A case of malignant peritoneal mesothelioma successfully treated with systemic chemothrapy

A 64-year-old man with a 2-month history of abdominal distension was admitted for transient cerebral ischemic attack. A CT scan revealed massive ascites. Laparoscopy showed multiple whitish nodules on the visceral peritoneum and the omentum. Peritoneal biopsy revealed tumor cells consistent with malignant peritoneal mesothelioma (MPeM). Pemetrexed in combination with cisplatin was administered because it has been reported to be active in patients with MPeM. However his disease progressed. As second-line therapy paclitaxel was tried which yielded a complete response (CR). Eighteen months later he developed abdominal pain of the right upper region where a CT scan showed a mass with surrounding inflammation. As third-line therapy, gemcitabine was administered and again resulted in a CR. He is alive at 3 years from first presenting. Searches for case studies published in medical journals on MPeM were carried out, and 59 cases were analyzed in comparison with this case.     

Peritoneal sarcoidosis: case report and review of the literature.

OBJECTIVES: This study was performed to report a patient with peritoneal sarcoidosis and review the literature for similar cases. METHODS: We described the clinical presentation, course, and outcome of the patient, and reviewed the medical literature from 1966 till 1997 using MEDLINE and the key words sarcoidosis, scar, and peritoneum. RESULTS: Our patient presented with a rapidly growing tumor-like mass at the site of an old appendectomy scar. Laparoscopy showed a large peritoneal mass and multiple small peritoneal nodules that were found to be noncaseating granulomas by pathology. The MEDLINE search uncovered only 16 cases of peritoneal sarcoidosis, most of which presented with ascites. CONCLUSION: This case illustrates the need to consider sarcoidosis, in addition to infections and neoplasms, in the differential diagnosis of peritoneal nodules and exudative ascites.     

A case of Sj?gren syndrome associated with sarcoidosis]

A 70 year-old female with a 6-year history of primary Sj?gren syndrome was admitted with fever and loss of weight. Examinations such as the lip biopsy reconfirmed the diagnosis of primary Sj?gren syndrome. Abdominal CT and gallium scan showed lymphadenopathy in the abdominal cavity, and an exploratory biopsy was done. Lymph nodes and liver biopsy specimens revealed multiple noncaseating granulomata with some Langhans' giant cells, consistent with sarcoidosis. It has been previously proposed that connective tissue disease and sarcoidosis are related in terms of pathogenesis because both diseases share common immunological features and abnormalities in regard to cellular and humoral immunity. Indeed some data in the literature show a striking similarity between the lymphocyte subpopulation in the parotid gland of Sj?gren syndrome and the bronchoalveolar lavage fluid of sarcoidosis. However, it is difficult to assume the same pathogenesis for both diseases, because this aspect of the diseases has not been completely clarified. Despite the fact that the two share common immunological features and abnormalities, the coexistence of primary Sj?gren syndrome and sarcoidosis has rarely reported.     

Identification of propionibacterium acnes in a case with stage III pulmonary sarcoidosis involving hepatosplenic disease]

A 35-year-old woman was admitted to our hospital because of an abnormal chest radiograph. Chest X-ray on admission showed multiple small nodular shadows in both lung fields but no bilateral hilar lymphadenopathy (BHL). Moreover, abdominal CT showed some nodules in the liver and spleen, and serum ACE was slightly increased to 23.3U/L (normal range: 8.3-21.4U/L). Transbronchial lung biopsy and liver biopsy resulted in a diagnosis of stage III pulmonary sarcoidosis with hepatosplenic disease. Histopathological findings demonstrated non-caseating epithelioid cell granulomas with giant cells in both specimens. Interestingly, propionibacterium acnes (P. acnes), the possible pathogen of sarcoidosis, was detected in giant cells in the lung and epitheliod cell granuloma of liver tissue. This case was of interest considering P. acnes might have been the causative pathogen.     

