Benign Spindle Cell Tumor Research Articles

Mammary myofibroblastoma of the male breast: a case report and literature review.

Mammary myofibroblastoma (MFB) is a rare benign spindle cell tumour predominantly affecting males, but also observed in postmenopausal females. Its diagnosis remains challenging owing to overlapping histological features with malignant lesions and limited tissue sampling in core biopsies. We present a case of incidentally discovered mammary MFB in a 63-year-old man and review its clinical, radiological and histopathological characteristics. The patient, who had a history of distal pancreatectomy and splenectomy, presented with an incidental left anterior chest wall nodule discovered on computed tomography scan. Clinical examination revealed a benign left retroareolar lump, confirmed by breast ultrasound and mammography. Ultrasound-guided core biopsy demonstrated characteristic spindle cells, prompting immunohistochemical staining confirming the diagnosis of MFB. The lesion was surgically excised with clear margins. Mammary MFB is commonly seen in postmenopausal women and older men, presenting as painless, mobile breast lumps. Imaging findings are nonspecific, resembling fibroadenomas or fat necrosis. Histologically, MFB lacks mammary ducts or lobules and displays characteristic spindle cells with collagenous stroma. Immunohistochemistry aids in differentiating it from other spindle cell tumours. Surgical excision is curative, with no reported cases with recurrence. Mammary MFB should be considered in the differential diagnosis of breast masses in males and postmenopausal women. Despite the challenges in diagnosis, its benign nature and favourable prognosis warrant timely recognition and appropriate management through surgical excision. Further research is needed to establish clear management guidelines and explore its underlying pathogenesis.

Role of Fine Needle Aspiration Cytology in Paratesticular Neoplasms-A Case Reports-Based Systematic Review.

The cytology features of neoplastic paratesticular lesions are mostly documented as case reports. Thus, we conducted a case report-based literature review to identify the characteristics of paratesticular neoplasms and tried to determine the significance of FNAC in these tumors. The studies were searched using PubMed and Scopus. The quality assessment was done using the JBI critical appraisal checklist for case reports. The articles that received an overall rating of "Include" underwent data extraction. The data were extracted from the articles and analyzed. We included 34 case reports from 33 published articles. The mean age of patients was 44.74 (13-85) years, and the most common clinical manifestation was scrotal swelling (70.59%, 24/34). Lesions were commonly observed on the right side (65.63%, 21/32), and the most common tumor site was epididymis (35.29%, 12/34). A total of 38.24% (13/34) cases were nonmalignant, and 61.76% (21/34) were malignant on the cytological diagnosis. The adenomatoid tumor (53.85, 7/13) was the most common nonmalignant tumor. Other entities were schwannoma, benign spindle cell neoplasm with atypia, nerve sheath tumor, lipoma, undifferentiated pleomorphic sarcoma, rhabdomyosarcoma, epithelioid sarcoma, liposarcoma, malignant rhabdoid tumor, pigmented neuroectodermal tumor of infancy and mesothelioma. Considering the malignant and nonmalignant groups, the sensitivity, specificity, and diagnostic accuracy of cytology were 95.00%, 84.62%, and 90.91%, respectively. Cytopathologists should be familiar with the cytological features of various malignant and nonmalignant paratesticular tumors to prevent unnecessarily invasive surgical management. The current systematic review emphasizes usefulness of FNAC in the preoperative cytological identification of paratesticular tumors.

Vaginal Cellular Angiofibroma: Report of a Rare Case with Literature Review

ABSTRACT Cellular angiofibroma (CA) is a relatively recently described, rare, benign soft tissue tumor that predominantly occurs in the vulvoperineal region. It was first described in 1997 as a mesenchymal-origin tumor composed of connective (fibrous) tissue and blood vessels. We report a case of vaginal CA in a 40-year-old woman who presented with pressure symptoms, incidentally diagnosed with a vaginal mass and clinically suspected as vaginal myoma. This case was diagnosed based on histomorphology and immunohistochemistry and underwent surgical treatment. This tumor was a diagnostic dilemma for other benign and aggressive spindle cell tumors in the same area, such as endometrial stromal nodule, spindle cell lipoma, solitary fibrous tumors, and mixed tumors of the vagina.

Spectrum of endoscopic gastric subepithelial lesions encountered on EUS-FNA: A single center experience.

Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) is a minimally invasive and reliable non-surgical technique for the diagnosis of gastrointestinal lesions. The present study aimed to evaluate the spectrum of lesions encountered in the gastric subepithelium on EUS-FNA at a tertiary care center. Archival data of all patients undergoing EUS-FNA for gastric submucosal lesions over a period of 5 years was retrieved. Patient demographics, clinical presentation, and EUS findings were recorded along with the FNA results. A total of 78 EUS-FNA samples were analyzed. Material was adequate in 62 cases (79.48%) and inadequate in 16 cases (12.82%) patients due to scant cellularity. Of the adequate samples, 34 (43.5%) were reported as neoplastic while 20 (25.64%) were non-neoplastic, and 8 (10.25%) were reported as suspicious of a neoplasm. In the neoplastic category, the predominant diagnosis was of spindle cell neoplasm comprising gastrointestinal stromal tumor (13), benign neural tumor (03), leiomyoma (02), and spindle cell tumors (03). The latter could not be categorized further due to a lack of IHC material. The next common diagnosis was adenocarcinoma (06) followed by neuroendocrine tumor (02) and poorly differentiated carcinoma (01). The non-neoplastic lesions included non-specific pathology (15), inflammatory lesions (08), and one case each of tuberculosis, pancreatic rest, and Brunner gland hamartoma. Cell blocks for ancillary testing were available in 54 cases (65.23%) and follow-up was available in 42 cases (53.84%). EUS-FNA is a good modality for the diagnosis of gastric submucosal lesions with a high diagnostic yield.

Gastric Perineurioma: A Rare Entity with Molecular Analysis and Literature Review.

Perineuriomas of the gastrointestinal tract are benign neoplasms that commonly develop in the distal colon and are identified during screening colonoscopy; however, perineuriomas of the stomach are exceedingly rare and less frequently identified. Differentiating gastric perineuriomas from other more serious gastric neoplasms is critical to avoid unnecessarily aggressive treatments. Thus far, only six patients with gastric perineurioma have been described, and the molecular characterization of this entity is still lacking. We report a 52-year-old woman who presented with abdominal pain and gastric acid reflux and was found to have a 1.5 cm subepithelial gastric neoplasm composed of bland spindle cells displacing the gastric glands with no cytologic atypia or mitotic activity, suggesting a benign spindle cell neoplasm. Immunohistochemical analysis showed reactivity for perineurial markers glucose transporter-1 and epithelial membrane antigen, consistent with benign gastric perineurioma. DNA extracted from the tissue was used for a capture-based target sequence enrichment panel followed by Illumina next-generation sequencing and targeted bioinformatic analysis for oncogenic alterations within defined disease-associated target regions. No sequence variants in the BRAF gene were identified. This rare case of gastric perineurioma helps solidify our understanding of how to discern various types of gastric neoplasms through traditional laboratory analysis alongside genetic sequencing approaches. Although extremely rare, gastric perineurioma should be kept in the differential diagnosis when assessing spindle cell gastric tumors to avoid unnecessary therapies, and physicians should understand the molecular characteristics of benign versus malignant tumors.

CASE REPORT- PRE AURICULAR SWELLING PRESENTED AS DERMATOFIBROSARCOMA

Background: Dermatofibrosarcoma protuberans a rare soft tissue tumour that involves the dermis, subcutaneous fat, and in rare cases muscle and fascia. Case Report: 34 yr old female patient presented with history of swelling in front of right ear since 2 years, associated with pain since 2 weeks , HRUSG neck reported as Neoplastic pathology and FNAC reported as benign spindle cell tumor. Patient underwent surgical excision of tumour. Frozen section sent was reported as synovial cell sarcoma or schwannoma. Hence on table superficial parotidectomy done. HPE showed Dermatofibrosarcoma protuberans . Conclusion: Dermatofibrosarcoma protuberans is considered intermediate-grade malignancy with low likelihood of metastasis hence treatment is complete surgical excision. As chance of recurrence is high regular follow up required.

Benign Spindel Cell Neoplasam: A Central Myofibroma of Maxilla in Male Child of Seven Years of Age: A Case Report

Myofibroma is a rare benign spindle cell neoplasm which occurs predominantly in infants and young children. It can occur as a solitary mass or as a multicentric lesion, consisting of myofibroblasts. In oral cavity, the sites most commonly involved are the mandible, tongue, lips and buccal mucosa however, it rarely involves the maxilla. It has aggressive clinical presentation mimicking malignancies. We report a case of a male child seven years of age presented with intraoral swelling on the left palatal aspect of the maxilla that extended from deciduous upper left second molar to the distal of upper permanent left first molar causing the mobility of these teeth. Radiologically, the lesion was osteolytic causing marked resorption of alveolar bone and displacement of permanent maxillary left second molar. A differential diagnosis of peripheral giant cell granuloma, central giant cell granuloma, fibroma, aggressive fibromatosis, nodular fasciitis, peripheral ossifying fibroma and spindle cell neoplasm was made. After the excluding malignant and vascular involvement, the surgical excision of the lesion was planned and was carried out under general anesthesia. Histologically, benign proliferation of spindle cells was seen giving the diagnosis of benign spindle cell neoplasm. Reports also showed positive immunoreactivity with vimentin and αSMA while non-responsive to desmin, validates the diagnosis of myofibroma. KEYWORDS: spindle cell neoplasm; myofibroma; maxilla

