Tamiya M, Yamane H, Terada H, Matsuno O, Yamamoto S.

Respiratory Department, NHO Osakaminami Medical Center.

Abstract

A 62-year-old man with pain in his hip joints and back was admitted to our hospital. His chest radiograph and CT showed a huge mass extending from the left upper pericardium to the left hilum, but no pleural effusion or other lesions. A contrast-enhanced abdominal CT showed multiple metastases to bones and both kidneys. Bronchoscopy revealed obstruction of the left B3 by a visible tumor. The biopsy specimens of the initial immunohistochemical staining were slightly positive for calretinin. However, we diagnosed the condition as sarcomatoid carcinoma of the lung on the basis of the clinical evaluation. Although radiotherapy was administered, his condition rapidly deteriorated and he died due to progression of the disease. Autopsy revealed extensive invasion, suggesting mesothelioma. Therefore, immunohistochemical staining was performed; the findings revealed sarcomatoid malignant mesothelioma. In conclusion, we encountered a rare case of sarcomatoid malignant mesothelioma (stage IV).

Glossary

carcinoma

(car-sin-o-ma) a malignant tumor that begins in the lining layer (epithelial cells) of organs. At least 80% of all cancers are carcinomas.

bronchoscopy

(BRON-kos-ko-pee) examination of the bronchi using a flexible, lighted tube called a bronchoscope.

biopsy

(buy-op-see) the removal of a sample of tissue to see whether cancer cells are present. There are several kinds of biopsies. In some, a very thin needle is used to draw fluid and cells from a lump. In a core biopsy, a larger needle is used to remove more tissue.

tumor

an abnormal lump or mass of tissue. Tumors can be benign (not cancerous) or malignant (cancerous).

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

pleural effusion

an abnormal accumulation of fluid, usually caused by trauma or disease, in the pleural space.

Kenmotsu H, Ishii G, Nagai K, Nakao M, Kawase A, Kojika M, Murata Y, Nishiwaki Y, Ochiai A.

Pathology Division, Research Center for Innovative Oncology, National Cancer Center Hospital East, Kashiwa, Japan.

Abstract

Pleomorphic carcinoma (PC) of the lung is classified as a subtype of sarcomatoid carcinoma of the lung, and peripheral PC is sometimes difficult to differentiate from the sarcomatoid component of mesothelioma. An 80-year-old man was referred to National Cancer Center Hospital East because a chest X-ray showed an abnormal shadow. CT scans of the chest indicated two solid masses located in the right lower lobe, and CT-guided needle biopsy yielded spindle-shaped tumor cells that were immunoreactive for both podoplanin and calretinin. Mesothelioma could not be ruled out, and the tumors were surgically resected to facilitate definitive pathological diagnosis. Both tumors were composed of undifferentiated carcinoma, bronchioloalveolar carcinoma and spindle cell carcinoma, and spindle cell component was immunoreactive for podoplanin and calretinin. Ten other tumors diagnosed as peripheral PC were also tested for podoplanin and calretinin expression. The sarcomatoid component in four of the 11 cases (36%) was immunoreactive with podoplanin, and it was calretinin positive in nine of the 11 cases (82%). When making the differential diagnosis between PC and the sarcomatoid component of mesothelioma, care is required in diagnosing biopsy specimens of peripheral lung spindle-cell tumors that are positive for both podoplanin and calretinin.

Glossary

oncology

(on-call-o-jee) the branch of medicine concerned with the diagnosis and treatment of cancer.

needle biopsy

removal of fluid, cells, or tissue with a needle for examination under a microscope. There are two types

diagnosis

identifying a disease by its signs or symptoms, and by using imaging procedures and laboratory findings. The earlier a diagnosis of cancer is made, the better the chance for long-term survival.

cell

the basic unit of which all living things are made. Cells replace themselves by splitting and forming new cells (mitosis). The processes that control the formation of new cells and the death of old cells are disrupted in cancer.

carcinoma

(car-sin-o-ma) a malignant tumor that begins in the lining layer (epithelial cells) of organs. At least 80% of all cancers are carcinomas.

cancer

malignancy; a group of diseases typified by abnormal, generally out-of-control, cell growth.

biopsy

(buy-op-see) the removal of a sample of tissue to see whether cancer cells are present. There are several kinds of biopsies. In some, a very thin needle is used to draw fluid and cells from a lump. In a core biopsy, a larger needle is used to remove more tissue.

tumor

an abnormal lump or mass of tissue. Tumors can be benign (not cancerous) or malignant (cancerous).

