Sarcoidosis diagnosed in a patient with known HIV infection

A 29-year-old black man with HIV infection had an abnormal chest x-ray film with bilateral hilar adenopathy. Sarcoidosis was suspected, but a thorough and comprehensive evaluation was completed to differentiate the multiple infectious and noninfectious causes of these findings. Biopsy of a hilar node and pulmonary tissue revealed sarcoidosis.     

A suspected case of perforation of a lymph node into the bronchus during the treatment of adult hilar lymph node tuberculosis]

A 27-year old patient was diagnosed as having post-primary hilar lymph node tuberculosis. First being admitted to the hospital with a high fever, a chest x-ray examination revealed a swelling of the left hilar lymph nodes and a sputum smear tested positive for acid-fast bacilli. Neither regular clinical examination or investigation had reported abnormality. The acid-fast bacilli was successfully treated through treatment using INH RFP SM. However, after two months, swelling was observed in the right para-tracheal lymph nodes, Further, a bronchoscopic examination revealed polyp-like tumors at the left upper and lower bifurcation. The swelling of the para-tracheal lymph nodes was considerably reduced and the tumors non-existent after five months. These lymph node reactions could have likely been a part of the so called early exacerbation. The polyp-like tumors were not found during the bronchoscopy performed during admission to the hospital. It is therefore suspected that the cause was perforation of the hilar lymph node into the bronchus.     

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.     

A case of resected squamous cell carcinoma of the lung complicated with sarcoidosis]

A 64-year-old man with uveitis was admitted to our hospital for detailed investigation of an abnormal shadow on his chest X-ray. Chest radiography and computed tomography of the chest showed mediastinal lymphadenopathy and a tumor shadow in the left hilum. Transbronchial tumor biopsy revealed squamous cell carcinoma. Left upper lobectomy and drainage of bilateral hilar and mediastinal lymph nodes were performed. Histopathological examination revealed the coexistence of squamous cell carcinoma with many non-caseating epithelioid cell granulomas in all hilar and mediastinal drainage lymph nodes, but no metastasis. Non-caseating epithelioid cell granulomas were also seen in the interstitium and alveolar spaces. Coexistence of sarcoidosis and lung cancer in the same patient is not common, and only 29 cases, including ours, have been reported. This case also provides the concept that surgical tumor resection should be considered even if bilateral mediastinal lymphadenopathy is found in a case of lung cancer complicated with sarcoidosis.     

BALF lymphocyte CD 4/8 ratio in a case of sarcoidosis with radiological resolution]

A 60-year-old man was given a diagnosis of pulmonary and ocular sarcoidosis. His radiological abnormalities and visual disturbance resolved after two years. Ten years later, at age 70, squamous cell carcinoma was detected in the right lower lobe of the lung. Lymphocyte CD4/8 ratio in bronchoalveolar lavage fluid has remained at a high level for the previous 10 years and was 7.41 immediately before the operation. Chest CT and gallium scintigram could not detect abnormalities in mediastinal or hilar nodes. The tumor and right lower lobe were resected with hilar and mediastinal lymph nodes. Histological examination revealed that there were numerous epithelioid cell granulomas in mediastinal and hilar lymph nodes. BALF analysis reflects residual granulomatous inflammation, even if the radiological findings do not show any abnormalities.     

Duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲ a hilar cholangiocarcinoma

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a singlelayer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow- up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.     

A case of diffuse endobronchial infiltration in a patient with non-Hodgkin's lymphoma]

A 38-year-old man with diffuse endobronchial infiltration with non-Hodgkin's lymphoma (NHL) is reported. He was admitted to our hospital on November 1990 because of lymph node swelling. Physical examination on admission revealed left axillary, bilateral cervical and inguinal lymph node swelling. Chest and abdominal CT scan showed para-aorta lymph node swelling, mild splenomegaly and heterogeneous density of the liver, although hilar and mediastinal lymphadenopathy were not found. Microscopic examination of the biopsy specimen obtained from the right inguinal lymph node and liver revealed NHL (B cell lymphoma, diffuse, mixed type). After two courses of chemotherapy, a chest roentgenogram showed bilateral hilar lymphadenopathy and reticular shadows. CT scan demonstrated a thickening of the left main bronchus. Bronchoscopic examination revealed a small submucosal nodule in the left main bronchus on February 1991, from which the biopsy specimen revealed NHL infiltration similar to that of the lymph node. At follow-up bronchoscopic examination, in spite of chemotherapy, scattered NHL infiltration was found in the submucosal space of the left vocal cord, carina and the bifurcation between left upper and lower bronchi. In patients with NHL, endobronchial involvement is rare. In this case, diffuse endobronchial infiltration was not thought to be direct invasion from the lymph node but hematogenous or lymphatic spread to the bronchi.     

