scholarly journals Fibromyxoid sarcoma of maxilla

Biomedicine ◽  
2021 ◽  
Vol 41 (4) ◽  
pp. 859-861
Author(s):  
Deviprasad Dosemane ◽  
Cheryl Sarah Philipose ◽  
Deepa Adiga ◽  
Meera Niranjan Khadilkar ◽  
Sanchit Bajpai

A recurrent mass in the nose can pose diagnostic and therapeutic challenges. Even more so, if it exhibits rapid growth, displays unusual clinical features, is a rare histopathological diagnosis or if the patient is unavailable for regular follow-up. We present a case of a middle-aged retrovirus-positive man who underwent surgical removal of a recurrent nasal mass. Histopathology revealed a diagnosis of low-grade fibromyxoid sarcoma, a rare entity in the sinonasal tract. Immunohistochemistry plays a crucial role in clinching the diagnosis, especially for lesions with fibrous and myxoid components. Early and complete surgical resection diminishes the likelihood of recurrence and metastasis.  

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 789
Author(s):  
Samy Chitayat ◽  
Rodrigo Barros ◽  
José Genilson Ribeiro ◽  
Heleno Augusto Moreira Silva ◽  
Flávio Rondinelli Sá ◽  
...  

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype. The most common tumor locations are the deep soft tissue of extremities or trunks. We report a rare case of recurrent LGFMS in the inguinal region involving the scrotum and both testicles. A 38-year-old male patient reported a history of multiple nodular lesions in the left inguinal region accompanied by local inflammation. The patient was submitted for local resection of the lesion at our institution, with histopathological diagnosis of LGFMS. He missed his follow-up, returning with a large bulge in the left inguinal region involving the scrotum with signs of tissue necrosis and local purulent discharge. Surgical exploration was performed and the patient underwent tumor resection in the left inguinal region and the entire scrotum, with bilateral orchiectomy, with the margins enlarged to the right inguinal region and proximal surface of the penis. Local reconstruction was performed with a left fascia lata tensor muscle flap and ipsilateral thigh coverage using partial skin graft. On microscopic examination, the tumor showed spindle cells arranged in bundles, with abundant collagen and myxoid stroma with interspersed prominent vessels. The immunohistochemical study carried out showed immunoreactivity with Ki67 (<5%), immunonegativity with desmin and S100, confirming the diagnosis of LGFMS. Postoperative recovery was good and no recurrence was seen after two years. The patient is in good health, realizing multidisciplinary outpatient follow-up and performing continuous testosterone replacement. Surgical resection with negative margins for localized disease remains the standard treatment for LGFMS.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 789
Author(s):  
Samy Chitayat ◽  
Rodrigo Barros ◽  
José Genilson Ribeiro ◽  
Heleno Augusto Moreira Silva ◽  
Flávio Rondinelli Sá ◽  
...  

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype. The most common tumor locations are the deep soft tissue of extremities or trunks. We report a rare case of recurrent LGFMS in the inguinal region involving the scrotum and both testicles. A 38-year-old male patient reported a history of multiple nodular lesions in the left inguinal region accompanied by local inflammation. The patient was submitted for local resection of the lesion at our institution, with histopathological diagnosis of LGFMS. He missed his follow-up, returning with a large bulge in the left inguinal region involving the scrotum with signs of tissue necrosis and local purulent discharge. Surgical exploration was performed and the patient underwent tumor resection in the left inguinal region and the entire scrotum, with bilateral orchiectomy, with the margins enlarged to the right inguinal region and proximal surface of the penis. Local reconstruction was performed with a left fascia lata tensor muscle flap and ipsilateral thigh coverage using partial skin graft. On microscopic examination, the tumor showed spindle cells arranged in bundles, with abundant collagen and myxoid stroma with interspersed prominent vessels. The immunohistochemical study carried out showed immunoreactivity with Ki67 (<5%), immunonegativity with desmin and S100, confirming the diagnosis of LGFMS. Postoperative recovery was good and no recurrence was seen after two years. The patient is in good health, realizing multidisciplinary outpatient follow-up and performing continuous testosterone replacement. Surgical resection with negative margins for localized disease remains the standard treatment for LGFMS.


1984 ◽  
Vol 61 (4) ◽  
pp. 665-673 ◽  
Author(s):  
Edward R. Laws ◽  
William F. Taylor ◽  
Marvin B. Clifton ◽  
Haruo Okazaki

✓ The authors conducted a retrospective review of surgically treated, histologically proven cases of low-grade (Grade 1 or 2) astrocytomas. Follow-up analysis, with survival time as the end-point, was completed using multivariant statistical analysis. In the 461 cases of supratentorial low-grade astrocytoma in this study, age of the patient at the time of surgery was by far the most important variable in predicting length of survival. Other variables correlating with increasing survival times were: gross total surgical removal, lack of major preoperative neurological deficit, long duration of symptoms prior to surgery, seizures as a presenting symptom, lack of major postoperative neurological deficit, and surgery performed in recent decades. The multi-variant regression analysis showed that radiation therapy was of clear benefit, primarily in older patients with incompletely removed tumors. For purposes of establishing prognosis and testing the results, a “score” was developed to predict survival times, based on the most important variables. The data in this study provide a basis for the analysis of future modes of management of low-grade gliomas.


