scholarly journals Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children

2021 ◽  
Vol 8 (1) ◽  
pp. 1-9
Author(s):  
Primali Rukmal Jayasooriya ◽  
Chamara Athukorala ◽  
Manjula Attygalla ◽  
Balapuwaduge Ranjit Rigobert Nihal Mendis ◽  
Tommaso Lombardi

Low-grade myofibroblastic sarcoma (LGMS) is a mesenchymal tumor of myofibroblasts that occurs more frequently in adults. A series of three cases is presented to illustrate that LGMS may also occur within the oral cavity in children and adolescents. The first case (Case 1) occurred intra-osseously in the mandible, while the remaining two presented as gingival swellings and were purely restricted to soft tissue (cases 2 and 3). The intra-osseous lesion arose in a 7-year-old girl, whereas the gingival lesions were observed in a 12-year-old girl (Case 2) and a 13-year-old boy (Case 3). Histopathologically, all cases were composed of spindle shaped cells arranged into long fascicles showing mild to moderate degree of nuclear atypia. Ki-67 (MIB-1) proliferation activity was relatively low, amounting to 3–5% in all cases. Immunohistochemically, all cases showed smooth muscle actin (SMA) positivity in spindle cells, while desmin, beta catenin, cytokeratin, and CD34 were negative, resulting in a diagnosis of LGMS. In conclusion, current series of three cases of LGMSs that occurred in the oral cavity in a child and two adolescent patients is presented to highlight an emerging disease that requires additional data for further characterization.

2018 ◽  
Vol 103 (9) ◽  
pp. 1259-1265 ◽  
Author(s):  
Xiao-Yi Qin ◽  
Zhe-Hao Jin ◽  
You-Pei Wang ◽  
Zong-Duan Zhang

Background/aimsTo describe the clinicopathological and immunohistochemical characteristics of 10 patients representing a new entity of benign conjunctival myxoid stromal tumours.MethodsRetrospective review of clinical findings, histopathological and immunohistochemical studies identified 10 cases of low-grade conjunctival myxoid stromal tumours. Specimens were routinely processed and stained with H&E. Immunohistochemical stains for CD34, CD68, vimentin, S100, smooth muscle actin (SMA), myosin, desmin, actin, Bcl-2 and Ki-67 were performed. Specific stains for Alcian-blue periodic acid-Schiff (AB-PAS) and aldehyde fuchsin stains were also performed.ResultsTen patients with an average age of 45.6±11.1 years had a tender white or faint yellow to red mass on the bulbar conjunctiva. All the lesions were completely removed, and none of the patients relapsed. Histologically, all neoplasms consisted of spindle-shaped cells that showed signs of pseudonuclear inclusions, multinuclear cells and had no atypia. The stroma consisted of a large amount of mucus and was infiltrated with delicate to ropey collagens, a few mast cells and new vessels. Immunohistochemical stains were positive for CD34, vimentin and Bcl-2; partial positive for CD68; very low for Ki-67; and negative for S100, SMA, myosin, desmin and actin. AB-PAS suggested that the stroma was mucinous.ConclusionsThese rare benign mesenchymal conjunctival tumours are mostly unilateral and occur in the bulbar conjunctiva. Complete resection is the radical treatment. These lesions are characterised by multiple spindle cells, a large amount of mucus, and sharing similar basic histopathological features with conjunctival myxoma and conjunctival stromal tumour. We suggest naming these lesions ‘conjunctival myxoid stromal tumours’.


