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They are benign lesions and malignant degeneration is rare. They are usually treated with curettage which however have a high recurrence rate of 25%. As such if an en-bloc resection is possible this is advisable
The diagnosis of a mediastinal germ cell tumor should be considered in all young males with a mediastinal mass. In addition to physical examination and routine laboratory studies, initial evaluation should include CT of the chest and abdomen, and determination of serum levels of HCG and alpha-fetoprotein.
Diagnosis is usually made by ultrasonography showing a solid ovarian lesion, or, on some occasions, mixed tumors with solid and cystic components. Computed tomography and magnetic resonance imaging can also be used to diagnose fibromas.
In a series of 16 patients, 5 (28%) showed elevated levels of CA-125.
The treatment of choice for both benign and malignant SFT is complete "en bloc" surgical resection.
Prognosis in benign SFTs is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery.
The prognosis in malignant SFTs is much more guarded. Approximately 63% of patients will have a recurrence of their tumor, of which more than half will succumb to disease progression within 2 years. Adjuvant chemotherapy and/or radiotherapy in malignant SFT remains controversial.
Plain film
often seen as a lobulated, eccentric radiolucent lesion
long axis parallel to long axis of long bone
no periosteal reaction (unless a complicating fracture present)
geographic bone destruction: almost 100%
well defined sclerotic margin: 86%
there can be presence of septations (pseudotrabeculation): 57% 2
there can be presence of matrix calcification in a small proportion of cases: 12.5%1
MRI
MR features are often not particularly specific. Signal characteristics include
T1 - low signal
T1 C+ (Gd) -
the majority (~70%) tend to show peripheral nodular enhancement
~ 30% diffuse contrast enhancement and this can be either homogeneous or heterogeneous 19
T2 - high signal
Bone scan
A scintigraphic "doughnut sign" has been described in this tumour type 11. However, this is very non-specific and can be found in a plethora of other bone lesions.
Usually the lesion is surgically removed. Primarily, there is concern that the lesion identified in a patient could be cancerous, but there is also the risk of torsion, and possibly the development of symptoms. A stable lesion, however, could be clinically followed.
Following thoracoabdominal trauma, most commonly a penetrating injury, laceration of the diaphragm, and spleen allows ectopic splenic tissue to reach the pleural space of the lung.
Affected persons are usually asymptomatic. However, on rare occasions, thoracic splenosis can present with chest pain and/or hemoptysis.
On radiological studies, thoracic splenic lesions are visualized using CT scans. Visualized lesions can be described as solitary or multiple nodules. The locations of the lesions are mostly in the lower left pleural space and/or splenic bed. Confirmation can be done using scintigraphy with 99mTc tagged heat-damaged red blood cells.
No treatment is required since thoracic splenosis is a benign condition.
Identification of pleural fluid biomarkers to distinguish malignant pleural effusions from other causes of exudative effusions would help diagnosis. Biomarkers that have been shown to be raised in malignant pleural effusions compared to benign disease include vascular endothelial growth factor (VEGF), endostatin, matrix metalloproteinases and tumour markers such as carcinoembryonic antigen. Pleural fluid mesothelin has a sensitivity of 71%, greater than that of cytology, and a specificity of 89% for the diagnosis of malignant mesothelioma.
Ectopic endometrial tissue reaches the pleural space of the lung or the right hemi-diaphragmatic region and erodes the visceral pleura, causing the formation of a spontaneous pneumothorax. The condition is often cyclical, due to its associations with the beginning of the menstrual cycle.
Affected persons usually present with recurrent spontaneous pneumothorax associated with the onset of the menstrual cycle. Additionally, chest/scapular pain and/or evidence of endometriosis in the abdominopelvic cavity are other manifestations.
On radiological studies, pneumothorax is visualized using conventional chest x-rays and CT scans. In 90% of the cases, the pneumothorax is located on the right side. In some cases, small nodules can be seen in the pleura using CT scans. Confirmation can be done using video assisted thoracoscopic surgery (VATS).
Treatment for the pneumothorax is with chest tube placement. As for the ectopic endometrial tissue, therapy with gonadotropin-releasing–hormone or resection of the lesions can improve symptoms.
Pleural fluid cytology is positive in 60% of cases. However, in the remaining cases, pleural biopsy is required. Image guided biopsy and thoracoscopy have largely replaced blind biopsy due to their greater sensitivity and safety profile. CT guided biopsy has a sensitivity of 87% compared to Abrams' needle biopsy, which has a sensitivity of 47%.
