Basic Data. 鍾 XX, female Age:59 y/o

Transcription

1 Basic Data 鍾 XX, female Age:59 y/o

2 Chief complain for evaluation of the left pleural mass

3 Clinical course This 59 years old lady Hypertension for 3 years, under herbal control. An episode of high BP up to 180/80 on CV OPD for consultation, with CXR routinely arranged. The CXR : a fuciform mass over left upper lung field attached to the chest wall. Transferred to CS OPD for consultation. Chest CT : fuciform pleural mass over left upper chest. Admitted for CT-guided biopsy.

4 Personal history Tobacco: No Alcohol drinking: No Betel nuts: No Family history father: HTN, DM, dyslipidemia mother: abdominal cancer with unknown name

5 Past history Medical: (Diagnosis, year, Drug, Result) HTN for 3 years under herbal medication

6 Review of system 1.General: fair, no body weight change 2.HEENT: no neck pain or stiffness 3.Cardio-Respiratory system: No Dyspnea or chest pain 4.Gastrointestinal system: No appetite change, no diarrhea, vomiting, or abdominal pain 5.Genito-urinary system: negative findings 6.Neuro- Psychiatric system: fair 7.Musculoskeletal system: free moveable, no muscle or joint pain noted 8.Skin: negative findings

7 Physical examination General: fair Consciousness: alert. E4M6V5 Neck: supple Chest: Inspection: symmetric expansion Auscultation: bilateral symmetric air entry, no crackles or Heart: RHB without murmur. Abdomen: Soft and flat, normal-active bowel sounds, soft, no muscle guarding nor tenderness. Percussion: mild tympanic,. Extremities: Free moveable

8 IMAGE FINDINGS

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15 Pathology CT-guided biopsy: solitary fibrous tumor brown and soft. measuring up to 1.2 x 0.1 x 0.1 cm, in size solitary fibrous tumor with spindle cells arranged in fascicles with interspersed collagen bundles. No mitosis is seen in the spindle cells. Positive immuno-reactivity to CD34.

16 DISCUSSION

17 Pleural tumor Primary Secondary (common) metastatic from the lung and breast. malignancy from any organ of the body Ovarian carcinomas, in both the abdominal and thoracic cavities. a serous or serosanguineous effusion follows that often contains neoplastic cells. --- cytologic examination

18 Solitary Fibrous Tumor benign mesothelioma or benign fibrous mesothelioma in the pleura and fibroma in the lung Grossly: dense fibrous tissue with occasional cysts filled with viscid fluid; Microscopically: reticulin and collagen fibers among, spindle cells, fibroblasts. Rarely malignant, with pleomorphism, mitotic activity, necrosis, and large size (>10 cm). The tumor cells are CD34+ and keratin-negative by immunostaining. ----v.s malignant mesotheliomas No relationship to asbestos exposure.

19 Malignant Mesothelioma Malignant mesotheliomas in the thorax arise from either the visceral or the parietal pleura. Increased incidence with heavy exposure to asbestos. United States and Great Britain, and in Canadian, Australian, and South African mining areas 90% of reported mesotheliomas are asbestos-related. The lifetime risk : in heavily exposed individuals : 7% to 10%. Asbestos bodies are found in the lungs of patients with mesothelioma. ( asbestos plaque) Cytogenetic studies : 60% to 80% have deletions in chromosomes 1p, 3p, 6q, 9p, or 22q 31% have p16 mutations.

20 Malignant Mesothelioma Symptoms and signs Persistent dry cough. Difficulty in swallowing food (also known as dysphagia). Unexplained fatigue, and tiredness. Coughing up of blood (also known as hemoptysis). Abrupt and unexplained weight loss. Appearance of lumps under the skin on the chest.

