronal responses to mechanical noxious stimuli of the joints are relatively few and (or) weak. Second, in sharp contrast, the studies performed in polyarthritic rats have emphasized the profound changes in the activities (spontaneous firing and responsiveness) of the somatosensory neurones at various levels of the central nervous system (CNS), including the thalamus and primary somatosensory cortex; many were spontaneously active and a majority of them could be maximally activated by gentle mechanical stimuli applied to the inflamed joints. Although the change in the sensitivity of the peripheral mechanoreceptors has a major role in the modifications described in the CNS, additional observations have suggested a complex interaction between peripheral and central processes. On the basis of the recent data obtained in poly- and mono-arthritic animals; the following phenomena have been successively considered: the segmental and hetero-segmental "cross-talk" and their possible relationship with referred pain; the involvement of "new" neuronal populations as a possible basis of a selective system for joint pain; and the possible involvement of changes in the various control systems that normally modulate the nociceptive inputs at different levels of the CNS.     

Reticulum Cell Sarcoma of the Central Nervous System

One variety of sarcoma of the central nervous system (CNS) has been referred to under such synonyms as perithelial sarcoma, microglioma, and reticulum cell sarcoma. A case of diffuse involvement of the CNS is reported and the author believes that the tumour is a reticulum cell sarcoma because: (1) in addition to involvement of the CNS, lesions characteristic of a diffuse lymphomatous process were present in extraneural sites; (2) silver carbonate impregnation of the tumour cells was demonstrated. This tinctorial property has been reported as characteristic of microgliomas but has been shown to be an unreliable criterion for the differentiation of the cells of microglial from those of reticulum cell sarcomas. It is concluded that in such cases the CNS is the initial and major focus of a multicentric malignant lymphoma which has the histologic characteristics of a reticulum cell sarcoma.     

Venous pseudo-aneurysm as a late complication of short-term central venous catheterisation

Background

Adrenocortical carcinoma is a rare, highly malignant tumor. Cardiac involvement of the tumor is very rare. Echocardiography facilitates the evaluation of the cardiac involvement of the tumor.

Case Presentation

We describe a patient with an adrenal tumor. Transthoracic echo showed its extension into the right atrium. Accordingly, a combined abdominal and cardiac operation was performed, monitored by transesophageal echocardiography.

Conclusion

This case highlights the importance of echocardiography in revealing the cardiac involvement by this tumor and in planning the operative procedure.     

HIV Immune Recovery Inflammatory Syndrome and Central Nervous System Paracoccidioidomycosis

The immune reconstitution inflammatory syndrome (IRIS) is a deregulated inflammatory response to invading microorganisms. It is manifested when there is an abrupt change in host immunity from an anti-inflammatory and immunosuppressive state to a pro-inflammatory state as a result of rapid depletion or removal of factors that promote immune suppression or inhibition of inflammation. The aim of this paper is to discuss and re-interpret the possibility of association of paracoccidioidomycosis (PCM) with IRIS in the central nervous system (CNS) in a case from Brazil published by Silva-Vergara ML. et al. (Mycopathologia 177:137–141, 6). An AIDS patient who was not receiving medical care developed pulmonary PCM successfully treated with itraconazole. The patient developed central nervous system PCM (NPCM) after starting the ARV therapy with recovery of immunity and control of HIV viral load, although it was not interpreted as IRIS by the authors, it fulfills the criteria for CNS IRIS. This could be the first case of NPCM associated with IRIS described. Although not frequent, IRIS must be considered in PCM patients and HIV, from endemic areas or patients that traveled to endemic areas, receiving ARV treatment and with worsening symptoms.     

以中枢神经系统受累为首发表现的干燥综合征1例并文献复习

目的:提高对干燥综合征中枢神经系统损害的认识,了解其特点及治疗。方法:报告1例以中枢神经系统受累为首发表现的干燥综合征病例,并对相关文献进行复习。结果:此例患者以脊髓病变为首发症状,长期口干、眼干,查体及实验室等相关检查诊断干燥综合征明确。结论:干燥综合征可存在中枢神经系统损害,其中脊髓病变较常见,应注意完善影像学及脑脊液检查,并与系统性红斑狼疮等结缔组织病相鉴别。     

