Paraneoplastic rheumatic syndromes

Paraneoplastic rheumatic syndromes are challenging from both a clinical and research standpoint. Progress over the past decade has provided clarification of clinical syndromes. At the same time, our increasing ability to define and quantitate mediators of inflammation is shedding new light on pathogenesis. In turn, this understanding may answer questions regarding more common rheumatic diseases.     

Paraneoplastic rheumatic syndromes

Paraneoplastic symptoms caused by a malignancy but not directly related to tumour invasion are the result of a wide variety of tumour-derived biologic mediators, such as hormones, peptides, antibodies, cytotoxic lymphocytes, autocrine and paracrine mediators. Recognition of paraneoplastic syndromes is important, as it may lead to an early diagnosis of cancer. On the other hand, the clinical severity of the symptoms can be used as a guide to the extent of response to underlying tumour therapy. The quality of life of the patient is affected, therefore the palliative treatment of paraneoplasia is very important.     

Eosinophilic cystitis: eight cases report and literature review

Historically, eosinophilic cystitis is a rare disorder of bladder inflammation with eosinophils infiltration diagnosed by pathologic examination. The etiology is unclear despite the past identification of many factors contributing to this disease. Eight patients with eosinophilic cystitis were reported. The intact history, clinical manifestation, radiological examination and response to therapy were all evaluated. The results showed that 7 patients developed hematuria, 6 patients were with dysuria, 4 patients with frequency and 4 patients with urine retention. Seven patients had abnormal urinalysis but no positive finding in culture. Radiological findings revealed that one patient had bladder mass lesions and upper urinary tract dilation. Cystoscopic examination was performed in every patient and showed mass-like, edematous, ulcerative or hyperemic mucosa lesions. Cold-cup biopsy or transurethral resection of bladder lesions were all performed and could be the first priority to be considered. However, partial or total cystectomy should be taken into consideration when simple treatment failed to resolve this problem. Additionally, antihistamines, steroids or antibiotics are given to control the clinical symptoms. The results of these treatments were good except for one case who suffered from recurrence but recovered after simple operation and oral therapy. Although good results were found concerning treatment, long-term follow-up is necessary.     

Paraneoplastic rheumatic syndromes.

Malignant neoplasms are associated with a wide variety of paraneoplastic rheumatological syndromes. Among these, hypertrophic osteoarthropathy, carcinoma polyarthritis, dermatomyositis/polymyositis, and paraneoplastic vasculitis are the most frequently recognized. Other less known associations are based upon a smaller number of reported patients, and include fasciitis, panniculitis, erythema nodosum, Raynaud's syndrome, digital gangrene, erythromelalgia and lupus-like syndromes. Musculoskeletal manifestations of malignancy may coincide, follow or antedate the diagnosis of cancer, or herald its recurrence. The clinical course generally parallels that of the primary tumour, and treatment of the underlying malignancy often results in regression of the rheumatic disorder. Awareness that cancer can cause certain non-metastatic symptoms is important for early diagnosis and treatment of an occult neoplasm. Rheumatic manifestations suggesting a hidden cancer include: rapid onset of an unusual inflammatory arthritis clubbing or diffuse bone pains in a patient 50 years of age or older, chronic unexplained vasculitis, refractory fasciitis, Raynaud's syndrome unresponsive to vasodilator therapy, rapidly progressive digital gangrene or Lambert-Eaton myasthenic syndrome. Management consists of control of the underlying cancer and symptomatic treatment of the rheumatic syndrome with non-steroidal anti-inflammatory drugs or corticosteroids.     

Paraneoplastic neurological syndromes associated with gastric cancer: a case report and review of the literature

Paraneoplastic neurological syndromes (PNS) are associated with small-cell lung cancer, breast and gynecological cancers. We describe a gastric neoplasm presented with neurological symptoms. A 74-year-old male presented with tonic?Cclonic seizures. Initial investigations were normal; however, brain magnetic resonance imaging showed abnormal signal intensity in the hippocampi. A diagnosis of PNS was suspected. The patient was then diagnosed with a gastric neuroendocrine carcinoma with N-type voltage-gated calcium channel antibodies. The neurological impairments improved after the primary was resected and the patient remains free of cancer and paraneoplastic syndrome. We reviewed 10 cases of PNS associated with gastric cancer and found several characteristics: (1) older men, (2) neuroendocrine component or predominance, (3) oncological outcome for patients with PNS is better than for patients without PNS, and (4) neurological impairment is diagnosed 6?months prior to the diagnosis of gastric malignancy. In conclusion, elderly men with symptoms suggestive of PNS should be investigated for a gastric neuroendocrine malignancy.     

