Integrated Molecular and Histological Insights for Targeted Therapies in Mesenchymal Sinonasal Tract Tumors.

PURPOSE OF REVIEW: This review aims to provide a comprehensive overview of mesenchymal sinonasal tract tumors (STTs), a distinct subset of STTs. Despite their rarity, mesenchymal STTs represent a unique clinical challenge, characterized by their rarity, often slow progression, and frequently subtle or overlooked symptoms. The complex anatomy of the sinonasal area, which includes critical structures such as the orbit, brain, and cranial nerves, further complicates surgical treatment options. This underscores an urgent need for more advanced and specialized therapeutic approaches. RECENT FINDINGS: Advancements in molecular diagnostics, particularly in next-generation sequencing, have significantly enhanced our understanding of STTs. Consequently, the World Health Organization has updated its tumor classification to better reflect the distinct histological and molecular profiles of these tumors, as well as to categorize mesenchymal STTs with greater accuracy. The growing understanding of the molecular characteristics of mesenchymal STTs opens new possibilities for targeted therapeutic interventions, marking a significant shift in treatment paradigms. This review article concentrates on mesenchymal STTs, specifically addressing sinonasal tract angiofibroma, sinonasal glomangiopericytoma, biphenotypic sinonasal sarcoma, and skull base chordoma. These entities are marked by unique histopathological and molecular features, which challenge conventional treatment approaches and simultaneously open avenues for novel targeted therapies. Our discussion is geared towards delineating the molecular underpinnings of mesenchymal STTs, with the objective of enhancing therapeutic strategies and addressing the existing shortcomings in the management of these intricate tumors.

Unexpected concomitant pituitary adenoma and suprasellar meningioma: a case report and review of the literature.

We report a case of a 75-year-old patient with hypopituitarism, bitemporal visual field deficits and a parasellar mass on pituitary MRI. During surgery, suspicion was raised that a non-functioning pituitary adenoma was accompanied by an abutting diaphragm sellae meningioma, which was confirmed at pathological examination. In retrospect, the initial MRI suggested two separate tumours on the basis of differing densities but this distinction was not seen on the last preoperative MRI.

A rare combination of tumor-induced osteomalacia caused by sinonasal glomangiopericytoma and coexisting parathyroid adenoma: case report and literature review.

BACKGROUND: Tumor-induced osteomalacia (TIO) is a rare, acquired disease of renal phosphate wasting and disturbed vitamin D homeostasis as a result of the action of a phosphaturic protein - FGF-23, produced by a neoplasm. Although the clinical and biochemical profile of the syndrome is characteristic, it remains underreported and unrecognized by clinicians. Hyperparathyroidism is rarely associated with oncogenic osteomalacia, but it should be considered because of potentially life-threatening hypophosphatemia caused by both conditions. CASE PRESENTATION: We report a case of a 42-year-old woman admitted to the Department of Otolaryngology of the Military Institute of Medicine in Warsaw for the endoscopic resection of hormonally active glomangiopericytoma extending into the anterior skull base. She presented with a 5-year history of musculoskeletal pain and progressive weakness of the extremities which finally led her to become bedridden. After the excision of the tumor her symptoms and laboratory results gradually improved except increasing PTH serum levels. Further examination revealed a parathyroid proliferative tumor, which was surgically removed. The patient walked without aids at follow-up 16 months after the surgery. CONCLUSIONS: This case is unusual because of tumor-induced osteomalacia and parathyroid adenoma occurring concomitantly. Further investigations of FGF-23 and PTH interplay should be conducted to elucidate the pathogenesis of hyperparathyroidism and tumorigenesis in some cases of TIO. By presenting this case, we wanted to remind clinicians of a rare and misdiagnosed paraneoplastic syndrome and highlight the importance of monitoring PTH concentrations during the follow-up of patients with TIO.

Orbital glomangiopericytoma in a 5-year-old child.

A 5-year-old girl presented with a 6-month history of strabismus and painless left proptosis. Magnetic resonance imaging revealed a homogenously enhancing, anterior, superomedial, left orbital mass. Excisional biopsy established the diagnosis of orbital glomangiopericytoma. Glomangiopericytoma of soft tissue is a perivascular myoid neoplasm with hemangiocytoma-like vascular channels that has overlapping features between glomus tumor and myopericytoma. To the authors' knowledge, glomangiopericytoma of the orbit has not previously been reported.