2015 ◽  
Vol 33 (7_suppl) ◽  
pp. 345-345
Author(s):  
Ana Collazo Lorduy ◽  
Mireia Castillo-Martin ◽  
Grace Hyun ◽  
Nataliya Gladoun ◽  
Carlos Cordon-Cardo

345 Background: Bladder cancer is a rare entity in the pediatric population making it difficult to define surveillance protocols and long term outcomes. Notably, most pediatric tumors are low grade and non-muscle invasive and do not recur. In order to determine the source of the different natural history between pediatric population and adults, we hypothesized that pediatric bladder cancer may potentially stem from different molecular pathways than its adult form. Our main objective was to study the molecular pathogenesis in this rare disease using immunohistochemical (IHC) and mutational analysis of the main known genes involved in bladder cancer. Methods: Paraffin-embedded tissue slides of bladder tumors from three pediatric patients were retrospectively identified from our institution's pathology archives (1990-present) and re-evaluated. Clinical data was reviewed. FGFR3, H-RAS, and PI3K mutational analysis of the most well-known mutated spots was conducted by PCR amplification and Sanger sequencing. IHC analysis was conducted using antibodies against p53, Pten, Rb, EGFR, Her2Neu and ki-67. Results: Two patients had low-grade Ta disease, whereas the other tumor was classified as a Papillary Urothelial Neoplasm of Low Malignant Potential (PUNLMP). None of the lesions recurred. Two patients were female and one was male. The ages at diagnosis were 13, 11, and 17, with a mean follow-up of 5.2 years (Range: 1.5-8.0 years). All specimens showed H-RAS G12V mutation, whereas they were characterized by wild-type FGFR3 and PI3K. Nuclear p53 was not detected, whereas PTEN and Rb expression were maintained. EGFR was homogenously expressed in the three cases, and Her2Neu was negative. The proliferation rate analyzed by Ki-67 expression was very low in all cases (<5%). Conclusions: Pediatric tumors may arise from a pathway that is not initiated by FGFR3 or p53 mutations, but by H-RAS mutations. This distinction may explain the relatively few recurrences seen in the pediatric population. Molecular investigation of larger series of pediatric tumors is warranted, and will aid in determining the surveillance and the clinical follow up, if any, needed in this rare entity.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Masakazu Hashimoto ◽  
Kei Koide ◽  
Michinori Arita ◽  
Koji Kawaguchi ◽  
Yoshihiro Mikuriya ◽  
...  

A low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor, with a benign histologic appearance but malignant behavior. This report describes a 74-year-old man with an internal abdominal oblique muscle mass. The tumor appeared as a well-defined ovoid mass on computed tomography, with mild uptake on fluorine-18-fluorodeoxyglucose positron-emission tomography images. Radical resection with wide safe margins was performed. Histologically, the tumor was composed of spindle-shaped cells in a whorled growth pattern, with alternating fibrous and myxoid stroma. MUC4 expression, a highly sensitive and specific immunohistochemical marker for LGFMS, was detected. Therefore, we diagnosed the tumor as LGFMS. At the 3-month follow-up, there was no sign of recurrence or metastasis. We report the first case of LGFMS arising from the internal abdominal oblique muscle.


2021 ◽  
Vol 14 (5) ◽  
pp. e237083
Author(s):  
Muhammad Hammad Deewani ◽  
Muhammad Hassan Danish ◽  
Muhammad Sohail Awan ◽  
Nasir Ud Din

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon soft-tissue malignancy. LGFMS preferentially affects trunks and extremities of young adults; however, occasional cases have been reported in different sites of head and neck region including oral cavity, larynx and oropharynx. LGFMS usually exhibit areas of collagenised and myxoid stroma with appearance of spindle cells in whorling pattern. It is a challenge to diagnose it accurately as most of the time it is misdiagnosed as benign neoplastic entity of spindle cells. There have been only few isolated cases of LGFMS reported in head and neck region and LGFMS originating from the parapharyngeal space has never been reported before. We recently experienced a case of low grade fibomyxoid sarcoma in parapharyngeal space of neck. LGFMS have the propensity to locally recur and to metastasise. Due to its rarity in head and neck region, there are no well-established treatment and follow-up guidelines.


2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Kaya Kilic ◽  
Emre Unal ◽  
Zafer Orkun Toktas ◽  
Fugen Vardar Aker ◽  
Akın Akakın ◽  
...  

