Variety Of Paraneoplastic Syndromes Research Articles

Stereotactic Radiotherapy in the Treatment of Paraneoplastic Vasculitis in Oligometastatic Renal Cell Carcinoma

Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT.

THE RELATIONSHIP BETWEEN OF CANCERS AND RHEUMATIC DISEASES

The literature review considers the fundamental elements of a relationship between cancers and rheumatic diseases, including a variety of paraneoplastic syndromes, a profile of rheumatic diseases associated with the development of oncopathology, and autoimmune rheumatic syndromes detected in patients who receive anticancer therapy. Emphasis is placed on the need for interdisciplinary interaction to deepen understanding of the pathophysiological mechanisms of both groups of diseases and to improve medical care for patients.

Reversible intrahepatic cholestasis in metastatic prostate cancer: An uncommon paraneoplastic syndrome.

As with other genitourinary malignancies, a variety of paraneoplastic syndromes have been revealed to occur in patients with prostate cancer. Stauffer's Syndrome is a well-described clinical syndrome which manifests via intrahepatic cholestasis in patients with renal cell carcinoma. Less common is intrahepatic cholestasis occurring in association with prostate cancer. The current case report discusses a 67-year-old man presenting with liver failure secondary to intrahepatic cholestasis co-existing with metastatic prostate adenocarcinoma. The patient's liver failure completely resolved with androgen-deprivation therapy suggesting an association between these two entities. The case report evaluates existing literature on this uncommon syndrome including the clinical presentation, natural history, and potential pathophysiology.

Cancer and Autoimmunity: Paraneoplastic Neurological Disorders Associated With Neuroblastic Tumors

Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks. In each clinical syndrome, a large subgroup of patients had no demonstrated tumor. Most neurological PNS are immunologically mediated, and CSF neuroimmunological studies show common elements of immune involvement in PNS as well as important differences. Future immunotherapy strategies may be able to take advantage of these abnormalities.

An Unusual Case of Cholestatic Hepatitis

Renal cell carcinoma can present with a variety of paraneoplastic syndromes. Reversible hepatic dysfunction in the setting of non-metastatic renal cell carcinoma is a rare paraneoplastic phenomenon that was first described by Stauffer in 1961. This clinical entity is characterized by reversal of liver chemistry abnormalities after resection of the primary tumor. Here we present an unusual case of non-metastatic hepatic dysfunction secondary to renal cell carcinoma, also known as Stauffer’s syndrome, in a 55-year old patient who presented with liver synthetic dysfunction and notable hyperbilirubinemia. Unlike earlier reports, the icteric variant of Staffer’s syndrome has been emerging as the predominantly reported variant in the recent literature. Moreover, poor correlation between the severity of hepatic dysfunction and the liver biopsy findings was noted. The variable and non-specific presenting symptoms and signs as well as the need for extensive evaluation for abnormal liver chemistry usually results in a delay in the diagnosis of the primary tumor. Therefore, physicians need to be familiar with this clinical entity as a possible reversible etiology of liver chemistry abnormalities in patients with renal and other solid tumors as this may guide further investigations, lead to earlier diagnosis and treatment of the primary tumor, and ultimately improve outcomes.

Paraneoplastic neurological syndromes in breast cancer

SUMMARY There is an increased understanding and recognition of paraneoplastic syndromes in patients with a variety of malignancies. Paraneoplastic syndromes are associated with many tumor types and can potentially affect most organ systems. Breast cancer is one of the most common cancers worldwide, including in the USA, and can be associated with a variety of paraneoplastic syndromes affecting many systems, including the endocrine, neurologic, dermatologic, rheumatologic, hematologic and psychiatric systems. These syndromes can cause significant morbidity; therefore, effective diagnostic and treatment strategies need to be applied to improve quality of life, enhance delivery of cancer therapy and potentially prolong survival. This review focuses on the diagnosis and treatment of major paraneoplastic neurologic syndromes associated with breast cancer.

Dermatomyositis associated with gallbladder carcinoma: A case report

Patients with gallbladder carcinoma can present with a variety of paraneoplastic syndromes, including Cushing's syndrome, hypercalcemia, acanthosis nigricans, bullous pemphigoid, dermatomyositis and the sign of Leser-Trélat. Surgical resection of the primary tumor results in resolution of these paraneoplastic syndromes. We present a 67-year old female with facial and cervical erythema who was initially diagnosed with dermatomyositis. However, an abdominal computed tomography (CT) and positron emission tomography-CT scan was suspicious for gallbladder carcinoma with lymph node metastasis. After surgical resection, her dermatomyositis was resolved. This case demonstrates that dermatomyositis may be a manifestation of preexisting gallbladder carcinoma.

