scholarly journals Giant Low-Grade Fibromyxoid Sarcoma in the Neck

Author(s):  
Jong Min Park ◽  
Hye Rin Lim ◽  
Jo Heon Kim ◽  
Dong Hoon Lee

Low-grade fibromyxoid sarcoma (LGFS) is a soft tissue tumor that rarely occurs in the head and neck region. It occurs mainly in the proximal extremities and the trunk and is prevalent in the young and middle-aged adults. In the present case, LGFS was present at an atypical location and at an unusual age. The treatment of choice for LGFS is radical wide surgical excision with a clear margin. Long-term follow-up is essential for all patients with LGFS, as it has the potential for late recurrence or metastasis.

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.


2011 ◽  
Vol 25 (1) ◽  
pp. 19
Author(s):  
Robert A. Edelstein

Low grade fibromyxoid sarcomas are rare lesions that typically develop in the soft tissue of the extremities and trunk. This patient presented with a somewhat unusual location in the mons pubis, and was treated by primary excision. She will require long term follow up, as local recurrences and late metastases are possible


2021 ◽  
Vol 09 (02) ◽  
pp. E258-E262
Author(s):  
Christian Suchy ◽  
Moritz Berger ◽  
Ingo Steinbrück ◽  
Tsuneo Oyama ◽  
Naohisa Yahagi ◽  
...  

Abstract Background and study aims We previously reported a case series of our first 182 colorectal endoscopic submucosal dissections (ESDs). In the initial series, 155 ESDs had been technically feasible, with 137 en bloc resections and 97 en bloc resections with free margins (R0). Here, we present long-term follow-up data, with particular emphasis on cases where either en bloc resection was not achieved or en bloc resection resulted in positive margins (R1). Patients and methods Between September 2012 and October 2015, we performed 182 consecutive ESD procedures in 178 patients (median size 41.0 ± 17.4 mm; localization rectum vs. proximal rectum 63 vs. 119). Data on follow-up were obtained from our endoscopy database and from referring physicians. Results Of the initial cohort, 11 patients underwent surgery; follow-up data were available for 141 of the remaining 171 cases (82,5 %) with a median follow-up of 2.43 years (range 0.15–6.53). Recurrent adenoma was observed in 8 patients (n = 2 after margin positive en bloc ESD; n = 6 after fragmented resection). Recurrence rates were lower after en bloc resection, irrespective of involved margins (1.8 vs. 18,2 %; P < 0.01). All recurrences were low-grade adenomas and could be managed endoscopically. Conclusions The rate of recurrence is low after en bloc ESD, in particular if a one-piece resection can be achieved. Recurrence after fragmented resection is comparable to published data on piecemeal mucosal resection.


FACE ◽  
2021 ◽  
pp. 273250162110536
Author(s):  
Joshua Harrison ◽  
Samantha Marley ◽  
Shawhin Shahriari ◽  
Christian Bowers ◽  
Anil Shetty

We report a rare case of an extramedullary plasmacytoma (EMP) in the frontal sinus with an indolent clinical presentation. Although a history of trauma was absent, the initial diagnosis was a mucocele, based on the radiological findings. Upon surgical excision, the patient was found to have an EMP. EMP, a form of solitary plasmacytoma, has a significantly high rate of conversion to multiple myeloma. This mandates long-term follow-up, even after successful radiotherapy and/or resection. While radiation therapy is generally considered a first line treatment for EMP, surgical intervention may provide optimal treatment in complicated cases. This case presentation highlights the prognosis of patients diagnosed with EMP.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii374-iii374
Author(s):  
Ai Muroi ◽  
Takao Tsurubuchi ◽  
Hidehiro Kohzuki ◽  
Ryoko Suzuki ◽  
Hiroko Fukushima ◽  
...  

Abstract OBJECTIVE The natural course of incidentally discovered small intracranial lesions has not been well discussed. Surgical intervention, including resection and biopsy, could be achieved if the lesion is growing. We present 13 cases with incidentally found, small non-enhancing lesions without related symptoms. METHODS We retrospectively reviewed a series of 13 children with T1 hypointense and T2 hyperintense intracranial lesions less than 20 mm in diameter without enhancement. We excluded the patients with NF-1 or Tuberous sclerosis. RESULTS Most patients underwent MRI for headache unrelated to the lesions. All cases were located supratentorially. The median age of the patients at the initial examination was 8.9 years (range, 2.2–14.6). Of these children, 2 patients (15.3%) underwent surgery because of progression on follow-up MR images. The pathological diagnosis was compatible with diffuse astrocytoma. Patients were followed for a median of 55 months (range, 11–87) and the overall survival rate was 100%. No patient experienced increase in size after 3 years of follow-up. CONCLUSIONS In most patients with small intracranial lesions, the lesions remained stable and conservative management was appropriate. However, in a few cases, the lesions changed in size or quality and surgical intervention was necessary. Long-term follow-up at least 3 years is mandatory.


2000 ◽  
Vol 118 (4) ◽  
pp. A764
Author(s):  
In Sung Song ◽  
You Sun Kim ◽  
Il Ju Choi ◽  
Joo Sung Kim ◽  
Hyun Chae Jung ◽  
...  

2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.


2011 ◽  
Vol 1 (3) ◽  
pp. 222-226 ◽  
Author(s):  
Sumaporn Makkun ◽  
Jenjeera Prueksadee ◽  
Jatuporn Chayakulkheeree ◽  
Darunee Boonjunwetwat

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