Paraneoplastic limbic encephalitis--case report and review of literature

We report a 77-year old man who presented with a sub-acute dementia associated with aggressive behaviour, ataxia, rapid progression and death. No cause for his illness could be detected in his lifetime, but at post mortem he was found to have paraneoplastic limbic encephalitis and a bronchogenic tumour. A diagnosis of paraneoplastic limbic encephalitis should be considered in the differential diagnosis of unexplained dementias and appropriate investigations performed to diagnose the condition.     

新生儿狼疮综合征八例报告并文献复习

目的探讨新生儿狼疮综合征(NLS)的发病机制、血清学改变、临床表现和对策。方法确定NLS的条件：①新生儿先天性心脏传导阻滞和新生儿或母亲的抗SSA、抗SSB抗体阳性；②确定的和NLS相关的皮肤损害和新生儿或母亲的抗SSA，抗SSB，或抗-U1RNP抗体阳性。总结分析8例NLS患儿和母亲的临床表现，检测母亲和患儿的自身抗体，进行长期随访，并复习相关文献。结果8例NLS中皮肤损害8例，血液学改变7例，心脏受损3例，肝脏受损3例，抗核抗体(ANA)阳性8例，抗SSA抗体阳性8例，抗SSB抗体阳性2例，抗dsDNA抗体阳性1例．抗ENA抗体阳性1例，血沉(ESR)增快2例，血清丙氨酸转氨酶增高3例。8例母亲中孕前7例无症状，1例日光性皮炎：孕3-5个月时5例母亲出现目光性皮炎：1例发热、脱发、全血细胞减少：产后1个月1例母亲出现皮疹、脱发；抗核抗体阳性8例，抗SSA抗体阳性8例，抗SSB抗体阳性2例，抗dsDNA抗体阳性1例，抗ENA抗体阳性1例：ESR增快2例。8例NLS中7例随访11个月～7年，心电图完全右束支传导阻滞1例失访。7例皮疹于3-6个月消退，自身抗体于6-9个月转阴，血液学改变、肝脏损害恢复正常、8例母亲中1例失访；6例确诊为SLE，1例确诊为SS。结论NLS是获得性的自身免疫疾病，NLS临床表现主要有免天性心脏传导阻滞、短暂性皮肤狼疮损害、血细胞减少、肝脏损害等．肾脏受损少见。抗SSA(特别是抗-52000SSA)或抗SSB抗体阳性的母亲是NLS的危险因素。多数NLS预后良好。     

Castleman's disease and lymphoma: report of eight cases in HIV-negative patients and literature review

Castleman's disease (CD) is a rare atypical lymphoproliferative disorder associated with a risk of developing malignant lymphoma. We have recorded 8 HIV-negative patients presenting this association, 6 with non-Hodgkin's lymphoma (NHL) and 2 with Hodgkin's disease (HD). After literature review, we analyzed all reported cases of association CD-NHL (n = 23) and CD-HD (n = 27). NHL is more often associated with multicentric CD, its diagnosis being concurrent with CD diagnosis or occurring within 2 years. B-NHL is predominant (71%), and mantle cell lymphoma represents 40% of these B-NHL cases. NHL displays an aggressive course and is liable for death, especially in multicentric CD. HD occurs in localized CD of plasma cell type, usually in the same areas, is more often of interfollicular subtype, and its clinical course seems better than NHL. The association of CD and lymphoma seems to be more than coincidental, and its pathogenesis is discussed.     