2019 ◽  
Vol 6 (2) ◽  
pp. 633
Author(s):  
Suresh K. Choudhary ◽  
Shalu Gupta ◽  
Somendra Bansal ◽  
Narender Kumar

Low-grade myofibroblastic sarcoma (LGMFS) is an uncommon tumor which develops mainly in the bone or soft tissues of the head and neck region, trunk, or extremities and extremely rarely found in the abdominal cavity. The rarity of the disease and its low-grade features make an accurate diagnosis difficult in most cases. We recently encountered a giant LGMFS which had developed in retroperitoneum and surgically resected with gratifying results. An 18 years old female presented with complaint of left sided abdominal pain since 8 months and left sided abdominal lump since 5 months. Imaging examinations revealed retroperitoneal soft tissue mass, and surgical treatment was scheduled. During operation, a tumor about 20x18x15cm in diameter with its anterior aspect covered with the pancreas, mesocolon and splenic flexure of colon with densely adhered to splenic vessels and left renal vessels was found. The tumor had firm adhesions to the surrounding tissues, and it was excised with concomitant distal pancreato-splenectomy, left nephrectomy and resection of splenic flexure of colon with colo-colic anastomosis. Histopathologically, fusiform cells were arranged in a complicated or storiform pattern, and immunohistochemical staining revealed that the tumor was positive for vimentin and focally positive for α-smooth muscle actin (SMA), negative for desmin, CD34, CD117, EMA, DOG-1 and S-100. Diagnosis of LGMFS was made. During 6 months follow up patient is asymptomatic and ultrasound abdomen is normal. In case of LGMFS, favorable prognosis can be attained by complete resection of the primary lesion and regular follow up of patient by physical examination and imaging modality.


2020 ◽  
Vol 6 (1) ◽  
pp. 49-51
Author(s):  
Annapurna Ahuja ◽  
Sharmila Kumari ◽  
Aiswarya Mishra ◽  
Ruchi Minz

Pyogenic granulomas are relatively common benign mucocutaneous lesions found in the oral cavity or extra orally; The first case was reported in 1844 by Hullihen and the term “pyogenic granuloma” or “granuloma pyogenicum” was coined only in 1904 by Hartzell. It is a reactive tumor like lesion which arises in response to various stimuli such as chronic low grade irritation[1], trauma and hormonal imbalance[2]. It's an inflammatory hyperplasia commonly seen in the oral cavity caused due to chronic irritation and trauma. In this case report we present a case of pyogenic granuloma associated with gingival/bony fenestration caused due to chronic irritation by exposed root tip.


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.


Author(s):  
Frederica Demarosi ◽  
Alessandro Bay ◽  
Laura Moneghini ◽  
Antonio Carrassi

2020 ◽  
Vol 26 (2) ◽  
pp. 35-36
Author(s):  
Jefferson Tenório ◽  
Frederico Medeiros ◽  
Machado Orsini ◽  
Santos dos

Low grade myofibroblastic sarcoma is an atypical myofibroblastic malignant neoplasm that rarely affects the oral cavity and presents a challenging histological diagnosis. Here we report the case of a 38-year-old woman who had a nodular and symptomatic lesion on the lateral border of the tongue. After the biopsy, the histopathological analysis revealed a spindle cell lesion with fascicular and storiform pattern. We performed immunohistochemical analysis that showed intense labeling for vimentin, smooth muscle actin and moderate labeling for calponin and S-100. We emphasize the rarity of low grade myofibroblastic sarcoma such as a nodular lesion on the tongue and the need for accurate diagnosis.


2016 ◽  
pp. 138-140
Author(s):  
S.I. Zhuk ◽  
◽  
O.A. Taran ◽  
A.N. Koshmienskaya ◽  
T.V. Lobastova ◽  
...  