A Case of Hypoglycemia,Lactic Acidosis,and Hematologic Malignancy

ObjectiveTo report a case of chronic and persistent hypoglycemia and lactic acidosis in a 74-year-old military veteran.MethodsThe clinical, laboratory, radiologic, and cytogenetic details of the case are presented, followed by a discussion of the related literature.ResultsThe patient was treated for septicemia without evidence of infection. Mitochondrial dysfunction was explored because of the possibility of environmental exposures during military service. On bone marrow biopsy, he was found to have immature B-cell lymphoma and myelodysplasia. There have been 28 previously reported cases of non-Hodgkin lymphoma-induced lactic acidosis in adult patients (11 with hypoglycemia), which has been associated with a high mortality rate.ConclusionOur case is unique because, to our knowledge, it is the first reported case of immature B-cell lymphoma/leukemia and myelodysplasia with a complex karyotype based on extensive cytogenetic studies in a patient presenting with hypoglycemia, lactic acidosis, and central nervous system involvement by lymphoma. (Endocr Pract. 2010;16:241-243)     

成人胶质瘤诊断的分子遗传学研究进展

胶质瘤是目前中枢神经系统中常见的恶性肿瘤,由于脑组织的特殊性,胶质瘤呈弥漫浸润性生长,恶性程度高,手术难以完整切除且易复发。2007年(第四版)WHO中枢神经系统肿瘤病理学和遗传学对胶质瘤进行了详细的组织学分类,但是循证医学发现依靠组织学的病理诊断标准并不能对胶质瘤的临床表现和预后评估作出精准的判断。近年来全世界都在开展胶质瘤相关的遗传学研究,许多遗传学分子改变被发现,如异柠檬酸脱氢酶(IDH)突变、染色体1p/19q缺失、TP53突变、ATRX突变和TERT启动子突变等,组织学诊断受到了挑战。因此更多的病理科和神经外科医生结合组织形态和遗传学改变对胶质瘤作出"综合性"诊断,使得病理诊断更接近胶质瘤的生物学本质,以便更精准的指导临床治疗。     

Neurotrophins play an important role in the perineural invasion and distant metastasis of malignant tumor

Neurotrophins (NTs) family was first discovered in nervous system and it regulates the proliferation and differentiation of many neural cell types in the peripheral and central nervous system.Due to their perineural invasive characters, certain part of malignant tumor cases was first diagnosed because of nerve paralysis or idiopathic neuralgia caused by perineural invasion. For this reason, the study on the association between NTs and perineural invasion of malignant tumor aroused the attention of many researchers. Increasing evidence indicates that NTs and their receptors, Trks, play important roles in malignant cells, especially the exhibiting perineural invasive phenotype. It was suggested that NTs produced by neural tissue can act as a chemotactic factor, and tumor cells in which the overexpression of Trks' exists seem to be selected to invade the perineural space. Except for contributing to perineural invasion of malignant tumor, accumulated evidence proved NTs now also significantly associated with the metastasis of malignant tumor. Overexpression of NTs or Trks often correlated with the tumorigenesis, angiogenesis and anoikis resistance in these malignancies, contributing significantly to the metastasis and poor prognosis.In summary, besides its role in development and function of nervous system, NTs also play an important role in the perineural invasion and metastasis of malignant tumor. Considering the role that NTs played in malignant tumor, we believe that further studies between NTs and malignant tumor are necessary. Research on the role of NTs pathway might allow advancements in this field.     

131I]metaiodobenzylguanidine therapy of malignant pheochromocytoma: interference of medication

Malignant pheochromocytoma may present as a widespread metastatic disease, which is little or non-responsive to external beam radiotherapy and chemotherapy. The prognosis of these patients is bad due to both the progressive metastasis and the secretion of excess catecholamines which may cause hypertensive episodes. For these conditions [131I]metaiodobenzylguanidine (131I-MIBG) therapy may be an alternative treatment modality to induce both tumor remission and reduction of hormonal activity of the disease. The experience with 131I-MIBG therapy in four patients with metastatic malignant pheochromocytoma at The Netherlands Cancer Institute is reviewed. One patient with abdominal tumor recurrence and metastases to the lymph nodes and lungs had a partial remission of disease for 3 years; a second had a mixed response together with palliation and two other patients had stable disease, but were relieved of bone pain and severe hypertension, respectively. It is essential to be aware of the medication the patient is using, as many drugs are known or may be expected to interfere with the uptake and/or retention of 131I-MIBG by the tumor cells. The case of a significant reduction of 131I-MIBG uptake and retention by Labetalol in one of the patients is discussed. It is concluded that 131I-MIBG therapy may induce objective remission in patients with malignant pheochromocytoma and is certainly meaningful in the reduction of hormonal activity, the control of hypertension and the relief of pain from metastases.     