Non-Hodgkin's lymphoma presenting as an endobronchial tumor: report of eight cases and literature review

Non-Hodgkin's lymphoma (NHL) involving the endobronchial tree is uncommon, and the initial presentation of NHL as an endobronchial tumor is extremely rare. In a series of 441 patients with newly diagnosed non-Hodgkin's lymphoma over a 7-year period, we reviewed the clinical features of eight patients who presented with an endobronchial tumor. All patients had local pulmonary disease without extrathoracic involvement. The major presenting symptoms were dyspnea, chest pain, cough, and hoarseness. None of the patients had systemic symptoms. Radiographs revealed lobar collapse in all cases. Five patients had mediastinal masses and three had isolated endobronchial lesions. Although MALT lymphoma is the most common primary pulmonary lymphoma, it was present in only one of our patients, while seven patients had aggressive lymphoma. All patients received chemotherapy. Six of the eight patients responded favorably to treatment with complete remission. The prognosis of patients with isolated endobronchial lymphoma is not worse than other local presentations of lymphoma. Bronchoscopic examination with biopsy is essential to differentiate these lesions from primary bronchongenic carcinoma.     

Esophageal endocrine cell carcinoma: a report of eight cases and a review of the literature

Esophageal endocrine cell carcinoma is a rare histopathological type thought to be highly malignant and to have a poor prognosis, but its clinical behavior has not been fully analyzed. We retrospectively reviewed our cases among 386 patients with esophageal cancer in our institution. Eight patients (2.1 %) were pathologically diagnosed with endocrine cell carcinoma. Median patient age was 63 years, and the male-to-female ratio was 7:1. Cancer stage according to the Japanese Classification of Esophageal Cancer (JCEC) 10th edition, was stage II in one case, III in three, IVa in one, and IVb in three. Chromogranin-A staining was positive in six cases, synaptophysin in six, cluster of differentiation (CD)56 in seven, and neuron-specific enolase (NSE) in seven. Treatment included surgery in two cases, chemoradiotherapy in four, best-supportive case in one, and rejection in one. Prognoses were 13 months after surgery for stage II, 6–28 months after chemoradiotherapy for stage III, and 0–10 months for stage IV.     

Cervical fracture complicating ankylosing spondylitis: A report of eight cases and review of the literature

Fracture of the cervical spine is a serious and often fatal complication of ankylosing spondylitis. An evaluation of eight patients and a review of 75 additional cases from the literature are presented. Although this complication is relatively uncommon, it is clear that people with advanced disease and complete ankylosis of the cervical spine are at increased risk of sustaining cervical fracture. When fracture occurs it usually stems from minor trauma resulting most commonly in disruption of the lower cervical segments (fifth through the seventh cervical vertebrae). Fracture is most likely the result of a hyperextension type injury, occurs through what was formerly an intervertebral space, and is unstable. Severe neurologic sequelae occur in 57 percent of the cases and the mortality rate (35 percent) is twice that observed with similar fracture involving normal spines. The majority of patients are best treated with closed reduction with halo traction together with body cast or jacket. Laminectomy is rarely indicated except in the event of an advancing neurologic deficit. With appropriate understanding and execution of management principles, the outcome in these patients can be favorable. Unfortunately, recognition of cervical fracture in patients with ankylosing spondylitis is often needlessly delayed. Distortion of normal anatomy in spondylitics, predominant fracture location in lower cervical spine segments and lack of obvious displacement make identification difficult. Thus, management is often inappropriate resulting in exessive neurologic injury and mortality.     

Paraneoplastic polyarteritis nodosa with cerebral masses: case report and literature review

Polyarteritis nodosa (PAN) as a paraneoplastic vasculitis is rarely described, especially in association with squamous cell carcinoma (SCC). Furthermore, only 5% of all PAN patients have central nervous system (CNS) involvement, almost exclusively in the form of cerebral infarction or intracerebral haemorrhage. We report the first case of PAN with multiple immunosuppressant‐responsive, cerebral vasculitic lesions in association with metastatic SCC.     

