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Oct 2022 DOI 10.14302/issn.2641-5518.jcci-22-4323
Introduction Primary sarcomas of the breast are <0.1% of all malignant tumours of the breast. To date, there are 13 major breast sarcoma series in English literature. This study adds to these series characterizing primary breast sarcoma among Philippine patients. Methods All breast biopsies from the pathology records of the University of the Philippines-Philippine General Hospital (UP-PGH) were searched for breast sarcoma cases from January 2000 to December 2010. Metaplastic carcinomas and phyllodes tumors were excluded. Results There were 52 patients (45 female, 7 male) ranging in age 25-83 years (median 46 years). Majority had lump, ten cases with pain. No history of previous cancer was given. No history of prior radiation was found. Histopathological diagnoses were spindle cell sarcoma (n=13), fibrosarcoma (n=6), liposarcoma (n=6), MPNST (n=5), stromal sarcoma (n=5), angiosarcoma (n=4), MFH (n=4), leiomyosarcoma (n=3), rhabdomyosarcoma (n=3), chondrosarcoma (n=2), and synovial sarcoma (n=1). Tumors were with grade 1 (n=18), grade 2 (n=8), and grade 3 (n=10). Necrosis was noted in 6 cases. Simple mastectomy was done in 19 cases (37%), MRM in 31 cases (59%), while 2 far advanced had no surgery (3%). None had adjuvant radiotherapy or chemotherapy. The duration of follow-up for 45 patients ranged from 1 – 117 months, excluding those who were lost to follow-up. All 15 deaths were due to progressive disease. Recurrences were observed in 9 patients. The disease-free survival (DFS) and overall survival (OS) was 73%and 75%, respectively. On multivariate analysis, DFS and OS were significantly correlated with size (HR=113.63; p=0.019 and HR=77.36; p=0.037), grade (HR=20.73 ; p=0.003andHR= 39.57; p= 0.004), and having a histology of angiosarcoma (HR=35.20 ; p=0.005and HR= 50.74; p=0.007), respectively. Conclusion Sarcoma remains an important clinical entity among primary breast cancers.
Nov 2021 DOI 10.14302/issn.2470-0436.jos-21-4016
Malignant transformation of peripheral nerve sheath tumor (MPNST) may develop from a plexiform type of Neurofibromatosis 1 (NF1) or previously irradiated areas. Generally, MPNSTs occur in about 2% to 5% of neurofibromatosis patients. In this paper, we present a 58-year-old male patient with neurofibromatosis who developed MPNST of the eyelids and nasal area. The patient had a history of multiple excision biopsies for facial tumors in 22 years at different institutions, allegedly revealing neurofibromas on histopathological evaluation. A recent consult with the Otorhinolaryngology Service (ORL) prompted an excision biopsy with results consistent with neurofibroma. The mass recurred and enlarged even more rapidly compared to the previously excised tumor. The patient also developed four tumors on the eyelids hence the referral to Ophthalmology Service. The eyelid masses and nasal mass were excised by the Ophthalmology and ORL Services. Histopathology revealed identical MPNST characteristics on all excised tumors. The patient was eventually referred to the Oncology Service to evaluate radio and chemotherapy. A rapid change in the size of a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain usually indicates a possible malignant transformation into MPNST. A high index of suspicion is helpful for clinicians when presented with a case of a recurrent neurofibromatosis, even if the only sign is the rapid growth of the mass since management of MPNST is very different from neurofibromatosis.
Dec 2015 DOI 10.14302/issn.2574-4496.jtc-15-779
It is extremely rare that sarcomas metastasize to the thyroid. We report a case of a 49 year old male with malignant peripheral nerve sheath tumor (MPNST) metastatic to the thyroid that was diagnosed by ultrasound guided fine needle aspiration (FNA). The FNA cytology showed numerous loosely cohesive pleomorphic small spindle cells, some of which were arranged in short fascicles or haphazard pattern. The nuclei were oval or spindle in shape, with hyperchromatic granular chromatin and inconspicuous nucleoli, and smooth nuclear membrane contours. The cytoplasm was scant to moderate in amount, and delicate. Some cells had long thin cytoplasmic projections. Based on the cytomorphology, a diagnosis of “consistent with metastatic MPNST from small intestine” was rendered and follow-up thyroidectomy confirmed the cytologic diagnosis. Therefore, FNA biopsy is a useful, easy to perform, cost effective, safe procedure that can diagnose secondary tumors of the thyroid, and help avoid unnecessary thyroidectomy in patients with a poor prognosis.