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23 Other differential diagnosis In the chest, lung cancer intrapleural sarcomas. A posterior paraspinal location a neurogenic tumor or round atelectasis. A more anterior and medial location a thymic neoplasm, germ cell tumor, or teratoma. well-circumscribed appearance --- generally rules out malignant pleural mesothelioma

24 Lary A. Robinson, MD Thoracic Oncology Program at the H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted March 28, 2006; accepted June 14, SOLITARY FIBROUS TUMOR OF THE PLEURA

25 Introduction The solitary fibrous tumor of the pleura (SFTP) rare primary tumor from mesenchymal cells in the arveolar tissue subjacent to the mesothelial-lined pleura.

26 Introduction 800 cases have been reported first mentioned by Wagner in 1870, first pathologic report:1931 by Klemperer and Rabin.

27 Introduction SFTP v.s. Malignant pleural mesothelioma, (the most common) over half of these cases an asymptomatic mass, quite large in size benign in 78% to 88% of patients.

28 Incidence Predominantly in the 60~70 y/o Equal frequency in both sexes there is No No apparent genetic predisposition for the tumor No relationship to exposure to asbestos, tobacco, or any other environmental agent. chromosome 8 suggests --- a more malignant behavior

29 Clinical Features The causes of HPO and clubbing are unknown Hypertrophic pulmonary osteoarthropat hy---hpo

30 Physical Signs A large tumor may cause enough lung compression to result in wheezing, dullness to percussion, or decreased breath sounds in the affected hemithorax. Clubbing is the most frequent physical finding in SFTP in the few patients (2% to 19%) who present with HPO. Clinically, the distal phalanx is enlarged and widened, with the characteristic spongy sensation on depression of the proximal nail bed.

31 Radiographic Features Size: Varity Well-circumscribed margins Located near the lung periphery in the projection of an inter-lobar fissure well-delineated, often lobulated masses Heterogeneous Arise from the visceral pleura half are pedunculated.

32 Radiographic Features Chest computed tomography (CT): shows the size and location of the tumor and aids in surgical planning

33 Radiographic Features at least one obtuse angle with the pleural surface

34 Radiographic Features Some tumors located in the interlobar fissures occasionally, grow into the lung parenchyma, ( so-called inverted tumor) --- usually requires major lung resection Occasional pedunculated tumor will show movement with change position on fluoroscopy or on chest CT scan.1

35 Radiographic Features

36 Radiographic Features 2/3 of the tumors enhance with contrast administration, suggesting a more vascular Calcification: 26% of tumors. A small pleural effusion : 6% to 37%. Large lesions can compress adjacent vascular structures or bronchi. Rarely chest wall invasion or rib notching

37 Radiographic Features Magnetic resonance imaging (MRI) evaluating potential invasion of the chest wall by a sessile tumor. help to clarify the tumor s relationship to the diaphragm. The tumor typically shows heterogeneous signal intensity on T1-weighted images, and contrast enhancement following gadolinium administration. Positron-emission tomography (PET), little Few cases utilizing PET In one report of three SFTPs, two had no FDG uptake and one had only minimal uptake (SUV 2.1).

38 Treatment Surgery In virtually all cases, the mainstay of treatment of SFTP is surgical resection. Adjuvant Therapy Due to the rarity of these tumors, there is no systematic assessment of the role of adjuvant therapy in SFTP. Neoadjuvant therapy has been suggested for large malignant tumors.

39 Results of Treatment Localized benign SFTPs Almost cured with complete surgical resection Recurrences as long as 17 years after the initial resection up to an 8% recurrence Malignant SFTP relatively poor outlook despite a complete resection only a 12% long-term survival

40 Results of Treatment Histologic characteristics are useful in estimating the risk of recurrence in SFTP. de Perrot et al: classification of SFTP according to tumor characteristics and prognosis: (1) benign pedunculated tumors had a 2% recurrence rate, (2) benign sessile tumors had a 8% recurrence rate, (3) malignant pedunculated tumors had a 14% recurrence rate, and (4) malignant sessile tumors had a 63% recurrence rate and a 30% mortality, with most deaths occurring within 24 months

41 Results of Treatment