Bacterial tracheitis: report of eight new cases and review

Bacterial tracheitis, previously referred to as nondiphtheritic laryngitis with marked exudate, was commonly discussed in pediatric textbooks before 1940. It seemed to disappear as a clinical entity after that time, but it has been recorded with increasing frequency in the pediatric literature since 1979. We describe eight new cases and review 110 previously described cases. The clinical course consists of a prodromal upper respiratory illness with stridor, fever, and a variable degree of respiratory distress. Unlike patients with croup, patients with bacterial tracheitis do not respond to aerosolized racemic epinephrine. Most patients require endotracheal intubation; some require tracheostomy. Reported complications include pneumonia, pneumothorax, formation of pseudomembranes, toxic shock syndrome, and cardiopulmonary arrest. Bacterial tracheitis is a secondary bacterial infection following a primary viral respiratory infection. The most common preceding viral infection is parainfluenza. Staphylococcus aureus and Haemophilus influenzae are the predominant causes of bacterial tracheitis. Secondary bacterial infection may occur as a result of tracheal mucosal injury or impairment of normal phagocytic function due to viral infection.     

Adenosine-induced atrial fibrillation during pharmacologic stress testing: report of eight cases and review of the literature

Adenosine-induced atrial fibrillation has been described in the setting of treatment of supraventricular tachycardia, but has been rarely reported during adenosine infusion for pharmacologic stress testing. We present 8 patients who developed atrial fibrillation during adenosine stress testing. The incidence of this arrhythmia was 0.41% in our laboratory. Atrial fibrillation was often preceded by frequent atrial premature beats and/or AV block, and the duration ranged from 15 seconds to 6 hours. All patients converted spontaneously to normal sinus rhythm. Atrial fibrillation is a relatively rare arrhythmic complication of adenosine infusion, and can be managed expectantly, without need of cardioversion.     

Inflammatory pseudotumor of the liver: report of eight cases, including three unusual cases, and a literature review

BACKGROUND: Inflammatory pseudotumor (IPT) of the liver is a rare but increasingly recognized tumor-like lesion. This condition is becoming an important differential diagnosis in patients with hepatic space-occupying lesions. This study reports the clinical features of eight cases of IPT of the liver, including the first report of three cases with an unusual associated disease or clinical course. METHODS: The study sample included cases of IPT diagnosed based on the histopathology in our institution from 1995 to 2004. Clinical events were reported and compared with reports in the literature. RESULTS: The age of the patients ranged from 28 to 78 years. The most common symptoms were abdominal pain, fever and bodyweight loss. Three patients were diagnosed incidentally. The initial clinical diagnoses were hepatocellular carcinoma in three cases, liver abscess in two cases, metastasis in one case and uncertain nature in two cases. Two cases were associated with preceding endophthalmitis and cholangiocarcinoma, respectively. Two patients had spontaneous lesion regression, but one of these had possible recurrence 10 months later. The other two patients experienced resolution of their lesions following antibiotic treatment. Four patients underwent surgical resection with subsequent recovery. CONCLUSIONS: The clinical and imaging features of IPT in this small series were found to sometimes mimic those of malignant liver tumors or abscess. Surgical resection was performed in half of the cases. However, IPT of the liver could resolve spontaneously or following antibiotics treatment.     

Collision sellar lesions: experience with eight cases and review of the literature

The concomitant presence of a pituitary adenoma with a second sellar lesion in patients operated upon for pituitary adenoma is an uncommon entity. Although rare, quite a great variety of lesions have been indentified coexisting with pituitary adenomas. In fact, most combinations have been described before, but an overview with information on the frequency of combined pathologies in a large series has not been published. We present a series of eight collision sellar lesions indentified among 548 transsphenoidally resected pituitary adenomas in two Neurosurgical Departments. The histological studies confirmed a case of sarcoidosis within a non-functioning pituitary adenoma, a case of intrasellar schwannoma coexisting with growth hormone (GH) secreting adenoma, two Rathke’s cleft cysts combined with pituitary adenomas, three gangliocytomas associated with GH-secreting adenomas, and a case of a double pituitary adenoma. The pertinent literature is discussed with emphasis on pathogenetic theories of dual sellar lesions. Although there is no direct evidence to confirm the pathogenetic relationship of collision sellar lesions, the number of cases presented in literature makes the theory of an incidental occurrence rather doubtful. Suggested hypotheses about a common embryonic origin or a potential interaction between pituitary adenomas and the immune system are presented.     

Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.