Anlotinib in combination with pembrolizumab for low-grade myofibroblastic sarcoma of the pancreas: A case report

BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we report a case involving the discovery of a pancreatic mass detected during a routine physical examination.Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas.Following surgical intervention,the patient experienced recurrence and metastasis.Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases.After communicating with the patients and their families,informed consent was obtained for the treatment of anlotinib combined with pembrolizumab.Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab.CONCLUSION Based on the comprehensive literature review,our report aimed to provide evidence for a better understanding of the disease characteristics,diagnostic criteria,imaging findings,and identification of LGMS.And explore novel treatment strategies for this disease.

Low-grade myofibroblastic sarcoma of the palate

Low-grade myofibroblastic sarcoma （LGMS） is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.

Low-grade myofibroblastic sarcoma of the liver misdiagnosed as cystadenoma:A case report

BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare malignant tumor.It has no specific clinical manifestations and commonly occurs in the head and neck,extremities and other body parts,with the liver not as its predisposing site.CASE SUMMARY We report a case report of a 58-year-old man with right upper abdominal pain for 11 d.Contrast-enhanced computed tomography(CECT),CE magnetic resonance imaging and CE ultrasound(US)all showed a cystic-solid mass in the right liver.As the initial clinical diagnosis was hepatic cystadenoma,surgical resection was performed,and the postoperative pathology indicated hepatic LGMS.The 3-mo follow-up showed favorable recovery of the patient.However,at 7-mo follow-up,two-dimensional US and CECT showed a suspected metastatic lesion in the rightmiddle abdomen.CONCLUSION Hepatic MS is particularly rare and easily misdiagnosed,more cases will contribute to the understanding and the diagnosis accuracy.

Low-grade myofibrosarcoma of the maxillary sinus:Two case reports

BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.

Relapse of a cardiac tumor from myxoma to low-grade malignant myofibroblastic sarcoma: malignant transformation or metachronous lesion?

A 47-year-old woman, who had undergone cardiac myxoma removal surgery six years ago, was readmitted due to a 2-week history of chest distress, and dyspnea with the change of body position. Both the examinations and the surgery confirmed a cardiac tumor relapse. Histology demonstrated that the mass resected in surgery had a pathologic change from myxoma to a low-grade malignant myofibroblastic sarcoma. The patient received heart transplantation 2 years later due to tumor recurrence, and finally died of cerebral hemorrhage.

High-grade myofibroblastic sarcoma in the liver: A case report

Only two cases of myofibroblastic sarcoma in the liver have been reported in the literature. Here, we report the case of a male patient with high-grade myofibroblastic sarcoma mimicking echinococcosis in the liver. The 25-year-old male patient complained of right upper quadrant swelling pain for one week and was initially diagnosed with echinococcosis. He was then scheduled for an exploratory laparotomy. During the operation, a huge mass exceeding 16 cm in diameter was found to occupy nearly the entire right trisegment of the liver, with a clear boundary and a round shape, and the mass was resected by right hepatic trisegmentectomy. Immunohistochemical staining revealed that the tumor tissue was positive for desmin, α-smooth muscle actin, CD56, and vimentin and negative for ALK-1, myogenin, calponin, β-catenin, S100, and glypican-3, with a Ki-67(MIB-1) index of approximately 20%. Based on the histological manifestations and immunohistochemical staining, a diagnosis of myofibroblastic sarcoma was established. The postoperative recovery was uneventful. There was no evidence of recurrence or metastasis through the last follow-up, 6 mo after surgery, despite a lack of postoperative chemotherapy or radiotherapy. To the best of our knowledge, the present case is the first reported case of high-grade myofibroblastic sarcoma in the liver, and it is also the first reported case in a male patient.

Metastatic low-grade endometrial stromal sarcoma with variable morphologies in the ovaries and mesentery: A case report

BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)classically exhibits a proliferative morphology.However,morphological variation of extrauterine tumors presents a diagnostic challenge.CASE SUMMARY We report the case of a 76-year-old female patient with extensive extrauterine and abdominal neoplastic lesions.Computed tomography showed massive pleural and ascitic fluid,and there was an increase in serum cancer antigen 125.She underwent bilateral adnexectomy and tumor resection.The right ovary had been replaced by a multinodular mass that was 8.5 cm×4.5 cm×3.5 cm in size.In addition,there was a 24 cm×15 cm×13 cm mesenteric mass,which was also multinodular,with local invasion of the intestinal serosa and underlying muscle.Under the microscope,the tumors in different places exhibited two different patterns,thus presenting great challenges to diagnosis and treatment.Thorough pathological assessment eliminated all differential diagnoses in favor of metastatic LGESS derived from a 20-year-old primary tumor initially misdiagnosed as leiomyosarcoma.CONCLUSION LGESS morphology varies according to tumor location.Accurate diagnosis is critical for appropriate treatment and improved prognosis and patient care.