An Unusual Presentation of Spindle Cell Tumor on Palate

Spindle cell tumors are rarely reported in the oral cavity. Since the subtypes of these lesions are very difficult to diagnose, immunohistochemistry is a useful diagnostic tool. Here, we report a case of benign spindle cell tumor on palate. A 49 year old male presented with irregular mass of approximately 2 × 2 cm on maxillary right posterior region of palate since 2 months. Excisional biopsy and immunohistochemistrydetermine it as benign spindle cell tumor.

Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3months. The duration of recurrence is between 3 and 24months. The size of the tumor ranges from 7.2 to 10.5cm. The wide local excision margins range from 2 to 4cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.

Inflammatory Myofibroblastic Tumor of the Bladder in an 8-Year-Old Girl: About a Case with Review of the Literature

Introduction: The inflammatory myofibroblastic tumor is a rare tumor. It most often occurs in the bladder in the genitourinary sphere. This benign and aggressive spindle cell tumor affects children and frequently girls. The objective of this work is to describe from a clinical case, the clinical, radiological, and histological characteristics of an inflammatory myofibroblastic tumor in an 8 years old girl. Case Presentation: An 8-year-old girl was followed for an inflammatory myofibroblastic tumor of the bladder revealed by micturition burns complicated by hematuria. She underwent an ultrasound and radiological workup demonstrating an anterior bladder parietal mass reaching the rectus abdomen is muscle. Subsequently, an excisional biopsy was performed and histological and immunohistochemical examination revealed an inflammatory myofibroblastic tumor. Discussion: Inflammatory myofibroblastic tumors of the bladder are benign tumors with aggressive features usually revealed by voiding symptoms, pelvic pain, and pelvic mass. Treatment is based on Trans urethral resection of the bladder. Anatomical pathology examination shows spindle-shaped myofibroblast and fibroblast cells, a collagenous or myxoid matrix, and inflammatory cells composed of plasma cells, lymphocytes, and eosinophils. The diagnosis is confirmed by immunohistochemistry with an expression of CAM5.2, desmin, aSMA, and vimentin and especially of Anaplasic Lymphoma Kinase (ALK). Conclusion: Rare tumor process, whose malignant potential remains unknown. Management is by conservative surgery of the bladder.

Intrapulmonary desmoplastic mesothelioma in a young adult – Case report

Introduction: Desmoplastic mesothelioma typically represents an extremely rare histological subtype of sarcomatoid malignant mesothelioma that usually presents with a diffuse pattern of growth. A marked increase in mesothelioma is predicted in developing countries, where asbestos usage is increasing exponentially. Only a few case series have been reported in the Indian literature where mesotheliomas have been analysed on routine histology and IHC. Case Presentation: We are reporting a case of a 28 years old man came with complaints of gradually progressive central non radiating chest pain for 3months with unremarkable physical findings. CT thorax revealed well defined homogenous slightly lobulated soft tissue attenuating mass lesion in anterior epicardial fat pad, biopsy reported benign spindle cell tumour possibly neural origin. Interventions: Patient underwent thoracotomy and tumour excision. Post-operative histopathological examination showed desmoplastic mesothelioma. The final diagnosis of intrapulmonary desmoplastic mesothelioma was confirmed by pathological and immunohistochemical examination. The patient underwent 6 cycles of PEM/CARBO adjuvant chemotherapy followed by adjuvant radiation of 50.4 Gy by VMAT Technique. Conclusion: Desmoplastic mesothelioma is a rare pathology and rarely seen in young patients. Desmoplastic mesothelioma continues to have a poor prognosis due to its highly malignant, aggressive, and refractory nature to local treatment which necessitates the early intervention to improve the survival.

Myofibroblastoma of the Breast: Diagnosis, Pathology, and Management.