Pulmonary malakoplakia coexistent with tuberculosis of the hilar lymph node mimicking malignancy

Pulmonary malakoplakia is a disease that is difficult to diagnose because its symptoms vary from those mimicking bronchogenic carcinoma to pneumonitis-like tuberculous infections. Malakoplakia is an unusual inflammatory condition characterized by the accumulation of macrophages with diagnostic Michaelis-Gutmann bodies. In this study, an immunocompromised patient with a tumor mass in the upper lobe of the right lung coexisting with hilar node enlargement is presented. A thoracoscopic biopsy revealed pulmonary malakoplakia with tuberculosis of the hilar lymph node. Microbiologic cultures were positive for acid-fast positive bacilli from the hilar node specimen, and negative for any other microorganisms such as Rhodococcus equi, but positive for Escherichia coli from the lung specimen. The significance of these findings and the coincidental association between malakoplakia and tuberculosis are discussed.     

Peroral direct cholangioscopic-guided biopsy and photodynamic therapy using an ultraslim upper endoscope for recurrent hepatocellular carcinoma with intraductal tiny nodular tumor growth

Bile-duct invasion is rare in patients with hepatocellular carcinoma (HCC). We report a case that received peroral direct cholangioscopy (PDCS)-guided endoscopic biopsy and photodynamic treatment (PDT) for recurrent HCC with intraductal tiny nodular tumor growth. A 64-year-old woman presented with recurrent right upper-quadrant pain. Six months previously she had been diagnosed with HCC with bile-duct invasion in the right anterior segment and had received right anterior segmentectomy. On pathological examination, the margin of resection was clear, but macroscopic bile-duct invasion was noted. On admission, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed a 0.5-cm-sized polypoid mass at the hilar portion. ERCP-guided biopsy failed, and an ampullary stricture was noted. PDCS-guided endoscopic biopsy was thus performed, and histopathology of the retrieved specimen revealed HCC. The patient submitted to PDT. There was no procedure-related complication. After 1 month of PDT the polypoid lesion and scar change at the hilar lesion had disappeared.     

Pulmonary Mycobacterium avium complex disease showing nodular bronchiectasis--: pathological findings in two cases

Histopathological examinations were carried out on 2 cases of Mycobacterium avium complex (MAC) disease of nodular bronchiectasis (NB) type on radiograms. The removed lung specimens revealed histological findings of granulomatous bronchiolopneumonia, consisting of epithelioid cell granulomas with lymphocytic infiltrations without exudation in the alveolar areas surrounding the respiratory bronchiole. The central bronchiolar walls were also affected by epithelioid cell granulomas with lymphocytic infiltration, occasionally showing polypoid protrusion into the bronchiolar lumen accompanying emphysema in the peripheral alveolar area. Bronchial lesions seemed to progress from peripheral to central airway with consequent atrophy and disappearance of intramural smooth muscles, resulted in bronchioloectasis. These histological findings well correspond to radiographical 'nodular bronchiectasis'. Large histiocytic granulomas without caseous necrosis developed in some area, which are not usually found in tuberculosis lesions. Epithelioid cell granulomas were occasionally found in the hilar lymph nodes as well as in the walls of lymphatic vessel in the pulmonary interlobular tissues, indicating intrapulmonary lymphatic spread of the mycobacteria.     

Mediastinal and hilar lymph node of cancer unknown origin: 3 case reports]

We encountered three rare cases of cancer of unknown origin affecting the mediastinal and hilar lymph nodes. Patient 1 was a 63 year-old man. Chest X-ray and CT films revealed an enlarged right hilar lymph node. A right mediastinal and hilar lymphadenectomy was performed. The histological diagnosis was metastatic squamous cell carcinoma (SCC). SCC of the right upper lobe appeared 34 months after the operation, requiring a right pneumonectomy. Patient 1 was alive 43 months after his first operation. Patient 2 was a 73 year-old man in whom left mediastinal and hilar lymph node swelling had been detected. A mediastinoscopy and lymph node biopsy were performed. The histological findings resulted in a diagnosis of metastatic small cell carcinoma. Chemotherapy was initiated, and the patient was alive 5 months after the biopsy procedure, Patient 3 was a 57 year-old man in whom right mediastinal and hilar lymph node swelling had been disclosed by chest CT scans. We performed a medianosternotomy and mediastinal and right hilar lymphadenectomy. Histologically, the diagnosis was metastatic adenocarcinoma. After the operation, radiation therapy was performed on the patient's mediastinum. Patient 3 was alive 5 months after the initial operation. The patients were given diagnoses of T0N1 or T0N2 lung cancer.     