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

Expression of GATA-6 transcription factor in pleural malignant mesothelioma and metastatic pulmonary adenocarcinoma.

Lindholm PM, Soini Y, Myllärniemi M, Knuutila S, Heikinheimo M, Kinnula VL, Salmenkivi K.

Department of Medicine, University of Helsinki and Helsinki University Central Hospital, Finland.

Abstract

Background: Malignant mesothelioma (MM) is a highly aggressive tumour with poor prognosis and limited response to therapy. New markers for the prediction of prognosis in MM and in pulmonary adenocarcinoma of the pleura are valuable. GATA-6 belongs to a six member zinc finger transcription factor family named after their recognition motif W-GATA-R. AIM: To clarify the distribution and possible function of GATA-6 transcription factor in MM and in pleural metastasis of lung adenocarcinomas.

Methods: 63 pleural MM and 36 pleural metastatic pulmonary adenocarcinomas were studied for GATA-6 expression by immunohistochemistry using tissue microarrays. Expression of GATA-6 was examined in relation to thyroid transcription factor-1 expression, survival, proliferation and apoptosis.

Results: Nuclear immunoreactivity for GATA-6 was stronger and more frequent in MM than in metastatic pleural adenocarcinoma. Prognosis was better in patients with GATA-6 expression when compared to those with no GATA-6 expression (p = 0.002); in the subgroup analysis the difference was significant in epithelial and sarcomatous mesothelioma. GATA-6 was not associated with spontaneous proliferation or apoptosis of the tumour cells in situ.

Conclusion: Results suggest that GATA-6 plays a role in pleural malignancies, predicting longer survival in subgroups of MM.

Glossary

adenocarcinoma

(add-en-o car-sin-o-muh). Cancer that starts in the glandular tissue, such as in the ducts or lobules of the breast.

therapy

any of the measures taken to treat a disease. Unproven therapy is any therapy that has not been scientifically tested and approved. Use of an unproven therapy instead of standard (proven) therapy is called alternative therapy. Some alternative therapies have dangerous or even life-threatening side effects. For others, the main danger is that a patient may lose the opportunity to benefit from standard therapy. Complementary therapy, on the other hand, refers to therapies used in addition to standard therapy. Some complementary therapies may help relieve certain symptoms of cancer, relieve side effects of standard cancer therapy, or improve a patient's sense of well-being. The ACS recommends that patients considering use of any alternative or complementary therapy discuss this with their health care team.

prognosis

(prog-no-sis) a prediction of the course of disease; the outlook for the cure of the patient. For example, women with breast cancer that was detected early and who received prompt treatment have a good prognosis.

pleura

(pler-uh) the membrane around the lungs and lining of the chest cavity. (Pleural mesothelioma.)

metastasis

(meh-tas-teh-sis) the spread of cancer cells to distant areas of the body by way of the lymph system or bloodstream.

in situ

in place; localized and confined to one area. A very early stage of cancer.

tissue

a collection of cells, united to perform a particular function.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

apoptosis

a type of cell death in which the cell basically commits suicide; scientists believe some types of cancer may originate from an interruption of this programmed cell death, allowing cells to grow out of control.

Junker K, Krismann M.

Zentrum für Pathologie, Klinikum Bremen-Mitte, Bremen. klaus.junker@klinikum-bremen-mitte.de

Abstract

In the WHO classification, pleural tumours of mesothelial and mesenchymal origin as well as lymphoproliferative disorders are distinguished, with malignant mesotheliomas forming the most common primary pleural tumour. Histologically, a prognostically relevant differentiation between epitheloid (40-60 %), sarcomatoid (20-30 %), and biphasic mesotheliomas (20-30 %) is made. Besides other rare primary tumours of the pleura, the differential diagnoses of malignant mesotheliomas comprise carcinomatous pleuritis of intra- and extrapulmonary malignancies. Especially in early tumour stages, the differentiation from reactive pleural changes requires a close interdisciplinary cooperation with a careful correlation between histopathological findings and clinical presentation.

Keywords: malignant pleural mesothelioma – carcinomatous pleuritis – solitary fibrous tumour of the pleura – WHO classification

Glossary

pleura

(pler-uh) the membrane around the lungs and lining of the chest cavity. (Pleural mesothelioma.)

differentiation

(dif-er-en-she-A-shun) the normal process through which cells mature so they can carry out the jobs they were meant to do. Cancer cells are poorly differentiated.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

Yildirim H, Metıntaş M, Ak G, Dündar E, Ergınel S.