Benign fibromas may, but need not be, removed. Removal is usually a brief outpatient procedure.
Treatment is varied and depends on the site and extent of tumor involvement, site(s) of metastasis, and specific individual factors. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.
The standard treatment of COC is enucleation and curettage (E&C). Recurrence following E&C is rare.
Malignant germ cell tumors of the mediastinum are uncommon, representing only 3 to 10% of tumors originating in the mediastinum. They are much less common than germ cell tumors arising in the testes, and account for only 1 to 5% of all germ cell neoplasms.
Syndromes associated with mediastinal germ cell tumors include Hematologic Neoplasia and Klinefelter's syndrome.
About 80% of pleural SFTs originate in the visceral pleura, while 20% arise from parietal pleura. Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic at diagnosis. While some researchers have proposed that a SFT occupying at least 40% of the affected hemithorax be considered a "giant solitary fibrous tumor", no such "giant" variant has yet been recognized within the most widely used pleural tumor classification scheme.
Some SFTs are associated with the paraneoplastic Doege–Potter syndrome, which is caused by tumor production of IGF-2.
Recurrence rate of solid form of tumour is lower than classic form.
The treatment for CGCG is thorough curettage. A referral is made to an oral surgeon. Recurrence ranges from 15%–20%. In aggressive tumors, three alternatives to surgery are undergoing investigation:
- corticosteroids;
- calcitonin (salmon calcitonin);
- interferon α-2a.
These therapeutic approaches provide positive possible alternatives for large lesions. The long term prognosis of giant-cell granulomas is good and metastases do not develop.
Given the anatomic site, a spindle cell lipoma, nuchal-type fibroma and fibromatosis colli are all included in the differential diagnosis.
Simple excision is the treatment of choice, although given the large size, bleeding into the space can be a potential complication. Isolated recurrences may be seen, but there is no malignant potential.
Definitive diagnosis of these tumours is based on the histology of tissue obtained in a biopsy or surgical resection. In a retrospective study of 72 cases in children and adolescents, the histology was important to prognosis.
A number of molecules have been proposed as markers for this group of tumours. CD56 may be useful for distinguishing sex cord–stromal tumours from some other types of tumours, although it does not distinguish them from neuroendocrine tumours. Calretinin has also been suggested as a marker. For diagnosis of granulosa cell tumour, inhibin is under investigation.
On magnetic resonance imaging, a fibroma may produce one of several imaging features that might be used in the future to identify this rare tumour prior to surgery.
The cause of development for cardiac fibroma is still unknown or unexplained. Some of these cases are observed to be linked to Gorlin syndrome; a complex genetic disorder causing the formation of tumors in various parts of the body. Research is currently being undertaken to identify relevant casual factors. Currently, there are no known methods for preventing cardiac fibroma.
A retrospective study of 83 women with sex cord–stromal tumours (73 with granulosa cell tumour and 10 with Sertoli-Leydig cell tumour), all diagnosed between 1975 and 2003, reported that survival was higher with age under 50, smaller tumour size, and absence of residual disease. The study found no effect of chemotherapy. A retrospective study of 67 children and adolescents reported some benefit of cisplatin-based chemotherapy.
The following tests and exams are taken to diagnose Cardiac fibroma:
1. Family medical history and thorough physical examination that includes examination of the heart. Close attention to abnormal heart sounds is important.
2. Echocardiography: Most valuable diagnosis because this can evaluate the morphology, location and range of the tumor. Also, it can access the degree of blood flow obstruction caused by tumor.
3. Magnetic Resonance Imaging (MRI) and computed tomography CT scan of the heart
4. Electrocardiogram (EKG): this is used to measure electrical activity of the heart and to detect arrhythmias.
5. Electrophysiological studies of an individuals heart to determine where arrhythmia is generated in the heart.
6. Doppler ultrasound to measure the speed and direction of blood flow from sound waves.
7. Tissue biopsy: a pathologist may examine the biopsy under a microscope to suggest a definitive diagnosis. This is considered a gold standard in arriving to a conclusive diagnosis. Biopsy specimens are studied by using Hematoxylin and Eosin staining.
Unilocular radiolucency may be seen and mutilocular also, and mixed too .irregular calcifications may be seen in some cases.
Treatment of Meigs' syndrome consists of thoracentesis and paracentesis to drain off the excess fluid (exudate), and unilateral salpingo-oophorectomy or wedge resection to correct the underlying cause.