Low Grade Myofibroblastic Sarcoma of the Tongue: A Case Report in an Infant

A case of a 12-month-old male infant, who presented a painless swelling of the right anterolateral edge of the mobile tongue, is reported. The treatment consisted of a partial glossectomy with total surgical excision of the tumor. The diagnosis of low grade myofibroblastic sarcoma on the histological arguments (tumor architecture, cytology, mitotic index) and immunohistochemistry was retained.

牙龈低度恶性肌纤维母细胞肉瘤伴颌骨破坏1例报告并文献复习

目的:探讨低度恶性肌纤维母细胞肉瘤(low-grade myofibroblastic sarcoma,LGMS)的临床和病理特征、鉴别诊断、治疗与预后,并通过报道发生在儿童下颌牙龈的1例LGMS,以提高临床医师对此疾病的认识。方法:对1例发生在右下颌牙龈伴颌骨破坏的LGMS儿童患者进行临床资料、治疗、病理分析,并对国内外相关LGMS进行分析和总结。结果:患者因右下颌牙龈一无痛性包块入院手术治疗,病理提示为牙龈瘤,术后2周复发,行二次扩大手术,术后病理提示为LGMS。结论:LGMS是一种罕见的来源于间叶组织的肿瘤,好发于口腔,无明显的临床特异性表现,需要通过组织病理检查进行确诊,同时应与炎性肌纤维母细胞瘤、平滑肌肉瘤等疾病相鉴别,临床上通常采用手术扩大切除,并应该长期随访观察。

儿童下颌骨低度恶性肌纤维母细胞肉瘤一例

低度恶性肌纤维母细胞肉瘤(low-grade myofibroblastic sarcoma,LGMS)是一种极其罕见的肌成纤维细胞恶性肿瘤,好发于头颈部,口腔内常见于舌部,发生于颌骨的病例较少见,目前仅有12例报道。LGMS常发生于成年男性,儿童少见。本文报道1例罕见的下颌骨LGMS儿童病例,患儿病变累及下颌骨,行手术切除,组织病理学结果显示有梭形细胞增生并向周边横纹肌侵袭性生长;免疫组化结果显示Vimentin、Calponin、SMA和Actin呈阳性表达,支持其肌纤维母细胞和平滑肌分化的特点,诊断为LGMS。本文通过相关文献复习对颌骨LGMS的免疫学特点、病理学特征、治疗和预后进行讨论,以丰富临床医生对该病的认识,提高诊断水平。

胸壁低度恶性肌纤维母细胞肉瘤一例

本文为回顾性研究,遵守《赫尔辛基宣言》,经过甘肃省妇幼保健院伦理委员会批准,免除受试者知情同意,批准文号:(2022)GSFY伦审[05]号。病例资料:患者女,48岁,2022年6月13日就诊于甘肃省妇幼保健院,自诉4月前无明显诱因出现左前胸壁针刺样疼痛,经期疼痛加重。

Myofibroblastic sarcomas:a clinicopathological study of 20 cases

Background Myofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations. Methods The paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student＇s t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas. Results Histologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform, tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophUic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin （18/20）, muscle specific actin （16/20）, fibronectin （20/20） and desmin （2/20）. Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease. Conclusions Myofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.

A nomogramfor predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis

Background:Low-grade endometrial stromal sarcoma(LG-ESS)is a rare tumor that lacks a prognostic prediction model.Our study aimed to develop a nomogram to predict overall survival of LG-ESS patients.Methods:A total of 1172 patients confirmed to have LG-ESS between 1988 and 2015 were selected from the Surveillance,Epidemiology and End Results(SEER)database.They were further divided into a training cohort and a validation cohort.The Akaike information criterion was used to select variables for the nomogram.The discrimination and calibration of the nomogram were evaluated using concordance index(C-index),area under time-dependent receiver operating characteristic curve(time-dependent AUC),and calibration plots.The net benefits of the nomogram at different threshold probabilities were quantified and compared with those of the International Federation of Gynecology and Obstetrics(FIGO)criteria-based tumor staging using decision curve analysis(DCA).Net reclassification index(NRI)and integrated discrimination improvement(IDI)were also used to compare the nomogram’s clinical utilitywith that of the FIGO criteria-based tumor staging.The risk stratifications of the nomogram and the FIGO criteria-based tumor staging were compared.Results:Seven variables were selected to establish the nomogram for LG-ESS.The C-index(0.814 for the training cohort and 0.837 for the validation cohort)and the time-dependent AUC(>0.7)indicated satisfactory discriminative ability of the nomogram.The calibration plots showed favorable consistency between the prediction of the nomogram and actual observations in both the training and validation cohorts.The NRI values(training cohort:0.271 for 5-year and 0.433 for 10-year OS prediction;validation cohort:0.310 for 5-year and 0.383 for 10-year OS prediction)and IDI(training cohort:0.146 for 5-year and 0.185 for 10-year OS prediction;validation cohort:0.177 for 5-year and 0.191 for 10-year OS prediction)indicated that the established nomogram performed significantly better than the FIGO criteria-based tumor staging alone(P<0.05).Furthermore,DCA showed that the nomogram was clinically useful and had better discriminative ability to recognize patients at high risk than the FIGO criteria-based tumor staging.Conclusions:A prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG-ESS patients.