56例结核性腹膜炎患者CT影像分析

目的 探讨结核性腹膜炎（TBP）特征性CT影像表现及治疗中影像动态变化的特点。方法 回顾性分析56例临床及病理确诊的TBP患者,观察分析CT影像特征,包括腹腔积液、腹膜（壁腹膜、肝包膜、大网膜及肠系膜）及腹腔淋巴结。56例均行CT平扫+增强扫描。治疗过程中收集动态复查CT影像。结果 腹腔积液45例,大量1例,中少量44例,限制性38例,CT值>20 HU者34例。壁腹膜增厚46例,肝脏边缘部腹膜均有受累,均匀增厚17例,扁丘状凸起或腹膜结核瘤29例,增强扫描结核瘤边缘强化,肝包膜或肝脏受累15例;网膜增厚35例,结节及斑片34例,网膜饼1例,增强扫描网膜饼呈轻度不均匀强化;肠系膜增厚41例,表现为斑片、结节及线状、星芒状条索状高密度影,与肠袢粘连,增强扫描肠系膜血管包埋其内,走形扭曲。腹腔及腹膜后淋巴结增大19例,伴钙化5例,簇集状排列,增强扫描环状或蜂窝状强化。17例完整的动态CT图像,腹腔积液吸收最快,网膜及肠系膜病变吸收稍慢,腹膜结核瘤缩小速度最慢。结论 TBP的CT诊断为多种征象的综合,中少量腹腔积液伴壁腹膜、网膜及肠系膜的增厚粘连是最为常见的影像表现;CT影像动态变化对临床评价疗效具有重要意义。     

Case report: peritoneal sarcoidosis in an unusual location

A 48-year-old woman underwent surgery for a lesion seen on ultrasound and interpreted as a uterine myoma. The surgery revealed multiple nodules that had seeded on the omentum, peritoneum, and ovaries. The macroscopic interpretation was either metastasis or tuberculosis. The biopsy showed noncaseating granulomas, and a diagnosis of peritoneal sarcoidosis was reported. The AFB (acid fast bacillus) and L?wenstein-Jensen culture were negative. She was treated with methylprednisolone for 1 year for pulmonary sarcoidosis progression, with a resulting decrease in her DLCO (diffusing lung capacity for carbon monoxide). Computed tomography showed only a slight decrease in the multiple nodules and cysts. She is currently symptom-free.     

C-C chemokine receptor 2 and sarcoidosis: association with Lofgren's syndrome

Sarcoidosis is thought to result from the interaction between an unknown environmental antigenic trigger and the host's genetic susceptibility. We hypothesized that sarcoidosis, or one of the disease subsets, could be associated with single nucleotide polymorphisms of C-C chemokine receptor 2 (CCR2) gene. Eight single-nucleotide polymorphisms in CCR2 were studied in a total of 304 Dutch individuals (90 non-L?fgren sarcoidosis, 47 L?fgren's syndrome, 167 control subjects). From the investigated CCR2 polymorphisms, nine haplotypes were deduced (haplotypes 1-9). In patients with L?fgren's syndrome, a strongly significant increase in the frequency of CCR2-haplotype 2, which includes four unique alleles (A at nucleotide position -6752, A at 3,000, T at 3,547, and T at 4,385), was observed compared with control subjects (74% vs. 38% respectively, p < 0.0001), whereas no difference was found between non-L?fgren sarcoidosis and control subjects (both 38%). The association between CCR2-haplotype 2 carriage frequency and L?fgren's syndrome (odds ratio, 4.4; p < 0.0001) remained significant after adjustment for human leukocyte antigen haplotype DRB1*0301-DQB1*0201 (odds ratio, 11.5; p < 0.0001) and female sex (odds ratio, 3.2; p = 0.003), two known risk factors for L?fgren's syndrome. In conclusion, this report describes a strong association between CCR2-haplotype 2 and L?fgren's syndrome. Further studies are needed to understand the molecular mechanisms underlying this association.     