Myofibroblastomas are rare benign spindle cell tumors that occur within both male and female breasts. They are composed of fibroblasts and myofibroblasts and are not associated with malignant potential. On mammographic and sonographic imaging, these tumors may present as oval circumscribed masses that overlap with the appearance of many benign entities, including fibroadenomas. Occasionally, the tumors may demonstrate interval growth or mimic imaging features of malignancy and require biopsy. Correct pathologic diagnosis is important because many morphologic variants exist, which complicates pathologic interpretation. The purpose of this article is to review the range of imaging manifestations and histopathological findings and to discuss current management.

Abstract #1141458: Undifferentiated Malignant Neoplasm of the Thyroid with Spindle Cells in a Patient with a Rapidly Enlarging Neck Mass and Pulmonary Nodules

Spindle cell neoplasms (SCNs) of the thyroid are rare, and can be classified into benign and malignant lesions. Benign SCNs are described as isolated tumors within the thyroid parenchyma that are composed of spindle-shaped cells. Medullary and anaplastic carcinoma of the thyroid are among the most well-established malignancies of the thyroid with spindle cell features.

Spindle cell tumors of the pleura and the peritoneum: pathologic diagnosis and updates.

A diverse group of both benign and malignant spindle cell tumors can involve the pleura or the peritoneum. Due to their rarity and overlapping morphologic features, these tumors can pose considerable diagnostic difficulty in surgical pathology. As these tumors differ in their prognosis and clinical management, their correct pathologic diagnosis is critical. In addition to histologic assessment, select immunohistochemical and molecular tools can aid the distinction among these tumors. In this review, we consider some of the major histologic differential diagnosis of spindle cell tumors involving these serosal membranes. This list of tumors includes solitary fibrous tumor, inflammatory myofibroblastic tumor, desmoid fibromatosis, synovial sarcoma, sarcomatoid carcinoma, spindle cell melanoma, dedifferentiated liposarcoma, epithelioid hemangioendothelioma, and sarcomatoid mesothelioma. We describe their salient clinicopathologic and genetic findings, with a review on some of the recent discoveries on their molecular pathogenesis.

English

Usually, a solitary encapsulated benign peripheral nerve sheath tumour called the schwannoma is seen in age groups of twenty to sixty years with mean age of fortyfive years. They are commonly located on the flexor surfaces of the extremities, posterior spinal roots, neck, retroperitoneum, mediastinum, and cerebellopontine angle. Schwannomas of the pleural surface of the lungs are usually rare and are benign, asymptomatic, and slow growing lesions that arise from the autonomic nerve fibre sheaths and their occurrence is more common in adults with a male predominance.1 Schwannomas may occur in patients with underlying genetic disorders like neurofibromatosis 2, schwannomatosis or Carney complex. Neurofibroma and other benign spindle cell tumours such as leiomyosarcoma, leiomyoma and calcifying aponeurotic fibroma which may also show palisading of the nuclei are the histopathological differential diagnoses.1 Schwannoma, which is a benign peripheral nerve sheath tumour is also called neurilemmoma. 1 % - 2% of the neoplasms of the thoracic cavity are the pleural schwannomas which are extremely rare. Here is a case report of a 50-year-old female diagnosed with primary benign pleural schwannoma which was discovered incidentally in a chest computed tomography. A video assisted thoracoscopic wide local excision of the right chest wall was done on the patient which showed histopathological and immunohistochemical features of schwannoma.

INNV-24. METASTATIC HEMANGIOPERICYTOMA TO THE LIVER: DEFINING THE IMPORTANCE OF NAB-STAT2 FUSION

Abstract BACKGROUND Meningeal hemangiopericytoma (HPC) is a rare and aggressive vascular tumor which typically occurs in younger adults and has a tendency for local recurrence with delayed metastases outside the CNS. HPC is considered a variant of solitary fibrous tumor (SFT), and is associated with the presence of the fusion oncoprotein NAB2-STAT6. This fusion results in over-expression of the EGR1 gene and results in tumorigenesis. CASE REPORT We present a case of a 49 y/o female with a remote history of left occipital lobe HPC, initially treated with resection and radiotherapy in 2007, who presented with symptoms of right upper quadrant abdominal pain. She was found to have a hypervascular 7 cm mass in the right upper lobe of the liver on magnetic resonance imaging (MRI) studies and subsequently underwent hepatectomy for removal of the mass. Histopathological findings revealed metastatic HPC with features consistent with her primary meningeal tumor that was removed over 13 years agoearlier. Presence of the NAB2-STAT6 fusion was confirmed by targeted genomic analysis. MRI revealed a small focus of nodular enhancement abutting the confluence of the superior sagittal sinus and transverse sinus. This was considered her source of disease and possible dissemination. She was treated with stereotactic radiosurgery to target the lesion. DISCUSSION Given the rarity and often remote medical history of the primary intracranial HPC, this report suggests that 1) long term follow up of HPC is essential and 2) detection of the NAB2-STAT6 fusion can identify and confirm recurrent HPC at metastatic sites. In regards to the latter, distinguishing HPCs from other more benign spindle cell tumors that may mimic SFT (such as fibroblastic meningiomas) is also of therapeutic significance as HPCs typically require adjuvant treatment, (i.e. radiotherapy) as opposed to surgical resection alone.