The authors present an extremely rare case of an aggressive and progressive vertebral capillary hemangioma of the lumbar spine secondary to a trauma. A 40-year-old man who complained of back and leg pain due to a hemangioma of L1 that had begun a year after the fracture of the same vertebra was subsequently operated on. Due to the profuse bleeding, only a subtotal removal was possible. Histopathological diagnosis of the lesion revealed a capillary hemangioma. Postoperative control MRI taken at eight months showed that the lesion and destruction of the L1 vertebra were progressive. A second embolization procedure was performed and this time the hemangioma was totally removed via an anterior approach and corpectomy. Fusion was achieved by Th12-L2 graft and plaque. In the fourteenth year of follow-up, he was symptom-free and radiologically clear of this lesion. We propose that progressive hemangioma is extremely rare and that its cure is possible by total surgical removal of the lesion. This case is the second extradural capillary hemangioma secondary to spinal trauma ever to have been documented in English literature. The emergence of a hemangioma in a fractured vertebra suggests that its pathogenesis can be related to the deviation of the angiogenetic pathways from the normal healing process.


2015 ◽  
Vol 47 (1-2) ◽  
pp. 16-20
Author(s):  
Zannatul Ferdous Jesmin ◽  
Afroza Khanam ◽  
Eti Saha ◽  
Md Mokter Hossain

Cervical cancer develops from early precancerous lesion known as cervical intraepithelial neoplasia (CIN). Khulna Medical college Hospital provides primary screening for preventing cervical cancer and a secondary referral centre for management of CIN by colposcopy. Primary objective of this study was to do an audit between January 2012 to February 2014 to update clinical efficacy of colposcopy based diagnosis, treatment and follow up of 510 CIN cases and also to document any shortcomings in existing services and suggestion for early rectification. Colposcopic findings in our study group were: 309(60.6%) cases were CIN-1, 124 (24.3%) cases was CIN II, and 49 (9.6%) cases were CIN III. Suspicious of invasive lesion was in 7 (1.4%) abnormal looking cervix and biopsy was done in 21(4.1%). Treatment procedure was done with individualization of cases and options were cold coagulation, Loop electrosurgical excision procedure (LEEP), hysterectomy, biopsy and post treatment follow up. Histopathological diagnosis was documented in 387 cases, where CINI was found in 161 (31.6%), CIN II in 92 (18%), CIN III in 26 (5.1%), non specific Inflammatory cervicitis in 87(17.1%), Squamous cell carcinoma in 15(2.9%), abnormalities consistent with koilocytic atypia in 6 (12%) and reports missed in 123 (24.1%). Cases results showed Positive predictive value (PPV) of 53% and 68% respectively for low grade (CIN I) and high grade lesion (CIN II, III). 481 patient were eligible for post treatment follow up but only 99 (20.5%) patient came and among them 74 (74.7%) were colposcopically negative, 25 (25.2%) had residual CIN and Risk Ratio (RR) was 0.25. Colposcopy is gold standard for diagnosis of CIN but our screening program is opportunistic and far way from population based. Histopathological correlation were often inaccurate with colposcopic diagnosis in practice and about one third case reports were missed. Majority of women did not complete follow up protocol. Residual or recurrent CIN lesion in dropped out cases would be a concern in near future. DOI: http://dx.doi.org/10.3329/bmjk.v47i1-2.22557 Bang Med J (Khulna) 2014; 47 : 16-20


2004 ◽  
Vol 100 (3) ◽  
pp. 431-437 ◽  
Author(s):  
Hugues Duffau ◽  
Ihab Khalil ◽  
Peggy Gatignol ◽  
Dominique Denvil ◽  
Laurent Capelle

Object. Although still controversial, many authors currently advocate extensive resection in the treatment of low-grade gliomas (LGGs). Because these tumors usually migrate along white matter pathways, the corpus callosum is often invaded. Nevertheless, there is evidently no specific study featuring resection of the corpus callosum infiltrated by glioma, despite abundant literature concerning callosotomy in epilepsy surgery or transcallosal ventricular approaches. The aim of this paper was to analyze functional outcome following removal of corpus callosum invaded by LGG and to analyze the impact of this callosectomy on the quality of resection. Methods. Between 1996 and 2002, a total of 32 patients harboring an LGG involving part of the corpus callosum and having no or only a mild preoperative deficit underwent surgery aided by intraoperative electrical mapping to preserve eloquent structures identified on stimulation and to perform the most extensive resection possible. Preoperatively, no clinical response was elicited on stimulation of the corpus callosum; thus, the part of this structure that was invaded by LGG was removed. Despite immediate postoperative neurological worsening, all patients but one recovered within 3 months and returned to a normal socioprofessional life. The additional callosectomy allowed for nine total resections, 18 subtotal resections, and five partial resections. Furthermore, only two cases of contralateral hemispherical migration occurred during a median follow up of 3 years. Conclusions. Resection of the corpus callosum infiltrated by glioma improves the quality of tumor removal without increasing the risk of sequelae.


2017 ◽  
Vol 80 (3) ◽  
pp. 281-283 ◽  
Author(s):  
Rohit Bhattar ◽  
S. P. Aggarwal ◽  
S. S. Yadav ◽  
V. Tomar

Sign in / Sign up

Export Citation Format

Share Document