Genomic profiling of plasmablastic lymphoma using array comparative genomic hybridization (aCGH): revealing significant overlapping genomic lesions with diffuse large B-cell lymphoma

Background

Cholestatic jaundice as a presenting symptom of Precursor T-lymphoblastic leukemia (T-ALL)/lymphoma (T-LBL) has never been reported in literature. Similarly, precursor T-ALL/T-LBL is characteristically negative for synaptophysin. We report the first case of a patient with precursor T-ALL/T-LBL who presented with cholestatic jaundice and aberrant tumor expression of synaptophysin.

Case report

42 year old male presented with anorexia, nausea, jaundice, pale stools, dark urine and about 35 pound weight loss over the previous 3 weeks. The initial laboratory work was suggestive of cholestatic jaundice. Markedly elevated LDH (2025 U/L) and CA 19-9 (1778 u/ML) were also noticed. The CT scan of abdomen showed massive hepatomegaly with coarse echotexture with contracted gall bladder and normal sized common bile duct. Chest x-ray revealed a mediastinal mass with mediastinal widening. CT scan of the chest showed anterior mediastinal mass (16 cm × 10 cm). CT guided biopsy of the mass showed malignant lymphoma with diffuse proliferation of medium sized lymphoid cells. The neoplastic cells were positive for CD1a, CD3, CD4, CD5, CD8 and CD43 with aberrant expression of synaptophysin. PET CT scan again showed a large anterior mediastinal mass with diffuse liver involvement and abnormal activity in axial bones. CT guided liver biopsy and bone marrow biopsy revealed the same morphology and immunohistochemistry. Bone marrow aspirate showed 85% lymphoblasts. Thus, the diagnosis of precursor T-ALL/T-LBL was made and jaundice with elevated CA 19-9 were attributed to intrahepatic cholestasis.

Conclusion

Our case illustrates an unusual presentation of hematological malignancies as cholestatic jaundice. It also indicates the non-specific nature of CA 19-9 for pancreaticobiliary malignancies. It is the first case report of neoplastic precursor T cell lymphoblasts with unusual expression of synaptophysin. Tissue biopsy with thorough immunohistochemistry is required to differentiate precursor T-ALL/T-LBL from thymoma and small cell carcinoma.     

Rosai-Dorfman disease manifesting as multiple subcutaneous nodules. Report of a case with diagnosis on a fine needle aspirate

BACKGROUND: Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a benign, self-limiting histiocytic proliferative disorder commonly involving the lymph nodes. Extranodal disease occurs in about 40% of cases, and the sites involved are skin, nasal cavity, paranasal sinuses, eyelids, orbit, bone and central nervous system. CASE: A case of RDD presented as subcutaneous nodules and was diagnosed on a fine needle aspirate. The aspirate revealed numerous histiocytes with phagocytosis of lymphocytes, plasma cells and neutrophils. Surgical biopsy and immunocytochemical stain for S-100 protein confirmed the diagnosis. Later the patient developed lymphadenopathy and involvement of the nasal septum. CONCLUSION: Extranodal RDD can be diagnosed by FNAC in conjunction with immunocytochemistry.     

Remarkable regression of a lung recurrence from an undifferentiated embryonal sarcoma of the liver treated with a DC vaccine combined with immune cells: A case report

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant tumor that occurs predominantly in children and has a poor prognosis. Here, we report a novel case in which the UESL presented in the left lobe of the liver and metastasized into both lungs after surgical resection. The patient recovered after our administration of an immunotherapeutic combination of DCs (Dendritic Cells) and multi-immune cells, such as cytokine-induced killer cells (CIKs) and natural killer cells (NKs). After the third cycle of immunotherapy, a CT scan showed a remarkable regression of the lung metastases. This finding supports the conclusion that the DC-based treatment is a promising potential strategy for treating patients with relapsed UESL.