Not a Huge but Two Tumors

A 73-year-old man presented to the thoracic surgery clinic with progressive difficulty breathing for about 3 months. He had a benign spindle cell tumor (about 3 centimeter in diameter) in right lung upper lobe, which had been resected by thoracotomy surgery 15 years earlier. The physical examination showed weakened right respiratory sounds. Computed tomography of the chest revealed a huge mass closed to the pulmonary vessels and bronchus, which measured more than 13 cm in greatest dimension (Panel A). Thoracotomy surgery was performed for the tumor resection again. Two separated tumors, instead of one huge tumor, originated from visceral pleura were found with well encapsulated (Panel B). And the histopathological analysis revealed solitary fibrous tumor. The immunohistochemical analysis was positive for Desmin-protein and CD34. Just followingup was initiated after surgery, and the patient was doing well after 3 months.

The first reported case of proliferative fasciitis in the oral cavity

<h3>Background</h3> Proliferative fasciitis is a benign and reactive lesion involving fibroblasts in the subcutaneous tissues and deep fascia, with a rare occurrence in the head and neck region. It is considered a variant of nodular fasciitis, which could involve trauma as an etiology. Proliferative fasciitis mostly occurs in adults, but cases in children have been reported. Clinically, it can present as an aggressive lesion with pain or no symptoms, mimicking sarcomas. <h3>Case Summary</h3> We report a case of a 53-year-old woman who presented to our clinic for an evaluation of a reddish lesion of the right mandibular gingiva around the molar areas with a relatively rapid onset. The patient had prediabetes with moderate oral hygiene and no other significant medical history.Intraoral examination revealed a poorly circumscribed gingival lesion on the posterior, <1 cm in diameter with no pain on palpation, and soft to firm in texture with no bleeding. A biopsy of the lesion was performed for histologic examination, and the microscopic differential diagnosis included benign and malignant spindle cell tumors; thus, immunohistochemistry was performed for more accurate diagnosis, and a specimen was sent to the pathology lab at Ohio (Central Ohio Skin and Cancer). The immunohistochemical findings were positive for vimentin and smooth muscle actin and negative for CD34, S100, and pan-cytokeratin. The lesion was diagnosed as proliferative fasciitis on the basis of histologic and immunohistochemical features. The feature that differentiates proliferative from nodular fasciitis is the basophilic component that closely resembles ganglion cells without Nissl substance. The treatment rendered was conservative surgical excision with 1-year follow-up, and no recurrence was observed. <h3>Conclusions</h3> Because proliferative fasciitis has not been reported in the oral cavity, to our knowledge, and because it poses a diagnostic challenge and can mimic malignancies, it is essential to know the salient diagnostic features to avoid aggressive treatment in patients presenting with such lesions in the oral cavity.

Radiological, histopathological, and immunohistochemical characterization of solitary fibrous tumor of the lacrimal sac: A rare case report

Solitary fibrous tumor (SFT) is a benign spindle cell neoplasm of mesenchymal fibroblast cells. It can appear in various locations, but SFT of the lacrimal sac is rare. Due to their variable clinical, histological, and radiological presentation, it can be easily misdiagnosed. We report a rare case of SFT of the lacrimal sac with its radiological, histological, and immunochemical characterization.

A Case of Solitary Fibrous Tumor in the External Auditory Canal

Solitary fibrous tumor (SFT) is a rare benign spindle-cell neoplasm, most often found in the pleura. Recently, it has been reported to appear throughout the body but rarely in head and neck region. Tumors in the external auditory canal, in particular, have been very rarely reported. We experienced a case of a 55-year-old male with a 6-month history of progressive swelling from the orifice of left external auditory canal. The tumor was round, well circumscribed, almost completely obstructing the left external auditory canal. On the enhanced temporal bone CT and enhanced paranasal sinus MRI, a 2.0×1.7 cm sized mass was observed at the left external auditory canal. He was treated with surgical excision under general anesthesia. Histopathologic examination confirmed the diagnosis of SFT. This case report presents a rare case of a SFT arising in the external auditory canal.