A case of small cell lung cancer associated with pulmonary sarcoidosis]

A 63-year-old man with pulmonary sarcoidosis, diagnosed by mediastinal lymph node biopsy in 1977, was admitted in Feb. 1987 because of shortness of breath and cough. Chest X-ray showed bilateral hilar lymphadenopathy and a tumor shadow in the right lung field. Histological examination of specimens biopsied from the right lung revealed small cell carcinoma (S.C.C.). Bronchoalveolar lavage was performed to evaluate the disease activity of sarcoidosis, and the total number of cells and T-lymphocytes; the ratio of CD4+ cells to CD8+ cells was not increased. He was treated with combination chemotherapy, however, he died of respiratory failure after 7 months. An autopsy was performed, and the lesions were examined histologically. The sarcoid lesion in a lymph node obtained at autopsy was not active, in contrast to that obtained by mediastinal lymph node biopsy. Lung cancer and sarcoidosis are both common diseases, but their coexistence in the same patient is not common, and autopsied cases are rare. In this case, an autopsy was performed, and BAL had been performed prior to his death. The relationship between the BAL findings and the histology of sarcoidosis was examined. Based on the results of autopsy and BAL, the sarcoidosis was inactive prior to death, but had been histologically active 10 years previously. Therefore, this is a very interesting case, since we can examine the relationship between the two diseases, and the progression of each disease. This case also provides an interesting example of differentiation of sarcoidosis from S.C.C. Metastatic invasion of the hilar lymph nodes without bronchial stenosis and changes secondary to stenosis may often occur in patients with small cell lung cancer. Such metastatic invasion closely resembles the bilateral hilar lymphadenopathy of sarcoidosis; therefore, in some cases, it may be extremely difficult to differentiate the two diseases.     

A case of pulmonary sarcoidosis with CD8 positive cell dominant alveolitis

A 55-year-old woman was admitted for examination of bilateral hilar lymphadenopathy. Serum angiotensin converting enzyme was not elevated, but gallium-scan revealed an increased uptake in both lung hilus. The percentage of lymphocytes in the bronchoalveolar lavage fluid (BALF) was elevated (28.9%), and the proportion of OKIa1 positive cells was also elevated (50.5%). However, the ratio of cluster differentiation (CD) 4/8 was inverted at 0.5. Microscopic examination of the mediastinal lymph nodes revealed epithelioid cell granulomas without ceasation, and a diagnosis of sarcoidosis was made. Immunohistochemical examination of the lymph nodes showed predominancy of CD8 positive cells. Patients with pulmonary sarcoidosis having a low CD4/8 ratio in BALF are extremely rare in the early stage of sarcoidosis. The inverted CD4/8 ratio was of interest in regard to the pathogenesis of sarcoidosis.     

FUO due to sarcoidosis-lymphoma syndrome

This is a patient who presented as a fever of unknown origin (FUO) due to sarcoidosis-lymphoma syndrome. In favor of sarcoidosis was an elevated angiotensin-converting enzyme level, hypercalciuria, and bilateral hilar adenopathy on chest x-ray and chest CT. CT-guided biopsy of a hilar node revealed B-cell lymphoma. The differential diagnosis of sarcoidosis versus lymphoma and sarcoidosis-lymphoma syndrome as a cause of fever of unknown origin is discussed in this article.     

Differentiating Immunoglobulin G4-Related Sclerosing Cholangitis from Hilar Cholangiocarcinoma

Background/Aims

Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC.

Methods

We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC.

Results

In patients with hilar CC, obstructive jaundice was more frequent (p<0.01), serum total bilirubin levels were significantly higher (p<0.05), serum CA19-9 levels were significantly higher (p<0.01), and serum duke pancreatic monoclonal antigen type 2 levels were frequently elevated (p<0.05). However, in patients with IgG4-SC, the serum IgG (p<0.05) and IgG4 (p<0.01) levels were significantly higher and frequently elevated. The pancreas was enlarged in all IgG4-SC patients but only in 17% of hilar CC patients (p<0.01). Salivary and/or lacrimal gland swelling was detected in only 50% of IgG4-SC patients (p<0.01). Endoscopic retrograde cholangiography revealed that the hilar or hepatic duct was completely obstructed in 83% of hilar CC patients (p<0.01). Lower bile duct stenosis, apart from hilar bile duct stenosis, was more frequent in IgG4-SC patients (p<0.01). Bile duct wall thickening in areas without stenosis was more frequent in IgG4-SC patients (p<0.01).

Conclusions

An integrated diagnostic approach based on clinical, serological, imaging, and histological findings is necessary to differentiate IgG4-SC from hilar CC.     