乳腺低度恶性肌纤维母细胞肉瘤1例

低度恶性肌纤维母细胞肉瘤(low-grade myofibroblastic sarcoma,LGMS)是一种由肌纤维母细胞组成或者起源于肌纤维母细胞的间叶组织肿瘤。在局部麻醉下,对1例原发于乳腺上的LGMS的患者行右侧乳腺肿物切除术,术后病理组织学形态表现为瘤组织由梭形细胞构成,呈漩涡状或束状排列,背景基质黏液样,核分裂象易见;免疫组织化学:波形蛋白、平滑肌肌动蛋白、CD34(+);对LGMS的临床病理特征、影像学特征、诊断与鉴别要点进行分析,以提高临床医师的认识。

低度恶性肌纤维母细胞性肉瘤

目的 探讨低度恶性肌纤维母细胞性肉瘤的临床病理学特征、免疫学表型和超微结构特点。方法 对 1例发生于左上颌窦的低度恶性肌纤维母细胞性肉瘤进行临床资料复习、光镜观察、免疫组化标记和电镜检测。结果 患者因“左上颌窦囊肿” 2次行切除活检 ,病理诊断分别为“纤维组织增生”和“鳞状细胞癌Ⅰ级”。 4个月后因左上颌骨隐痛行左上颌骨部分切除术 ,术后病理检查未发现肿瘤组织。 5个月后左上后磨牙区肿块复发 ,再行左上颌、颊、颈联合根治术 ,诊断为“侵袭性纤维瘤病”。 9个月后左侧颧部出现包块 ,术后病理为“左上颌纤维肉瘤 ,部分伴平滑肌分化”。复片显示 ,第 1次活检标本中的“纤维组织增生”实际上是梭形细胞肿瘤组织 ,而第 2次活检中的“鳞状细胞癌Ⅰ级”实为鳞状上皮假上皮瘤样增生。第 3次术后标本中肿瘤组织不明显 ,而第 4次术后标本则由成束的梭形细胞组成 ,弥漫浸润至邻近的软组织内 ,类似侵袭性纤维瘤病 ,但部分区域内可见鱼骨样排列结构 ,类似低度恶性纤维肉瘤。第 5次术后标本中瘤细胞显示轻～中度的异型性 ,可见核分裂象(2个 / 10HPF) ,并弥漫浸润横纹肌组织 ,在部分区域内 ,瘤细胞穿插在肌束之间形成类似增生性肌炎中的棋盘样结构 ,另一些区域则在形态上类似经典的纤维肉瘤。

腹腔上皮样炎性肌纤维母细胞肉瘤2例临床病理观察

目的探讨腹腔上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma,EIMS)的临床病理学特征、诊断和鉴别诊断、分子遗传学进展、治疗及预后。方法回顾性分析2例EIMS的临床表现、大体及组织学形态、免疫表型,并复习相关文献。结果例1,男性,15岁;例2,女性,21岁,均因腹部疼痛不适入院,入院后行肿物切除术。肿瘤由致密区和富于黏液的疏松区构成,肿瘤细胞圆形、上皮样,核圆,核仁明显,可见病理性核分裂及瘤巨细胞,伴肿瘤性坏死,背景中可见丰富的炎细胞,以中性粒细胞为主体伴少量淋巴细胞和浆细胞。免疫表型:肿瘤细胞表达ALK、vimentin、desmin和CK(AE1/AE3)(灶阳性),不表达Calretinin、CD30、CD31、CD33、SMA、HHF35、Myogenin、S-100、HMB-45、CD20、CD79a、CD3、CD5、CD45和CD68。FISH检测显示2例均有ALK基因相关易位。结论腹腔EIMS罕见,作为炎性肌纤维母细胞肿瘤的独立亚型之一,其正确诊断尤为重要。ALK抑制剂或使ALK阳性的EIMS患者获益。