Contrast-enhanced ultrasonography in hepatosplenic sarcoidosis

We report a case of a 38-year-old woman with atypical pain in the left lower hemi-abdomen. On abdominal B-mode ultrasonography the liver was normal; the spleen showed multiple subcentimetric hypoechoic nodules. A multidetector CT-examination revealed multiple small low-attenuation nodules in the liver and the spleen, suggestive for metastatic disease. Contrast-enhanced ultrasound (CEUS) revealed two hypoechoic nodules in the liver that were visible in the portal phase and disappeared in the late phase. The focal splenic lesions were visible as irregular hypo-enhancing nodules. An MRI examination, including T1, T2 and contrast-enhanced images, could not confirm the exact nature of the lesions. A core biopsy of a splenic nodule yielded non-caseating epithelioid cell granulomas. Different complementary examinations were normal and hepatosplenic sarcoidosis was diagnosed. The pain in the left lower hemi-abdomen was ascribed to irritable bowel syndrome.     

CT平扫对结核性腹膜炎的诊断价值

目的 探讨结核性腹膜炎的CT平扫表现特征。方法 回顾性分析46例有CT扫描资料的结核性腹膜炎患者,其中经手术病理证实6例,腹腔穿刺证实2例,腹腔镜检证实4例,试验性治疗诊断34例。观察内容包括结核性腹膜炎累及壁腹膜、大网膜、肠系膜及腹膜后间隙的CT表现特征。结果 （1）壁腹膜增厚33例,其中均匀、光滑增厚27例,局部不规则增厚6例;（2）腹腔积液31例,其中20例为少量积液;（3）大网膜增厚26例,表现为污迹样增厚22例,饼状增厚2例;（4）肠系膜受累32例,其中9例合并小结节影：（5）淋巴结增大16例;（6）肠壁增厚1例,肠间距增宽2例。结论 结核性腹膜炎CT平扫的主要表现有少量腹腔积液、均匀性腹膜增厚、大网膜污迹样增厚,以及肠系膜、肠管受累与腹腔淋巴结增大,CT平扫能为结核性腹膜炎的诊断提供重要信息。     

Sarcoidosis with multiple organ involvement emerging as Löfgren's syndrome

A 52-year-old woman was admitted because of high-grade remittent fever, erythema nodosum, and arthritis which had been lasting two months. Antibiotics did not improve her condition. A chest CT scan examination revealed bilateral hilar and mediastinal adenopathy and multiple nodular opacities in the bilateral lungs. The wedge biopsy of the right lower lobe using video-assisted thoracoscopy presented the histological findings of sarcoidosis. Finally, this case fulfilled the criteria of L?fgren's syndrome. Due to the uncovered cardiac involvement, the systemic glucocorticoid therapy had to be initiated. This case suggests that atypical forms of sarcoidosis should be kept in mind as well, when facing cases with unknown fever.     

Primary peritoneal sarcoidosis

Summary A 14-year-old male was admitted for the evaluation of tense exudative ascites. Despite thorough evaluation, the diagnosis remained cryptic until peritoneoscopy revealed diffuse studding of the entire visualized peritoneum with multiple miliary nodules, and peritoneal biopsy demonstrated multiple noncaseating epithelioid granulomas. After other causes were excluded, a diagnosis of sarcoidosis was considered and confirmed with classic endobronchial findings at bronchoscopy. Involvement of the peritoneum with sarcoidosis is rare and, to our knowledge, only one other case describes this as the initial manifestation of this disease.     