Granulomatous mediastinitis due to aspergillus flavus in a nonimmunosuppressed patient

A patient with granulomatous mediastinitis due to Aspergillus flavus is described. A 22 year old black man presented with cough, fever and a right hilar mass. Mediastinal biopsies revealed granulomatous fibrosing mediastinitis with fungal elements compatible with aspergillus species. A. flavus was isolated on culture of this material and later from sputum and bronchial washings. Studies of the patient's immune status revealed normal humoral and cellular immunity. No underlying neoplasm was found. The patient was treated with amphotericin B and 5-fluorocytosine but esophageal and superior vena caval compression developed and he died. This is the first reported case of granulomatous mediastinitis due to A. flavus in a patient whose immune responses were not suppressed (nonimmunosuppressed patient). Infection with Aspergillus species should be considered in the differential diagnosis of granulomatous mediastinitis.     

Hilar cholangiocarcinoma with intratumoral calcification: A case report

This report describes a rare case of hilar cholangiocarcinoma with intratumoral calcification that mimicked hepatolithiasis. A 73-year-old man presented to a local hospital with a calcified lesion in the hepatic hilum. At first,hepatolithiasis was diagnosed,and he underwent endoscopic stone extraction via the transpapillary route. This treatment strategy failed due to biliary stricture. He was referred to our hospital,and further examination suggested the existence of cholangiocarcinoma. He underwent left hepatectomy with caudate lobectomy and extrahepatic bile duct resection. Pathological examination revealed hilar cholangiocarcinoma with intratumoral calcification,while no stones were found. To the best of our knowledge,only one case of calcified hilar cholangiocarcinoma has been previously reported in the literature. Here,we report a rare case of calcified hilar cholangiocarcinoma and reveal its clinicopathologic features.     

Partial embolization as re-treatment of hypersplenism after unsuccessful splenic artery ligation

Ligation of splenic artery(LSA) is used for the treatment of liver cirrhosis with hypersplenism. However, hypersplenism is not significantly improved following LSA treatment in some cases, and there are few reports of retreatment of hypersplenism after LSA. We report the case of a47-year-old man with liver cirrhosis and hypersplenism who underwent LSA treatment, but did not significantly improve. Laboratory tests revealed severe leukocytopeniaand thrombocytopenia. Celiac computed tomography arteriogram and digital subtraction angiography revealed two compensatory arteries connected to the hilar splenic artery from the left gastro-epiploic artery and from the dorsal pancreatic artery. Partial splenic embolization(PSE) was performed through the compensatory arteries. As a result, the patient achieved partial splenic ischemic infarction, and white blood cell and platelet counts rose and remained in the normal range. PSE is an effective therapeutic modality for the retreatment of hypersplenism when other modalities have failed.     

Nodular lung disease with five year survival and unilateral pleural effusion in AL amyloidosis

A 67-year-old female patient with biopsy proven AL systemic amyloidosis developed rapidly progressive dyspnea. Chest roentgenogram and CT scan revealed a large right pleural effusion in addition to nodular lesions with bilateral hilar lymphadenopathy. The patient's serum showed IgG λ type monoclonal gammopathy and she also had Bence Jones proteinuria. The pleural effusion was an exudate that contained many mononuclear cells and a high concentration of protein. Cardiac function was not seriously disturbed Except for amyloidosis, no other causes for the severe pleural effusion were found. This patient was treated with chemical pleurodesis using Picibanil and a low dose of prednisolone. Eighteen months after this treatment, her right pleural effusion did not recur. Bronchopulrnonary tissues are known to be frequently involved by AL systemic amyloidosis, but a nodular pattern of pulmonary amyloid deposition and a unilateral large pleural effusion are rare clinical manifestations in this disease.     

Sclerosing Carcinoma of the Right Hepatic Duct at the Porta Hepatis: Anicteric Presentation of Early Hilar Cholangiocarcinoma

Asymptomatic presentation of hilar cholangiocarcinoma is exceptional. We report an asymptomatic patient presenting with an abnormal alkaline phosphatase who proved to have a unilateral hilar cholangiocarcinoma obstructing the right heptic duct at the porta hepatis. Hyperbilirubinemia was absent. Subsequent to partial tumor resection and palliative decompression with right intrahepatic cholangiojejunostomy, the patient developed cholangitis with abscess formation. The efficacy of biopsy, tumor resection, and surgical decompression in hilar cholangiocarcinoma is discussed. Postmortem histology revealed biliary cirrhosis and acute cholangitis in the obstructed right liver lobe and minimal portal fibrosis in the unobstructed left liver lobe. The absence of bile stasis suggests compensatory bile excretion mechanism in the unobstructed left lobe. We believe the clinical and pathological findings in the patient to be part of the early natural history of hilar cholangiocarcinoma. Complete workup of asymptomatic alkaline phosphatase elevations in patients without previous history of hepatobiliary disease may prove fruitful in revealing incipient localized, unilateral bifurcation tumors.