Right pulmonary artery sarcoma

A 57-year-old man presented with cough, chest pain and dyspnea. Peripheral lung opacities detected radiologically on admission were surgically resected and histologically confirmed as pulmonary infarcts. Subsequent radiological examinations revealed a stricture of the right pulmonary artery with evidence of pulmonary hypertension. The patient was commenced on anticoagulant therapy, which resulted in some clinical improvement. A hilar mass detected later was resected together with the right lung. Pathological examination of the hilar mass revealed a tumour within the lumen of the pulmonary artery causing almost total occlusion of the artery. Histology showed angiosarcomatous and osteosarcomatous areas. The patient is well and disease-free 9 months following resection.     

Malignant fibrous histiocytoma with widespread metastasis and pulmonary cancer

An autopsy case of malignant fibrous histiocytoma (MFH) with widespread metastases and lung carcinoma in a 64-year-old Japanese woman is reported. The initial signs were cough and sputum, followed by hemosputum. A chest X-ray photo showed a right pleural tumor, which could not be identified from a biopsy specimen, but was identified as MFH by light and electron microscopic studies on biopsy specimens of tongue tumors. Autopsy examination revealed metastases of the MFH to the brain, lung, liver, kidney, adrenal, pancreas, retroperitoneum, and some bones, and pulmonary adenocarcinoma.     

Primary intimal sarcoma of pulmonary valve and trunk with osteogenic sarcomatous elements. Report of a case considered to be pulmonary embolus.

A 49-year-old woman had a six-week history of increasing shortness of breath and fatigue. X-ray films and pulmonary scans showed multiple areas of emboli, especially in the right lung. Treatment with heparin was unsuccessful, and although a repeat scan showed only slight changes, the patient's condition deteriorated. Acute pulmonary edema and ventricular tachycardia preceded her death. Autopsy revealed a primary intimal sarcoma with osteogenic elements arising in the posterior leaflet of the pulmonary valve and obstructing the main pulmonary artery and its right branch.     

兔肺动脉高压时肺组织中内皮素和鸟苷环－磷酸含量的变化及意义

１１只兔进行从降主动脉至左肺动脉的分流手术，观察三个月后形成了左侧肺动脉高压。取左、右肺组织测定内皮素（ＥＴ）和ｃＧＭＰ，发现左侧肺组织中ＥＴ含量较右侧为高，而ｃＧＭＰ则较右侧为低，两侧比较差异显著。因组织中ｃＧＭＰ的含量间接反映了内皮舒缩血管因子（ＥＤＲＦ）的含量，故认为肺高压时肺组织中ＥＴ分泌增加和ＥＤＲＦ分泌减少。两者的变化在肺高压的形成过程中可能起重要作用。     

MALIGNANT FIBROUS HISTIOCYTOMA WITH WIDESPREAD METASTASIS AND PULMONARY CANCER

An autopsy case of malignant fibrous histiocytoma (MFH) with widespread metastases and lung carcinoma in a 64-year-old Japanese woman is reported. The initial signs were cough and sputum, followed by hemosputum. A chest X-ray photo showed a right pleural tumor, which could not be identified from a biopsy specimen, but was identified as MFH by light and electron microscopic studies on biopsy specimens of tongue tumors. Autopsy examination revealed metastases of the MFH to the brain, lung, liver, kidney, adrenal, pancreas, retroperitoneum, and some bones, and pulmonary adenocarcinoma.     

Primary giant cell malignant fibrous histiocytoma of the lung: A case report

A rare case of malignant fibrous histiocytoma of giant cell type originating in the lung of a 46-year-old woman is presented. The patient complained of having a cough that had lasted for a few weeks. A chest X-ray photograph showed a tumor shadow on the left lung. Histological and cytological examination of the biopsy specimen revealed that the tumor was a kind of sarcoma. An operative procedure was selected because of tumor invasion into the trunk of the left pulmonary artery, which was discovered on computed tomography examination, and because metastatic tumor was excluded clinically. The tumor was almost encapsulated and 6 x 6 x 6 cm in size; however, it also showed invasion into the pulmonary artery and bronchial lumen. A histological survey of the tumor showed a wide range of patterns such as fibrous, pleomorphic, fascicular and osteoclast-like giant cell figures; however, the osteoclast-like giant cell area was predominant. Immunohistochemically, the tumor cells were positive for vimentin, CD68 for histiocytic marker and alpha1-antichymotrypsin, and negative for keratin, epithelial membrane antigen, S-100 protein, MT-1, desmin, myoglobin and lysosome. No primary tumor was found clinically in any part of the patient's body at 2 and 4 months after operation. Consequently, she was diagnosed as having primary giant cell malignant fibrous histiocytoma of the lung.     