Thymic Neoplasms: a Clinical Update

Thymoma is a unique neoplasm of the anterior mediastinum that is frequently associated with indolent growth and a variety of paraneoplastic syndromes. One third of cases are detected during the evaluation of myasthenia gravis. Classification systems of thymoma have limited ability in accurately predicting prognosis and course of disease. Thus, staging is the only way to predict clinical behavior. Encapsulated tumors that are surgically resected carry the best prognosis. Adjuvant radiotherapy is recommended for incompletely excised and most invasive thymomas. Chemotherapy in anthracycline-based chemotherapy remains the most effective chemotherapy for neoadjuvant, adjuvant or palliative treatment.

Paraneoplastic sensory neuropathy associated with small cell carcinoma of the gallbladder

A small number of patients with carcinoma of the gallbladder can present with a variety of paraneoplastic syndromes, including the Cushing syndrome, hypercalcemia, acanthosis nigricans, bullous pemphigoid, dermatomyositis, and the Leser-Trélat sign. We report on what appears to be the first case of a patient, a 48-year-old woman, with anti-Hu paraneoplastic sensory neuropathy and small cell carcinoma of the gallbladder. The patient's neurologic symptoms preceded the diagnosis of small cell carcinoma by 11 months. These symptoms improved after surgical removal of the tumor and chemotherapy. The small cell carcinoma was relatively small and was not associated with gallstones. In spite of the small size of the tumor, it metastasized to a celiac lymph node and probably to the liver. Anti-Hu paraneoplastic sensory neuropathy should be added to the list of paraneoplastic syndromes associated with small cell carcinoma of the gallbladder.

Initial Evaluation of the Patient With Lung Cancer: Symptoms, Signs, Laboratory Tests, and Paraneoplastic Syndromes: ACCP Evidenced-Based Clinical Practice Guidelines (2nd Edition)

This chapter of the guidelines is intended to provide an evidence-based assessment of the initial evaluation of patients recognized as having lung cancer and the recognition of paraneoplastic syndromes. The current medical literature that is applicable to this issue was identified by a computerized search and was evaluated using standardized methods. Recommendations were framed using the approach described by the Health and Science Policy Committee of the American College of Chest Physicians. Patients with lung cancer usually present with multiple symptoms, both respiratory related and constitutional. There is usually a time delay between symptom recognition by the patient and the ultimate diagnosis of lung cancer by the physician. Whether this time delay impacts prognosis is unclear, but delivering timely and efficient care is an important component in its own right. Lung cancer may be accompanied by a variety of paraneoplastic syndromes. These syndromes may not necessarily preclude treatment with a curative intent. The initial evaluation of the patient with known or suspected lung cancer should include an assessment of symptoms, signs, and laboratory test results in a standardized manner as a screen for identifying those patients with paraneoplastic syndromes and a higher likelihood of metastatic disease.

Novel association between thymomas and T-cell Non-Hodgkin's lymphomas: Evaluation of 5 cases

7326 Background: Thymomas are often accompanied by a variety of paraneoplastic syndromes and have been associated with abnormal intra-thymic T-cell development, as well as oligoclonal T-cell populations in the bone marrow of certain patients. Prior series have shown an increased incidence of secondary malignancies in patients with thymoma. Recently, Engels et al. (Int. J. Cancer: 105, 546–551) demonstrated an excessive occurrence of Non-Hodgkin's lymphomas (NHL) after a thymoma diagnosis, identifying 7 cases among 9 SEER registries; however, only B-cell NHL cases were found. We report the association between thymomas and T-cell NHLs and examine its possible causal relationship to abnormal T-cell maturation within the thymus, employing immunohistochemical stains and assessment of T-cell clonality. Methods: Five patients with thymoma and T-cell NHL (both biopsy-proven) were identified through the Thymoma Clinic database at Indiana University. The T-NHL diagnosis could precede, concur or be subsequent to the thymoma. Original thymic samples are being stained for CD2, CD3, CD5, CD4, CD8, CD25, CDK6, p53 and compared to NHL samples. Thymic T-lymphocytes and original T-NHL lymphocytes are being subjected to T-cell receptor gene rearrangement studies by PCR. Results: Median age at the time of thymoma diagnosis was 38 years (range, 28–56). Patient characteristics are summarized in the table. All patients had cortical thymoma. Two patients (cases 4 and 5) presented with the T-NHL in the setting of persistent thymoma. Conclusions: We describe a novel clinical association between thymomas and T-cell NHLs. Abnormal T-cell maturation induced by thymomas may play a role in T-NHL pathogenesis in these patients. Correlative studies will be available for presentation at the time of the Annual Meeting. No significant financial relationships to disclose.