Department of Chest Diseases, Faculty of Medicine, Osmangazi University, Eskişehir, Turkey. heylul2002@yahoo.com.

Abstract

Almost all cancers can cause distant pleural metastases. However, pleural metastases of soft tissue sarcoma that constitute less than 1% of adult solid malignancy are extremely rare. It is very difficult to distinguish them form sarcomatous malignant mesothelioma on histopathological features. We report a 57 year-old man who presented to us with left chest pain and progressive dyspnea and was diagnosed to have a pleural metastases of soft tissue sarcoma by thoracoscopic biopsy.

Glossary

sarcoma

(sar-co-muh) a malignant tumor growing from connective tissues, such as cartilage, fat, muscle, or bone.

biopsy

(buy-op-see) the removal of a sample of tissue to see whether cancer cells are present. There are several kinds of biopsies. In some, a very thin needle is used to draw fluid and cells from a lump. In a core biopsy, a larger needle is used to remove more tissue.

tissue

a collection of cells, united to perform a particular function.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

Hjerpe A, Dobra K.

Department of Laboratory Medicine, Division of Pathology, Karolinska Institutet, Stockholm, Sweden.

Abstract

Malignant mesothelioma (MM) is a connective tissue tumor with partial epithelioid differentiation. The pattern of proteoglycan (PG) expression by epithelioid and fibroblast-like (sarcomatoid) MM cells differ; cell surface PGs being more abundant in the former phenotype and matrix PGs in the latter. The differentiation as well as much of the malignant nature of these tumors is dependent on the expression of surface PGs. The syndecans, however, also translocate to the nucleus for an as yet unknown function.

Glossary

nucleus

(new-clee-us) the center of a cell where the DNA is housed and replicated. Studying the size and shape of a cell's nucleus under the microscope can help pathologists distinguish cancer cells from benign cells.

differentiation

(dif-er-en-she-A-shun) the normal process through which cells mature so they can carry out the jobs they were meant to do. Cancer cells are poorly differentiated.

cell

the basic unit of which all living things are made. Cells replace themselves by splitting and forming new cells (mitosis). The processes that control the formation of new cells and the death of old cells are disrupted in cancer.

tissue

a collection of cells, united to perform a particular function.

tumor

an abnormal lump or mass of tissue. Tumors can be benign (not cancerous) or malignant (cancerous).

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

Neragi-Miandoab S, Richards WG, Sugarbaker DJ.

Department of Surgery, Cardiothoracic Surgery, Boston University Medical Center, School of Medicine, One Boston Medical Center Place, Boston MA 02118, United States.

Abstract

Aim: The survival of patients with malignant pleural mesothelioma (MPM) who do not seek treatment ranges from 4 to 12 months. To date, the optimal procedure for resection of malignant pleural mesothelioma is controversial, extrapleural pneumonectomy has been most consistently associated with long-term survival and has provided the most radical cytoreduction; but, unfortunately, not all patients qualify for this invasive surgical approach.

Methods: Between 1992 and 2000, 64 patients underwent pleurectomy as a palliative treatment for MPM. This retrospective study evaluates the operative outcome and the impact of some prognostic factors on patients’ survival. Preoperative evaluation included chest X-ray, CT and/or MRI. Diagnosis was made by pleural biopsy via needle, open, or VATS biopsy. The Kaplan-Meier curve and the Log-Rank test were used to analyze the data.

Results: The median age of the study group was 65 (with a range of 29-84 years). Thirty-six patients had epithelial histology, and 28 patients had sarcomatoid or mixed type (e.g., epithelial+spindle, epithelial+sarcomatoid). The 30-day mortality rate was 3.1%. The overall survival rate was 43%, 28%, and 10% at 1, 2, and 3 years, respectively. The overall median survival was 9.4 months (ranging from 1.15 to 52.7 months). The overall median survival with epithelial histology (n=36, 56%) was 21.7 months (with a range of 1.4-52.7 months) versus 5.8 months (with a range of 1.15-18.3 months) for the sarcomatoid or mixed type (n=28, 44%), p=0.0001. The morbidity included atrial fibrillation (n=5), wound infection (n=2), prolonged intubation (longer than 24h, n=8), reintubation for respiratory failure (n=2), pulmonary emboli (n=1), UTI (n=16), DVT (n=5), MI (n=4), and postoperative bleeding (n=7). Univariant analysis demonstrated that the only prognostic factors influencing survival in our series was the histologic type. Age, gender, and the affected side of the lung did not affect the median survival.