Fetal lung interstitial tumor: The first Japanese case report and a comparison with fetal lung tissue and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3

Fetal lung interstitial tumor, a newly recognized lung lesion in infants, was first reported in 2010. Here, we report the first Japanese case of fetal lung interstitial tumor which was originally diagnosed as atypical congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. A 7‐day‐old girl was referred to our hospital with respiratory distress and a left lung mass and she subsequently underwent left lower lobectomy. The specimen showed a 5 cm solid mass with a fibrous capsule. Histological examination revealed immature airspaces and interstitium, containing bronchioles and cartilage. The epithelial and interstitial cells contained abundant glycogen granules. Immunohistochemistry showed nuclear/cytoplasmic expression of β‐catenin in the epithelial and interstitial cells. β‐catenin gene mutations and trisomy 8 were not detected, so a neoplastic origin could not be confirmed. The histological findings were partly consistent with normal fetal lung at the canalicular stage, pulmonary interstitial glycogenosis, and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. In this report, we compare the above conditions and discuss the pathogenesis of fetal lung interstitial tumor.     

Coronary artery bypass grafting with concomitant resection for carcinoma of lung

A 69-year-old woman with angina had a lesion in the left lower lobe on chest film. Angiography revealed coronary artery disease in three vessels. Combined off pump coronary artery bypass grafting (CABG) and left lower lobectomy were performed through median sternotomy. This approach avoids complications due to staged operations and cardiopulmonary bypass (CPB). This report shows that simultaneous off pump CABG and pulmonary operations can be performed safely in patients with coronary artery disease (CAD) associated with lung cancer.     

Hyalinizing spindle cell tumor with giant rosettes with pulmonary metastasis after a long hiatus: a case report

Hyalinizing spindle cell tumor with giant rosettes (HSCTGR) is a recently described tumor, which is regarded as an unusual variant of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. The patient in the report was a 35-yr-old woman who showed multiple bilateral pulmonary nodules with massive pleural effusion in the right side. She had a history of a mass excision in the right thigh 11 yrs ago at another hospital, which was reported as a "leiomyoma". Two years before this presentation, the patient received a routine chest radiograph which demonstrated bilateral multiple pulmonary nodules. A lobectomy of the left upper lung was performed. The histological findings revealed a well-circumscribed nodule that was characterized by a spindle-shaped fibrous to hyalinized stroma with criss-crossing short fascicles and giant collagen rosettes surrounded by a rim of spindle-shaped cells. Electron microscopy confirmed the fibroblastic nature of the tumor. This case, in addition to at least two other cases reported in the literature, demonstrates that the HSCTGR is a malignant neoplasm with the capacity to metastasize after a long hiatus.     

Primary pulmonary artery sarcoma. Report of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature

Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistochemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed.     

Fine-needle aspiration cytology of pulmonary mucinous cystadenocarcinoma

A 64-year-old man presented with symptoms of a lung infection. Radiologic work-up revealed opacity in the upper lobe of right lung. A computer tomographic (CT) guided fine-needle aspiration biopsy revealed sheets and clusters of mild to moderately atypical epithelial cells, small amount of mucin, histiocytes, multinucleated giant cells, and stromal fragments. The differential diagnosis included an inflammatory process and a well-differentiated adenocarcinoma. A lobectomy was performed and revealed abundant extracellular mucin surrounded partially by a thick fibrous wall, which was lined by neoplastic glandular cells. The histology was consistent with a pulmonary mucinous cystadenocarcinoma. Fibroblastic proliferation and foreign-body giant reaction secondary to extravasation of mucin were also noted. The patient was disease free 1 year after initial diagnosis. The relative lack of mucin as well as the presence of well-differentiated neoplastic cells, the inflammatory response to extravasated mcuin constitutes a potential pitfall in recognizing pulmonary mucinous cystadenocarcinoma cytologically.     

A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction

Pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.     

Malignant solitary fibrous tumor of the thyroid gland: report of a case and review of the literature

Solitary fibrous tumors of the thyroid gland (T-SFT) are rarely described, with only 21 cases being reported in the English literatures, all showing benign clinical characteristics. We herein present a 76-year-old woman presenting with a 3-month history of rapidly enlarging neck masses and the CT showed masses with partial calcification in the right thyroid lobe. We performed right hemithyroidectomy and isthmectomy with negative margin under general anesthesia. Histologically, the masses consisted of pleomorphic spindle cells with high mitoses and collagen bands. Immunohistochemically, the tumor cells showed positive reactions for CD34, vimentin and bcl-2, then a diagnosis of malignant solitary fibrous tumor of the right thyroid was made. Six months postoperatively, the CT showed the recurrence of the thyroid tumor and the presence of many nodules of varying sizes throughout bilateral pulmonary lobes. To our knowledge, this is the first case of malignant solitary fibrous tumor of the thyroid gland (T-SFT) with local recurrence and pulmonary metastasis and T-SFT must be considered in the differential diagnosis of spindle cells lesions in the thyroid gland. Correct diagnosis of the malignant T-SFT plays an important role in choosing appropriate therapeutic strategies and long-term follow-up is also extremely essential for these patients.     