Liver cirrhosis with encapsulating peritoneal sclerosis after 4 years of peritoneal dialysis: A case report

Rationale:Encapsulating peritoneal sclerosis (EPS), or abdominal cocoon, is a rare but fatal syndrome characterized by intestinal obstruction owing to adhesions in a diffusely thickened peritoneum. Long-term peritoneal dialysis (PD) for more than 5 years is commonly associated with EPS, while liver cirrhosis also carries a risk of EPS. However, there have been only a few reports that describe a case of EPS complicated with both cirrhosis and PD. We herein describe a case of advanced liver cirrhosis with end-stage renal disease (ESRD) who developed EPS after 4 years of PD and who was successfully recovered by surgery.Patient concerns:A 58-year-old man with alcoholic liver cirrhosis suffered abdominal pain. The patient had a 4-year history of continuous cycling PD to manage ESRD as well as cirrhotic complications of refractory ascites and hypotension. Laboratory test results showed increased levels of inflammation, and contrast-enhanced computed tomography scan showed dilated loops of small bowel proximal to the site of intestinal obstruction. The patient was suspected to have developed intestinal obstruction owing to EPS. The patient discontinued continuous cycling peritoneal dialysis and switched to hemodiafiltration.Diagnoses:Laparoscopy revealed a whitish membranous material wrapped around the bowel, especially at the terminal ileum with a narrowed portion, consistent with EPS.Interventions:Repeated decortication of fibrous peritoneal membranes successfully released the intestinal obstruction.Outcomes:The postoperative course went well and abdominal pain remained in remission. Because abdominal distension owing to ascites got intolerable in a few days after surgery, a PD catheter was re-inserted and ascitic fluid drainage was resumed with peritoneal lavage. The patient continued hemodiafiltration using vasopressor agents.Lessons:The Cirrhotic patient with ESRD undergoing PD could develop EPS after a short duration of PD.     

A rare case of sarcoidosis with bilateral leg lymphedema as an initial symptom

A 55-year-old man was admitted to our hospital because of bilateral leg lymphedema. He also showed subcutaneous nodules and CT scan disclosed multiple enlarged lymph nodes at thoracic, abdominal, and inguinal areas. Biopsy of the inguinal lymph node and the subcutaneous nodule revealed noncaseating epithelioid cell granuloma, a finding consistent with sarcoidosis. Lymphedema was attributed to the blockade of lymph flow by the systemic lymph node involvement of the disease. Within 1 week after the initiation of steroid therapy, his leg edema disappeared. Lymphedema could be the initial symptom of systemic sarcoidosis.     

Sarcoidosis with tumorous hepatic and bone lesions mimicking disseminated malignancy: a case report.

An asymptomatic 49-year-old woman with a diffuse abnormal shadow detected on a routine chest rentogenogram was referred for evaluation. Ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) revealed space-occupying lesions in the liver and spleen. Lymphadenopathy was observed in the mediastinum and abdomen on CT, and multiple lesions of low signal intensity were observed in the spine on MRI. Furthermore, the presence of multiple tumorous nodules in the liver suggesting metastatic hepatic cancer was demonstrated on laparoscopy. Biopsy of the liver tumors revealed sarcoidosis; transbronchial biopsy specimens showed identical histological features. After administration of prednisolone for one month, the space-occupying lesions in the liver, spleen and spine improved. This case of sarcoidosis exhibited hepatic tumorous nodules and space-occupying lesions in the spleen and the spine which mimicked the presence of metastatic hepatic cancer.     

Sarcoidosis with tumorous hepatic and bone lesions mimicking disseminated malignancy: A case report

An asymptomatic 49-year-old woman with a diffuse abnormal shadow detected on a routine chest rentogenogram was referred for evaluation. Ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) revealed space-occupying lesions in the liver and spleen. Lymphadenop athy was observed in the mediastinum and abdomen on CT, and multiple lesions of low signal intensity were observed in the spine on MRI. Furthermore, the presence of multiple tumorous nodules in the liver suggesting metastatic hepatic cancer was demonstrated on laparoscopy. Biopsy of the liver tumors revealed sarcoidosis; transbronchial biopsy specimens showed identical histological features. After administration of prednisolone for one month, the space-occupying lesions in the liver, spleen and spine improved. This case of sarcoidosis exhibited hepatic tumorous nodules and space-occupying lesions in the spleen and the spine which mimicked the presence of metastatic hepatic cancer.     