Conclusion: Our results show that pleurectomy can be performed as a means of palliation for advanced-stage disease with a low mortality rate and may, in fact, improve survival in patients with epithelial subtype as compared with historical controls in the literature with no surgical intervention.

Glossary

survival rate

the percentage of survivors with no trace of disease within a certain period of time after diagnosis or treatment. For cancer, a 5-year survival rate is often given. This does not mean that people can't live more than five years, or that those who live for 5 years are necessarily permanently cured.

resection

surgery to remove part or all of an organ or other structure.

morbidity

a measure of the new cases of a disease in a population; the number of people who have a disease.

mortality

a measure of the rate of death from a disease within a given population.

MRI

Stands for magnetic resonance imaging. A method of taking pictures of the inside of the body. Instead of using x-rays, MRI uses a powerful magnet and transmits radio waves through the body; the images appear on a computer screen as well as on film. Like x-rays, the procedure is physically painless, but some people find it psychologically uncomfortable to be in the small core of the MRI machine.

diagnosis

identifying a disease by its signs or symptoms, and by using imaging procedures and laboratory findings. The earlier a diagnosis of cancer is made, the better the chance for long-term survival.

biopsy

(buy-op-see) the removal of a sample of tissue to see whether cancer cells are present. There are several kinds of biopsies. In some, a very thin needle is used to draw fluid and cells from a lump. In a core biopsy, a larger needle is used to remove more tissue.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

extrapleural pneumonectomy

(EPP) surgery to remove the pleura, diaphragm, pericardium, and entire lung involved with the tumor. You can view a web cast from Brigham and Women's Hospital in Boston of this procedure being done by Dr. David Sugarbaker: see the extrapleural pneumonectomy (EPP) web cast here.

Klebe S, Mahar A, Henderson DW, Roggli VL.

Department of Anatomical Pathology, Flinders University of South Australia, Adelaide, SA, Australia.

Abstract

Only a small number of malignant mesotheliomas with heterologous elements have been described. There are currently no criteria for diagnosis and little data regarding prognosis. We suggest that the term heterologous mesothelioma should be reserved for tumours that show malignant heterologous elements, notably osteosarcomatous, chondrosarcomatous, or rhabdomyoblastic elements but have immunohistochemical and clinical characteristics of mesothelioma. We identified 27 such cases and characterized the clinical and pathological characteristics of these tumours. In our series, 89% originated in the pleura, and 11% from the peritoneal cavity. The median age at diagnosis was 68 years, ranging from 27 to 85 years. Of these cases, 93% occurred in males and 7% in women. Of the 27 mesothelioma cases 16 (59%) were sarcomatoid, 10 (37%) were biphasic, and one was reported as epithelioid; 40% (11 cases) showed osteosarcomatous elements only, 19% showed areas of rhabdomyosarcoma only, 19% contained areas of chondrosarcoma only, and 22% exhibited osteochondromatous elements. Immunohistochemical labelling for cytokeratins was present in the majority of cases. Exposure to asbestos was identified in all the 17 cases for which an exposure history was available (63%). Median survival was 6 months after diagnosis, similar to the survival seen in sarcomatoid mesotheliomas. The differential diagnosis includes primary and secondary pleural sarcomas, including osteosarcomas and chondrosarcomas. Immunohistochemical labelling for cytokeratins is helpful in the distinction, but lack of labelling for cytokeratins in a spindle cell/sarcomatoid tumour does not exclude the diagnosis of mesothelioma, irrespective of the presence of heterologous elements. We suggest that if the anatomical distribution conforms to that of mesothelioma, a diagnosis of heterologous mesothelioma should be made in preference to a diagnosis of primary pleural osteosarcoma or chondrosarcoma, regardless of cytokeratin positivity, as for conventional non-heterologous sarcomatoid mesothelioma.