Malignant fibrous histiocytoma of the lung

A 75-year-old woman was admitted to a hospital for diagnosis of pulmonary infarction and died during treatment. An autopsy revealed a tumor 5 cm in diameter in the hilus of the left lung, spreading into the posterior mediastinum, and a metastasis was also found in the right lung. Histologically, this tumor consisted of two kinds of cells; one of fibroblast-like cells and the other of histiocyte-like cells, showing a storiform pattern. Furthermore, a positive staining for alpha 1-antitrypsin, but negative for CEA, keratin or S-100 protein was seen. Therefore, it was diagnosed as malignant fibrous histiocytoma (MFH) originating in the hilus of the left lung. In addition, many foci of hemorrhagic infarction due to metastasis and infiltration of the tumor into the pulmonary arteries were observed in the right lung. MFH is one of the rarest primary tumors of the lung.     

Life-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion

We present the case of a patient who has a life-threatening hemoptysis caused by occlusion of the right pulmonary vein owing to external compression by excessive fibrous tissue. Because the patient's lung was essentially nonfunctional and hemoptysis persisted, we performed a pneumonectomy on her. Pathological analysis of the lung showed severe hypertensive changes in the arterial and venous microvasculature of the lung. Parenchymal-pleural to intercostal systemic venous connections had developed, facilitating venous drainage of the lung circulation. Upon review of the patient's history and the pathological and radiological evidence, we concluded that the pathological changes were caused by a variant of mediastinal fibrosis termed chronic idiopathic pulmonary hilar fibrosis, of which only a few cases have been described in the literature. A detailed case history and review of the literature are presented.     

PRIMARY PULMONARY ARTERY SARCOMA. Report of Two Autopsy Cases Studied by Immunohistochemistry and Electron Microscopy, and Review of 110 Cases Reported in the Literature

Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistocyto-chemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed. ACTA PATHOL JPN 38: 883∼896, 1988.     

Pulmonary Mucormycosis with Fatal Massive Hemoptysis

A case of pulmonary mucormycosis in a 57 year old woman with acute promyelocytic leukemia (APL) who died of massive hemoptysis is reported. Chest radiography revealed changes that began with a small focal infiltration and progressed to a large round nodule with cavity formation. Postmortem examination showed the nodule to be composed mainly of infarcted lung tissue with saprophytic growth of Mucor. An adjacent proximal branch of the left pulmonary artery was thrombosed with mucoraceous hyphae, and it had ruptured into the cavitary space around the necrotic tissue and then into a conducting bronchus. In general, both fatal massive hemoptysis and cavity formation are rare in pulmonary mucormycosis. In our present case, the histological findings suggested that both phenomena were closely related to the pulmonary infarction caused by Mucor invasion of the pulmonary artery.     

Waldenström's macroglobulinemia

Summary An autopsy case of Waldenström's macroglobulinemia is reported, in whom an abnormal pulmonary shadow had already existed 2 years before the diagnosis of the disease and was proved to be pulmonary involvement. Immunoelectrophoresis demonstrated a monoclonal increase in immunoglobulin M with kappa light chain. A chest X-ray film showed a reticulo-nodular shadow in the right lower lobe of the lung. A bronchial biopsy specimen revealed a diffuse and dense lymphocytic infiltration. Bone marrow aspirate revealed no remarkable change except for a slight increase in plasma cells (1.7%) and an appearance of atypical lymphocytes (0.5%). At autopsy, more than half of the right lower lobe of the lung was occupied by a pale whitish, viscid and glossy tumour mass. Heptosplenomegaly and lymph node enlargement were not observed. Histological findings of the tumour tissue were similar to those of the biopsy specimen. Lymphocytic infiltration was observed also in the liver, kidneys, spleen, bone marrow and lymph nodes, but was of minor degree. Other reported cases of Waldenström's macroglobulinemia accompanied by pulmonary involvement are reviewed.     

Main pulmonary artery stenosis caused by fibrocalcified mass in a young infant

We present a rare case of main pulmonary artery stenosis secondary to protruding fibrous material in the main pulmonary artery associated with patent ductus arteriosus. A 1-month-old baby boy manifested cardiac murmur. Echocardiogram showed circumferential high echogenic mass inside the main pulmonary artery with pressure gradient of 49 mmHg and patent ductus arteriosus. The mass did not regress during 3 months' follow-up period. Angiographic images showed that the circular filling defect was located at the main pulmonary artery distal to pulmonary valve, and pulmonary valve and both pulmonary arteries were normal. After surgical removal of the circumferential material and ductus ligation, the pressure gradient became negligible. The material was consisted of scarcely cellular fibrous tissue, abundant coagulum of fibrinous material and dense calcification.     

Pulmonary mucormycosis with fatal massive hemoptysis.

A case of pulmonary mucormycosis in a 57-year-old woman with acute promyelocytic leukemia (APL) who died of massive hemoptysis is reported. Chest radiography revealed changes that began with a small focal infiltration and progressed to a large round nodule with cavity formation. Postmortem examination showed the nodule to be composed mainly of infarcted lung tissue with saprophytic growth of Mucor. An adjacent proximal branch of the left pulmonary artery was thrombosed with mucoraceous hyphae, and it had ruptured into the cavitary space around the necrotic tissue and then into a conducting bronchus. In general, both fatal massive hemoptysis and cavity formation are rare in pulmonary mucormycosis. In our present case, the histological findings suggested that both phenomena were closely related to the pulmonary infarction caused by Mucor invasion of the pulmonary artery.