scholarly journals Epithelioid Variant of High-Grade Myxofibrosarcoma in the False Vocal Fold: A Case Report

2021 ◽  
Vol 64 (12) ◽  
pp. 954-958
Author(s):  
Seulki Song ◽  
Dae Hyun Song ◽  
Jin Pyeong Kim

Myxofibrosarcoma (MFS) is a histologic subtype of malignant fibrous histiocytoma (MFH), with a predominant myxoid component. MFS is characterized by locally aggressive behavior and a high rate of local recurrence, however, with a good prognosis. Head and neck MFS accounts for 3% of all cases of MFS. To date, only two cases of laryngeal MFS have been reported. Owing to the rarity of MFS, the clinical characteristics and optimal treatment options remain controversial. Surgical resection with a clear margin is considered the treatment of choice. Compared to traditional MFS tumors, epithelioid variants have worse prognosis. Other factors associated with a poor prognosis of MFS tumors include inadequate surgical margins, large tumor size, old age, and high-grade tumors. Herein, we report a case of high-grade epithelioid variant MFS located in the false vocal fold, requiring total laryngectomy to obtain an adequate surgical margin. To our knowledge, this is the first case report of epithelioid variant of high-grade MFS presenting in the larynx.

2020 ◽  
Vol 1 (1) ◽  
pp. 34-38
Author(s):  
Hussein Hmadeh ◽  
Mohammad Rakka ◽  
Layan Abbas ◽  
Nizar Bitar ◽  
Raed Chehab El Dine ◽  
...  

Background: Primary leiomyosarcoma (LMS) of the breast is an extremely rare histologic subtype of malignant breast tumors. While breast sarcomas account for 0.0006% of all breast malignancies, primary leiomyosarcoma constitutes only 5-10% of breast sarcomas themselves. To date, the total number of reported cases in the literature is less than 70 cases with the first case reported in 1968. This is, to the best of our knowledge, the first case reported from Lebanon, with only 2 cases previously reported in the Middle East, specifically in Morocco. Case Report: We report the case of a 48-year-old, white, previously healthy female patient, presenting for a breast mass. She first noticed it three months prior to presentation in her left breast and reports that since then, the lump has been growing and her breast has become more painful and erythematous over time. Physical examination revealed an immobile and tender mass of around 12 cm in diameter. A core biopsy was done followed by a left modified radical mastectomy with lymph node dissection. Pathology of the specimen showed a tumor composed of spindle cells arranged in fascicles invading the dermis, with irregular nuclei and prominent nucleoli. Immunohistochemical staining confirmed the diagnosis of high-grade leiomyosarcoma. Conclusion: We presented this case of leiomyosarcoma with lymph node metastasis to contribute to the scarce literature regarding this disease. Due to the rarity of this diagnosis, not enough data exists regarding treatment and prognosis. Physicians need to review the literature for relevant cases to achieve the best outcome for their patients. Keywords: breast mass, leiomyosarcoma, rare, case report, breast sarcomas, lymph node


2009 ◽  
Vol 36 (2) ◽  
pp. 235-238 ◽  
Author(s):  
M. Angelillo ◽  
S. Mazzone ◽  
G. Costa ◽  
A. Mazzone ◽  
U. Barillari
Keyword(s):  

Author(s):  
Vincenzo Dario Mandato ◽  
Riccardo Valli ◽  
Monica Silvotti ◽  
Valentina Mastrofilippo ◽  
Giovanni Casali ◽  
...  

2021 ◽  
Vol 11 ◽  
Author(s):  
Quanquan Gu ◽  
Yajing Huang ◽  
Hao Zhang ◽  
Biao Jiang

BackgroundDiffuse midline glioma with H3K27-altered (DMG-H3K27a) is a novel tumor entity of the pediatric-type diffuse high-grade tumor in the latest WHO CNS 5. It mostly affects children and is only rarely found in adults. The tumor has a high level of aggressiveness, with a rapid progression and bad prognosis. In adults, the spinal cord is the most common site of DMG-H3K27a. Rare adult cases of primary DMG-H3K27a in the spinal cord were reported in this study, together with clinico-histopathologico-radiographic data.MethodsFrom January 2016 to December 2020, we conducted a retrospective study of five adults with primary DMG-H3K27a in the spinal cord, analyzing their clinical, pathohistological, and radiographic datasets from the first diagnosis to follow-up.ResultsAll five patients were diagnosed for the first time and were given full treatment. In three of the five patients, post-operative follow-up revealed tumor recurrence. The longest survival of the five patients was 45 months at the time of report submission, while the longest progression-free survival (PFS) following surgery was 20 months. Immunohistochemical studies showed the tumors featured aggressive behavior (grade 4) and were positive for the H3K27M mutation. The radiographic appearances were varied, but they were all initially mistaken as benign. DMG-H3K27a in the spinal cord was characterized by isointense/hyperintense on T1WI and isointense/hyperintense on T2WI, as well as cystic necrosis and peripheral spinal cord edema, as well as central canal enlargement and other types of enhancement.ConclusionThis is the first case report focusing on adult DMG-H3K27a of the pediatric-type diffuses high-grade gliomas in the spinal cord. In our cases, we discovered the following: 1) adults had a better prognosis with a longer PFS compared with prior pediatric reports; 2) despite aggressive behavior under the microscope, radiographic appearances of the tumors were less aggressive; and 3) adjuvant treatment, including TCM, may have played a role in the prognosis.