肺上皮样炎性肌纤维母细胞肉瘤临床病理观察

目的提高对肺上皮样炎性肌纤维母细胞肉瘤的认识。方法报道1例罕见的肺上皮样炎性肌纤维母细胞肉瘤,进行组织形态学观察及免疫组化检查,并结合相关文献进行讨论。结果患者青年男性,21岁。体检发现左下肺肿物,行"左下肺叶切除并肺门及纵隔淋巴结清扫术",术后2个月出现双下肢麻木、右下肢活动障碍及排尿困难。PET-CT示全身弥漫性骨及骨髓内肿瘤转移。肺部肿物镜下呈胖梭形的纤维母细胞与肌纤维母细胞混杂排列,有黏液样基质及多量炎细胞浸润。局灶细胞呈上皮样,胞质丰富嗜双色性,胞核常偏位,泡状核,可见单个明显核仁,并见大量神经节样细胞。细胞异型性明显,核分裂>4个/10HPF,并有较多多核巨细胞及灶性坏死。转移灶均为异型的上皮样细胞及神经节样细胞伴明显的黏液样基质,核分裂>10个/10HPF,病理性核分裂多见。免疫组化示梭形细胞区及上皮样细胞区瘤细胞vimentin、ALK、SMA及EMA均(+),部分瘤细胞p53(+),上皮样区Ki-67增殖指数40%。患者出院20天后死亡。结论肺上皮样炎性肌纤维母细胞肉瘤罕见,诊断时需与未分化大细胞癌、ALK阳性的间变性大细胞淋巴瘤及其他类型肉瘤鉴别。正确诊断对临床制定合适的治疗方案非常重要。

腹腔上皮样炎性肌纤维母细胞肉瘤临床病理观察

目的 探讨上皮样炎性肌纤维母细胞肉瘤（EIMT）的临床病理特点、诊断与鉴别诊断要点.方法 总结1例腹膜后EIMT的临床、病理形态及免疫表型特点,并复习相关文献.结果 患者女性,47岁.左上腹疼痛半年,疼痛加重伴腹胀1个月.CT示左上腹腔占位.术中见肿瘤位于腹膜后降结肠脾曲肠系膜内,与脾、胰尾、膈肌关系密切.大体见肿瘤呈结节状,位于肠系膜内,侵及肠壁并与周围脂肪、脾、胰及横纹肌组织粘连.镜下见肿瘤细胞排列成片状或结节状,间质明显黏液样变性及中性粒细胞、淋巴细胞、浆细胞和嗜酸性粒细胞浸润.瘤细胞圆形、多角或梭形,胞质丰富,呈上皮样,染色质空泡状,核仁大而明显,核分裂象易见.免疫组化示肿瘤细胞vimentin、desmin、ALK和AAT弥漫强（＋）,部分瘤细胞EMA、CD30、WT-1、D2-40、CD99和p53（＋）,Ki-67阳性指数10％ - 20％.荧光原位杂交示有ALK基因重排.患者术后2个月肿瘤局部复发,出现肝、肺多发转移,于术后4个月死亡.结论 上皮样炎性肌纤维母细胞肉瘤是炎性肌纤维母细胞瘤罕见的恶性上皮样亚型;诊断依据部位、典型的形态学和免疫组化特点,特别是核膜和核周ALK（＋）.治疗以手术切除为首选,术后放、化疗和靶向治疗为辅助治疗.此类肿瘤恶性度高,预后差.

低度恶性肌纤维母细胞肉瘤3例临床病理观察

目的探讨低度恶性肌纤维母细胞肉瘤（LGMS）的临床病理特征及免疫组化特点。方法对3例LGMS临床资料、病理学形态及免疫组化进行分析。结果镜下肿瘤组织由轻、中度异型的长梭形／胖梭形肿瘤细胞组成，核分裂象2—3个／10HPF。瘤细胞束状排列，浸润周边脂肪组织或横纹肌组织，似侵袭性纤维瘤病或形成类似增生性肌炎中的棋盘样结构；部分区域细胞密度增加，呈交织束状或鱼骨样排列。免疫组化：3例梭形肿瘤细胞均vimentin和SMA（＋），1例desmin（＋）；3例S-100、calponin、lysozyme、EMA和AE1／AE3均（-）。结论LGMS是肌纤维母细胞分化的低度恶性间叶源性肿瘤，易复发，亦可转移，明确诊断需组织形态学与免疫组化相结合。