Glossary

prognosis

(prog-no-sis) a prediction of the course of disease; the outlook for the cure of the patient. For example, women with breast cancer that was detected early and who received prompt treatment have a good prognosis.

pleura

(pler-uh) the membrane around the lungs and lining of the chest cavity. (Pleural mesothelioma.)

diagnosis

identifying a disease by its signs or symptoms, and by using imaging procedures and laboratory findings. The earlier a diagnosis of cancer is made, the better the chance for long-term survival.

cell

the basic unit of which all living things are made. Cells replace themselves by splitting and forming new cells (mitosis). The processes that control the formation of new cells and the death of old cells are disrupted in cancer.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

peritoneal

(pair-uh-tuh-nee-al) the serous membrane that lines the cavity of the abdomen. (More on Peritoneal Mesothelioma.)

Yamazaki M, Ohwada A, Miyaji A, Yamazaki H, Nara T, Hirai S, Fujii H, Uekusa T, Suzuki M, Iwase A, Takahashi K.

Department of Respiratory Medicine, Juntendo Tokyo Koto Geriatric Medical Center, Tokyo. mariko@med.juntendo.ac.jp

Abstract

A 72-year-old man patient was referred to our institution for evaluation and treatment of right pleural effusion. Eosinophilic pleural effusion and peripheral eosinophilia were identified during the course of hospitalization. Pulmonary paragonimiasis was confirmed by the presence of paragonimus-specific IgG antibodies for Paragonimus (P.) westermani and P. miyazakii in his serum. Although Praziquantel, a highly effective agent for the treatment of lung flukes was repeatedly administered, the pleural effusion did not subside and the patient’s condition gradually deteriorated until his death due to circulatory insufficiency. Postmortem examination revealed malignant mesothelioma of the sarcomatous type encasing the right lung and heart. Cardiac involvement accompanied with old and recent-onset myocardial ischemic changes resulted in death of this patient. Here, we report a very rare case of malignant mesothelioma with a concomitant infection of parasitic lung fluke.

Keywords: paragonimiasis, malignant mesothelioma, eosinophilia

Glossary

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

pleural effusion

an abnormal accumulation of fluid, usually caused by trauma or disease, in the pleural space.

Moran CA, Albores-Saavedra J, Suster S.

Department of Pathology, M D Anderson Cancer Center, Houston, TX 77030, USA. cesarmoran@mdanderson.org

Abstract

Aims: To present eight cases of primary diffuse peritoneal malignant mesothelioma in children <15 years old, with a discussion of the pitfalls of this diagnosis in the paediatric age group.

Methods and Results: The cases were selected based on the following criteria: (i) primary peritoneal neoplasms confined grossly or radiographically to the abdominal cavity; (ii) negative history of previous or another associated malignancy; (iii) histopathological confirmation. All patients (five female, three male) presented clinically with symptoms of abdominal pain, distention and ascites. Grossly, the tumours showed multiple, diffuse peritoneal nodules. Histologically, seven cases corresponded to epithelioid mesotheliomas and one case displayed biphasic (epithelioid and spindle) cellular proliferation. Immunohistochemical studies for cytokeratin (CK) 5/6, calretinin and low-molecular-weight CK (CAM5.2) showed strong cytoplasmic positivity in the neoplastic cells. Three patients were treated by chemotherapy. On clinical follow-up, four patients with epithelioid mesotheliomas were alive and well from 12 to 18 months after initial diagnosis; one patient with a mixed (biphasic epithelioid/sarcomatoid) mesothelioma died of tumour 24 months after diagnosis.

Conclusions: Peritoneal malignant mesothelioma in children is a rare condition that can introduce difficulties in histopathological diagnosis.

Glossary

diagnosis

identifying a disease by its signs or symptoms, and by using imaging procedures and laboratory findings. The earlier a diagnosis of cancer is made, the better the chance for long-term survival.

chemotherapy

(key-mo-THER-uh-pee) treatment with drugs to destroy cancer cells. Chemotherapy is often used with surgery or radiation to treat cancer when the cancer has spread, when it has come back (recurred), or when there is a strong chance that it could recur.

cancer

malignancy; a group of diseases typified by abnormal, generally out-of-control, cell growth.

mesothelioma

a tumor derived from mesothelial tissue, such as the peritoneum (lining the abdomen) or pleura (lining the lungs). More on mesothelioma.

peritoneal

(pair-uh-tuh-nee-al) the serous membrane that lines the cavity of the abdomen. (More on Peritoneal Mesothelioma.)

ascites

(uh-sigh-tees) excess fluid accumulation in the abdominal (peritoneal) cavity.