The objective: the finding of protein expression of apoptosis regulator BCL-2, Smooth Muscule Actin and the antigen Ki-67 in cervical intraepithelial neoplasia of different severity to optimize the diagnosis and prognosis of the disease. Patients and methods. The study involved 42 women of reproductive age with cervical intraepithelial the neoplasia of the cervix varying degrees applied to the doctor of cervical pathology Zhitomir regional oncologic dispensary. All women (n=42) were divided into groups. The first group included 15 patients (35.7%) with cervical intraepithelial neoplasia with mild. The second group included 13 women (31%) with cervical intraepithelial neoplasia a moderate degree. The third group was represented by patients with cervical intraepithelial neoplasia with severe – 14 respondents (33.3 per cent). Results. Marker BCL-2 in patients of the first group was positive in 7 patients (46.7%), Smooth Muscule Actin was positive in 9 patients (60%) and Ki-67 was diagnosed in 8 of the surveyed women (53.3%). In the second group of BCL-2 was positive in 8 patients (61.5%), Clone 124, Smooth Muscule Actin, Clone 1A4 was positive in 9 patients (69.2%), and Ki-67 was diagnosed in 12 of the surveyed women (92.3%). Marker BCL-2 in patients of the third group was positive in 12 patients (85.7%), Smooth Muscule Actin was positive in 10 patients (71.4%) and Ki-67 was diagnosed in 13 of the surveyed women (92.9% ). Conclusion. Carcinogenesis is associated with molecular genetic damage to the cervix. Some of the products of this process can be used as prognostic and diagnostic markers of tumor progression. Determination of protein expression of apoptosis regulator BCL-2, Smooth Muscule Actin and the antigen Ki-67 in cervical intraepithelial neoplasia makes it possible to accurately verify the diagnosis and to predict the course of pathological changes in the flat epithelium of the cervix. Key words: cervical intraepithelial neoplasia, cervical cancer, morphological diagnostics of precancerous lesions, BCL-2, Smooth Muscule Actin, Ki-67.


Author(s):  
Kunihiko Matsuno ◽  
Yoshikazu Kanazawa ◽  
Daisuke Kakinuma ◽  
Nobutoshi Hagiwara ◽  
Fumihiko Ando ◽  
...  

AbstractReports of gastric collision tumors, comprising adenocarcinoma and gastrointestinal stromal tumor, are extremely rare. Here, we report the case of a 68-year-old male who was diagnosed with a lower-body, moderately differentiated, tubular-type adenocarcinoma and submucosal tumor and underwent an elective D2 distal gastrectomy. The tumor cells of the gastrointestinal stromal tumor were positive for H-caldesmon and CD117, weakly positive for smooth muscle actin and DOG-1, and negative for desmin, S-100 protein, CD31, and AE1/AE3. The tumor had grown into a mixed form of adenocarcinoma and gastrointestinal stromal tumor. Thus, we report the first case of a preoperatively diagnosed collision tumor in the stomach consisting of adenocarcinoma and gastrointestinal stromal tumor.


2021 ◽  
Vol 22 (4) ◽  
pp. 2048
Author(s):  
Pierluigi Giampaolino ◽  
Virginia Foreste ◽  
Claudia Di Filippo ◽  
Alessandra Gallo ◽  
Antonio Mercorio ◽  
...  

Polycystic ovary syndrome (PCOS) is a complex and heterogeneous endocrine disease. The hypothesis that alterations in the microbiome are involved in the genesis of PCOS has been postulated. Aim of this review is to summarize the available literature data about the relationship between microbiome and PCOS. A search on PubMed and Medline databases was performed from inception to November 20Most of evidence has focused on the connection of intestinal bacteria with sex hormones and insulin-resistance: while in the first case, a relationship with hyperandrogenism has been described, although it is still unclear, in the second one, chronic low-grade inflammation by activating the immune system, with increased production of proinflammatory cytokines which interfere with insulin receptor function, causing IR (Insulin Resistance)/hyperinsulinemia has been described, as well as the role of gastrointestinal hormones like Ghrelin and peptide YY (PYY), bile acids, interleukin-22 and Bacteroides vulgatus have been highlighted. The lower genital tract microbiome would be affected by changes in PCOS patients too. The therapeutic opportunities include probiotic, prebiotics and synbiotics, as well as fecal microbiota transplantation and the use of IL-22, to date only in animal models, as a possible future drug. Current evidence has shown the involvement of the gut microbiome in PCOS, seen how humanized mice receiving a fecal transplant from women with PCOS develop ovarian dysfunction, immune changes and insulin resistance and how it is capable of disrupting the secondary bile acid biosynthesis. A future therapeutic approach for PCOS may involve the human administration of IL-22 and bile acid glycodeoxycholic acid.


Sign in / Sign up

Export Citation Format

Share Document