Novel association between thymomas and T-cell Non-Hodgkin's lymphomas: Evaluation of 5 cases

7326 Background: Thymomas are often accompanied by a variety of paraneoplastic syndromes and have been associated with abnormal intra-thymic T-cell development, as well as oligoclonal T-cell populations in the bone marrow of certain patients. Prior series have shown an increased incidence of secondary malignancies in patients with thymoma. Recently, Engels et al. (Int. J. Cancer: 105, 546–551) demonstrated an excessive occurrence of Non-Hodgkin's lymphomas (NHL) after a thymoma diagnosis, identifying 7 cases among 9 SEER registries; however, only B-cell NHL cases were found. We report the association between thymomas and T-cell NHLs and examine its possible causal relationship to abnormal T-cell maturation within the thymus, employing immunohistochemical stains and assessment of T-cell clonality. Methods: Five patients with thymoma and T-cell NHL (both biopsy-proven) were identified through the Thymoma Clinic database at Indiana University. The T-NHL diagnosis could precede, concur or be subsequent to the thymoma. Original thymic samples are being stained for CD2, CD3, CD5, CD4, CD8, CD25, CDK6, p53 and compared to NHL samples. Thymic T-lymphocytes and original T-NHL lymphocytes are being subjected to T-cell receptor gene rearrangement studies by PCR. Results: Median age at the time of thymoma diagnosis was 38 years (range, 28–56). Patient characteristics are summarized in the table. All patients had cortical thymoma. Two patients (cases 4 and 5) presented with the T-NHL in the setting of persistent thymoma. Conclusions: We describe a novel clinical association between thymomas and T-cell NHLs. Abnormal T-cell maturation induced by thymomas may play a role in T-NHL pathogenesis in these patients. Correlative studies will be available for presentation at the time of the Annual Meeting. No significant financial relationships to disclose.

Paraneoplastic cerebellar degeneration with ovarian endometroid carcinoma

Paraneoplastic cerebellar degeneration with ovarian endometroid carcinoma

Paraneoplastic syndromes associated with ovarian neoplasms

This pictorial review illustrates a wide variety of paraneoplastic syndromes possibly associated with ovarian tumors. Many paraneoplastic syndromes are caused by unknown mechanisms, while others can be explained by the ectopic production of hormones (paraendocrine syndromes). Paraneoplastic syndromes frequently antedate the recognition of the tumor, and disappear with the removal of the tumor. Knowledge of the variety of paraneoplastic syndromes may allow early detection of the underlying disease.

Cancer-associated retinopathy

A variety of paraneoplastic syndromes affect the central nervous system including eye. So far, two types of retinopathy are known to be associated with patients with malignancies, cancer-associated retinopathy (CAR), and melanoma-associated retinopathy (MAR). CAR is associated with epithelial cancers, mostly lung small cell carcinoma, and is characterized by retinitis pigmentosa-like retinal degeneration. Usually CAR can be found before an underlying primary cancer is diagnosed. MAR is associated with cutaneous malignant melanoma and is characterized by the relatively sudden onset of photophobia and nyctalopsia. The flash electroretinogram (ERG) of MAR patients shows a negative waveform, reduced b-wave amplitude, and reservation of a-wave amplitude, suggesting that bipolar cells may be affected. CAR and MAR are believed to result from an autoimmune response. In CAR, a calcium binding protein called recoverin, a 70 kDa protein, and neurofilaments are the retinal antigens recognized by the patient's serum. In contrast, the retinal antigens in MAR have not yet been identified, although patient sera specifically recognized retinal bipolar cells in immunocytochemistry.

Biochemical aids to cancer management.

The clinical biochemist has an important role to play in the evaluation of new strategies for the improvement of the immediate and long-term end results of cancer management. In the past, clinicians relied on the laboratory to help in the recognition and treatment of the gross disturbances of metabolic balance that arose as the result of mechanical obstruction of the major systems by cancer, such as the uretus, bile ducts and intestinal tract. Equally, it has long been known that cancer may produce a variety of paraneoplastic syndromes and disturbances of normal regulatory mechanisms. Fever, cachexia, dermatomyositis, hypertrophic pulmonary arthropathy, neuropathy, defects of bone marrow cell production and coagulation. Some of the mechanisms of these complications can be explained biochemically, others cannot; if we were able to identify the regulatory molecules, it might be an advance in medicine.

HEPATOMA WITH DYSPROTEINEMIA AND ERYTHROCYTHEMIA.

PRIMARY carcinoma of the liver has been associated with a variety of paraneoplastic syndromes, suggesting various metabolic, endocrine, hematologic, vascular, and osseous diseases.1The reported association of hepatoma and dysproteinemia has been rather infrequent. In 1962 Viallet et al reported a single case and were able to collect only three additional cases from the literature.2-5On the other hand, secondary polycythemia in association with hepatoma has been reported with greater frequency.6-11The observation of a patient with hepatoma showing both dysproteinemia and erythrocythemia forms the basis for this report. The present patient, additionally, showed significant plasmacytic reaction in the tumor as well as in the bone marrow. Report of Case A 58-year-old Negro laborer was first admitted to the Veterans Administration Hospital, Washington, DC, on July 5, 1960, because of weakness of the left arm. He had sustained a fall four months previously, bruising the deltoid region