|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 1 | Page : 160-161
Giant malignant solitary fibrous tumor: A rare thoracic mass in a patient of neurofibromatosis 1
Arjun Khanna1, Jagdish Chander Suri1, Animesh Ray1, Pooja Taneja2
1 Department of Pulmonary, Critical Care and Sleep Medicine, Safdarjang Hospital, New Delhi, India
2 Department of Pediatrics, Dr. B. L Kapur Memorial Hospital, New Delhi, India
|Date of Web Publication||13-Apr-2015|
Dr. Arjun Khanna
Department of Pulmonary, Critical Care and Sleep Medicine, Safdarjang Hospital, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Khanna A, Suri JC, Ray A, Taneja P. Giant malignant solitary fibrous tumor: A rare thoracic mass in a patient of neurofibromatosis 1. Arch Med Health Sci 2015;3:160-1
|How to cite this URL:|
Khanna A, Suri JC, Ray A, Taneja P. Giant malignant solitary fibrous tumor: A rare thoracic mass in a patient of neurofibromatosis 1. Arch Med Health Sci [serial online] 2015 [cited 2019 Jun 20];3:160-1. Available from: http://www.amhsjournal.org/text.asp?2015/3/1/160/154973
Solitary fibrous tumors (SFT) are rare neoplasms that most commonly involve the pleura, mediastinum, and lung. They are believed to be sub-mesothelial in origin. Histologically, they are characterized by fibroblast like cells and connective tissue in varying proportions. Approximately, 78-88% of SFTs are benign and 12-22% are malignant. Giant malignant SFT's of the thorax are rare tumors and literature is therefore scarce. We report a 14 year old, who presented with a large thoracic mass with pleural and liver metastasis. The diagnosis of malignant SFT was confirmed by histopathology and immunohistochemistry. The patient was also diagnosed to be suffering from Neurofibromatosis-1 (NF-1). The rarity of co-existence of malignant SFT and NF-1 urges us to report this case. A 14-year-old girl presented with low-grade fever, right-sided pleuritic chest pain, and upper abdominal discomfort for 1 month. A pleural tap was attempted and when it failed, she was referred to us for treatment. Clinical examination revealed that she, her father, and two brothers were also suffering from NF-1. On examination, she was found to have multiple cafe-au-lait spots all over the body and Lisch nodules in both the eyes. She had absent breath sounds in the right infra-axillary, infrascapular, and inframammary region. A hard nodular lesion was also palpable in the epigastrium and the right hypochondrium.
On investigation, hemoglobin was 8 mg/dl. Chest X-ray was suggestive of a large mass lesion in the right hemithorax. Contras enhanced computed tomography (CT) of thorax and abdomen revealed a mildly enhancing hypodense lobulated lesion with necrotic areas in the right paracardiac supradiaphragmatic region extending upto right lateral chest wall and almost completely filling the right hemithorax approximate size being 19 × 14 cm. The lesion was closely abutting the right cardiac border and extending inferiorly through the right dome of diaphragm and closely abutting and compressing the liver. Note was made of a few homogenous-enhancing lesion in segment 5-8 and segment 2a of the liver suggestive of metastasis. Multiple heterogenously enhancing deposits were seen in the right pleural cavity suggestive of metastasis. MRI of brain and spinal cord revealed hamartomas in bilateral basal ganglia and hypoplastic C4-C5 vertebral bodies consistent with NF-1.
Ultrasound-guided tru-cut biopsy revealed a tumor of spindle cells with elongated nuclei with abundant mitotic figures consistent with malignant SFT. Further immunohistochemistry was positive for CD 34 and vimentin and immunonegative for smooth muscle actin, desmin, S100 protein, factor 8, and CD 31. This confirmed the diagnosis of a malignant SFT. Owing to the large size of the lesion and metastasis, surgery was not offered to the patient. The patient was started on palliative Ifosfamide- and Doxorubicin-based chemotherapy. After the first cycle, the attendants refused further treatment and the patient succumbed to her illness.
Solitary fibrous tumors are rare fibrous tumors, the origin of which has been controversial. They have been variably called as localized fibrous tumors, sub-mesothelial fibroma, localized pleural mesothelioma, etc.  However, they are now considered to arise from sub-mesothelial areolar mesenchyme. Immunohistochemistry and electron microscopy have led to these neoplasms being named as solitary fibrous tumor.  Cytogenetic data have shown various abnormal karyotypes and supernumerary chromosome 8 has suggested malignant behavior of the tumor.  Solitary fibrous tumors occur in a wide age range (5-87 years) with a fairly equal frequency in both sexes.  Most patients with small benign lesions are diagnosed incidentally. However, large masses and frankly malignant tumors are also known. These tumors are now known to have accompanying paraneoplastic syndromes of which hypertrophic pulmonary osteoarthropathy is the most common.  Histologically, they exhibit a morphology varying from low grade spindle cell neoplasm to frank malignancy with mitotic figures and abnormal neoplastic cells. These tumors may assume alarming proportions. However, the size has been reported to not have any bearing on its being malignant, resectable, or curable.  Contrast enhanced CT is an important investigation as it clearly shows the size and the location of tumor and aids in surgical planning. Majority of SFTs exhibit homogenous alteration along with necrosis, hemorrhage, or cystic changes. The characteristic immunohistochemistry reveals that these tumors are immunopositive for CD 34 and vimentin. The criteria for malignancy are presence of necrosis, increased mitotic activity (> = 4 mitotic figures/10 hpf), greater cellularity, and cellular polymorphism. SFT should be considered as malignant in the presence of at least one of these criteria.  The cornerstone of treatment is large en-bloc resection.  Mediastinal lymphnode dissection is not indicated and adjuvant treatment is not usually employed.  In case of distant metastasis or locally advanced disease as was in this case, palliative radiotherapy or chemotherapy may be considered. The most common type of chemotherapy to be employed is Doxorubicin +/− ifosfamide.
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