A case of acute sarcoidosis with polyarthralgia and erythema nodosum; L?fgren's syndrome]

A 49-year-old woman was admitted to our hospital with a dry cough and bilateral hilar lymphadenopathy. She had a history of acute arthralgia and erythema nodosum 4 months before admission. Chest CT scans on admission revealed multiple mediastinal lymphadenopathy. Broncho-alveolar lavage showed CD4-dominant lymphocytosis. A biopsy specimen of the lung revealed non-caseating granuloma of epithelioid cells with giant cells, confirming the diagnosis of sarcoidosis. She recovered in one month, and has since remained well and free of the symptoms. L?fgren's syndrome is acute sarcoidosis, characterized by polyarthralgia, erythema nodosum and bilateral hilar lymphadenopathy. It is common in European countries, but very rare in Japan, from where only two case reports have appeared. This syndrome is closely related to HLA-B8, DR-3, and DR-17, but our case did not have these HLA loci. We report this case as the third case of L?fgren's syndrome in Japan.     

Nodular cerebral sarcoidosis simulating metastatic carcinoma

A 51-year-old woman with known sarcoidosis was admitted to the hospital with grand mal seizures. The enhanced cranial computed tomographic (CT) scan showed bilateral, discrete, nodular lesions simulating metastatic carcinoma. Results of a workup for malignant neoplasm and inflammatory disorders were normal. Diagnosis of nodular cerebral sarcoidosis was made, and the patient received prednisone therapy. After four months of therapy, the CT scan showed resolution of the nodules.     

ACTH independent Cushing's syndrome occurring in siblings

Familial Cushing's syndrome due to ACTH independent bilateral macronodular adrenocortical hyperplasia occurring in siblings is reported. The proband was a 69-year-old woman who presented with a typical Cushingoid appearance. The serum cortisol level was elevated, with a loss of diurnal rhythm, and the plasma ACTH level was undetectable. Dynamic testing showed no suppression of urinary 17-OHCS by high dose dexamethasone and no stimulation by metyrapone. An abdominal CT scan showed bilateral adrenal enlargement. The patient died of a subarachnoid haemorrhage, and autopsy revealed a massively thickened adrenal cortex composed of nodules up to 3.5 cm in diameter. A pituitary adenoma was not found. We learned that the patient's elder brother was also diagnosed at 59 years of age with Cushing's syndrome due to bilateral macronodular adrenocortical hyperplasia. His plasma cortisol levels were not suppressed by high dose dexamethasone and the plasma ACTH level was undetectable. Screening of the available family members by administering 1 mg dexamethasone at midnight and performing abdominal CT scan revealed impaired suppressibility of serum cortisol associated with enlarged bilateral adrenal glands in a 64-year-old sister and a 54-year-old brother. The 64-year-old sister was considered as a possible 'affected' case in the early stages of development, because the basal level of ACTH was not suppressed and hyperplasia of the bilateral adrenal glands as revealed by CT scan was less evident.     

Löfgren syndrome in Turkey

Abstract
In the 36-year period between 1966 and 2002, 514 patients were diagnosed with sarcoidosis at Cerrahpala Medical Faculty, Gstanbul, Turkey, and of these 98 (19.1%) had Löfgren syndrome. The frequency of female patients with Löfgren was higher than the frequency among other sarcoidosis patients (female : male ratio 4.8 vs . 1.64; P  < 0.001). Erythema nodosum was diagnosed in 72.4% of the subjects and arthritis or arthralgia was diagnosed in 51%. Erythema nodosum and arthritis or arthralgia were more frequent in Löfgren; however, pulmonary parenchymal involvement was more frequent in other sarcoidosis patients (all P ‐values < 0.001). (Intern Med J 2003; 33: 535−537)     

A rare complication of a common disease： Bouveret syndrome, a case report

This is a case report of an 85-year old patient who presented with abdominal pain, nausea and vomiting associated with altered liver function test. The plain X-rays and CT scan showed pneumobilia with an ectopic gallstone. The patient was diagnosed with Bouveret syndrome and managed surgically. The report is followed by a discussion about Bouveret syndrome.