2021 ◽  
Vol 14 (4) ◽  
pp. e240780
Author(s):  
Tiago Correia de Sá ◽  
Jorge Silva ◽  
Carla Freitas

Paraoesophageal hernias are common, but symptoms are not always present nor pathognomonic of this condition, and patients may be suffering for many years before a diagnosis is made. Incarceration and strangulation are rare, but they are life-threatening complications, warranting an emergent surgical repair. Hiatal hernia incarceration causing distal oesophagus perforation is even rarer. To our knowledge, this is the first case report in adult patients. Despite many advances in care, a high grade of suspicion is needed to diagnose this condition, and the mortality rate for an oesophageal perforation remains high, achieving 50% in some series. We present the surgical management of a case of hiatal hernia incarceration with distal oesophagus perforation by combined laparoscopy and upper endoscopy.


CytoJournal ◽  
2014 ◽  
Vol 11 ◽  
pp. 4 ◽  
Author(s):  
Julie Huss ◽  
Rachel Conrad ◽  
Sharon Hirschowitz ◽  
Neda Moatamed

Salivary duct carcinoma (SDC) comprises a small proportion of salivary gland tumors; however, it is known to be aggressive with a high rate of metastasis. Although frequent references are made to pulmonary dissemination, metastases in the pleural fluid have not been described. In this article, we report the cytologic features of metastatic SDC in the pleural fluid. The clinical history, cytomorphology and immunohistochemical features used for diagnosis are described. To the best of our knowledge, this is the first case of pleural fluid involvement by salivary duct carcinoma reported in the literature.


2021 ◽  
Vol 5 (3) ◽  
pp. 43-50
Author(s):  
Mercedes Bravo-Taxa ◽  
◽  
Luis Taxa-Rojas ◽  
Aldo López-Blanco ◽  
◽  
...  

Primary sarcomas of the vulva are rare, with an incidence of 1.5% to 5% of all vulvar malignancies. There are only 41 cases described in the English literature of proximal epithelioid sarcoma of the vulva, which also constitutes a highly aggressive neoplasm with a high rate of recurrence and metastasis. We are reporting the first case of vulvar epithelioid sarcoma reported in our country, Peru, and Latin America. We present the case of a 53-year-old woman with no significant history who presented a large tumor mass of 25 cm in the left hemivulva of 2 years of evolution, associated with intense pain. She underwent radical surgical resection with left inguinopelvic lymphadenectomy and reconstruction of Rectus Abdominal Muscle Myocutaneous Flaps (TRAM).


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Hirotaka Yonezawa ◽  
Norio Yamamoto ◽  
Katsuhiro Hayashi ◽  
Akihiko Takeuchi ◽  
Shinji Miwa ◽  
...  

Abstract Background Low-grade myofibroblastic sarcoma (LGMS) is described as a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for the head and neck, especially the oral cavity and larynx. LGMS arising in the levator scapulae muscle is extremely rare. Case presentation A 69-year-old woman was admitted to our hospital because she noticed a hard mass in her left neck six months prior. Magnetic resonance images (MRI) showed a soft tissue tumor of the left levator scapulae muscle. A core needle biopsy showed cellular fascicles or a storiform growth pattern of spindle-shaped tumor cells with minimally atypia. Immunohistochemistry revealed focally positive for α-smooth muscle actin (α-SMA), negative for S-100, and a low-grade spindle cell sarcoma was suspected. Following a biopsy, the tumor was resected with a wide surgical margin. Immunohistochemical staining was a positive for vimentin and α-SMA and negative for desmin, CD34, nuclear β-catenin, and h-caldesmon. LGMS diagnosis was determined based on the histopathological findings. The patient was alive with no evidence of disease eight years after the surgery. Conclusions To the best of our knowledge, this is the first case report of LGMS arising in the levator scapulae muscle. In addition to the case report, 48 reports with 103 LGMS cases are reviewed and discussed. In previous reports of LGMS, there were 43 females and 60 males, with a mean age of 43.0 years (range, 2–75). There were 13 (12.6%) patients aged < 18 years, 67 (65.1%) patients aged 18 to 59 years, and 23 (22.3%) patients aged ≥60 years. The average tumor size was 4.4 cm (range: 0.4–22.0). The commonest sites of LGMS was the tongue. Tumor growth patterns were evaluated in 52 cases, and 44 cases (84.6%) showed infiltrative growth patterns. Local recurrence was 26.7%, and distant metastasis was 4.4%. Because of the locally aggressive feature, it is important to diagnose LGMS with biopsy and to excise the tumor with an adequately wide margin.


2016 ◽  
Vol 2 (3) ◽  
pp. 161
Author(s):  
Evita Bonita Henderson-Jackson ◽  
Jaime Caracciolo ◽  
Douglas Letson ◽  
Marilyn Bui

<p>Dedifferentiated chordoma is a rare and aggressive malignant bone tumor. It is known as a variant of conventional chordoma, which possesses additional high-grade sarcomatous elements. Dedifferentiated chordoma is commonly identified in axial location as recurrences, or following radiation therapy of primary tumors. Prognosis is poor and there is a potential risk for metastasis. There have been only a few reports of primary dedifferentiated chordoma. Owing to its rarity, especially when it is in an unusual extra-axial location, primary dedifferentiated chordoma presents a diagnostic challenge. Brachyury, a recently described immunohistochemical marker specific for chordoma, has improved the diagnostic accuracy of chordoma and its variants in extra-axial sites. We herein report an exceptional case of extra-axial dedifferentiated chordoma confirmed by the expression of brachyury, the first case report of this kind. The review of relevant literature and discussion of practical diagnostic approach from sarcoma pathologists’ perspective are intended to provide an update of this topic. </p>


Sign in / Sign up

Export Citation Format

Share Document