Sinonasal Glomangiopericytoma: Review of Imaging Appearance and Clinical Management Update for a Rare Sinonasal Neoplasm.

Introduction: Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity or paranasal sinuses with low malignant potential. Initially deemed a hemangiopericytoma, in 2005 it was classified as a distinct entity by the World Health Organization (WHO). Case Presentation: A male patient in his early 60s presented with new-onset right arm and leg weakness/numbness, who was incidentally found to have a left ethmoid sinus mass with extension in the olfactory fossa. On CT and MRI, the mass enhanced with well-defined borders and eroded the bone, but without dural enhancement. The mass was surgically excised, and pathology confirmed the diagnosis of glomangiopericytoma by microscopic appearance and staining. Discussion: Glomangiopericytoma has less than 0.5% incidence of all neoplasms of the sinonasal cavity, making it rare. Most diagnosed patients are in their 6th or 7th decade of age, with a slight female predominance. Treatment is complete surgical excision, with excellent prognosis, although there is up to 17% local recurrence. Despite the non-specific appearance on CT and MRI, imaging can help provide differential diagnosis, tumor extent, size, and reassuring non-aggressive characteristics of the tumor prior to surgery. GPC tumors are relatively resistant to radiation and chemotherapy. Conclusion: It is important to recognize glomangiopericytoma in the differential of masses of the nasal cavities or paranasal sinuses, as they rarely warrant aggressive treatment beyond local excision. Each reported case of glomangiopericytoma helps to build guidance for imaging and treatment since GPC is rare and not well-represented in the medical literature.

Glomangiopericytoma: A rare tumour of sinonasal cavity.

The history of glomangiopericytoma began in 1924 when it was initially diagnosed by Stout and Murray. It is a rare tumour of the respiratory mucosa, with a prevalence of less than 0.5% among all sinonasal tumours. Literature shows female predominance among patients who develop glomangiopericytoma. So far, no accurate aetiology has been discovered, but there are certain risk factors, including trauma, use of corticosteroids and high blood pressure, which are believed to cause glomangiopericytoma. Patients usually present with a history of epistaxis or nasal blockage, though symptoms can get worse if the tumour is not resected timely and can lead to visual disturbance, chronic sinusitis and headache. It has reddish polypoidal appearance on examination. The best modality for the treatment of glomangiopericytoma is endoscopic surgical resection via trans-nasal approach. We present the case of a 70-year-old man, with nasal blockage and epistaxis who underwent endoscopic sinus surgery for this condition.

Pericytic Lesions: A Paradigm of Overlapping Features.

Although myopericytoma and glomangiopericytoma are considered the subtypes of the group myopericytomas (MPC), they differ morphologically. Both of the tumors arise from pericytic cells. Scalp MPC are infrequent, but extranasal glomangiopericytomas are extremely rare. Herein, we report a case of a 36-year-old female presenting with slowly growing scalp swelling. Histopathology showed a dermal tumor with features of myopericytoma as well as glomangiopericytoma within the same lesion. Immunohistochemistry and reticulin staining confirmed pericytic origin but could not differentiate between the two tumors. This case report adds to the rarity and morphologic heterogeneity of the group MPC.

Glomangiopericytoma Presenting as a Middle Ear Mass.

We describe an unusual case of glomangiopericytoma presenting as a mass filling the middle ear, enveloping the ossicles, and extending into the mastoid antrum without bony destruction. Management involved three surgeries and stereotactic radiosurgery, which achieved short-term local control with no evidence of disease on MRI imaging 12 months after radiation. Facial nerve function and hearing were preserved. This is the first report to our knowledge of a glomangiopericytoma presenting as a primary temporal bone lesion. Treatment with surgery and stereotactic radiosurgery for residual or recurrent disease is a reasonable approach to achieve local control and functional preservation. Laryngoscope, 134:1426-1430, 2024.

A case report of sinonasal glomangiopericytoma: An important reminder to always collect specimen.

OBJECTIVES: To present a case report of sinonasal glomangiopericytoma (GPC) in a female patient in her thirties and to highlight the importance of collecting pathology specimens even in routine sinus surgery cases. METHODS: A case report detailing the diagnosis of GPC in a female in her thirties, including her initial presentation, treatment, and follow-up, along with a brief review of the literature. RESULTS: Pathology of the collected specimen revealed sinonasal GPC along with chronic rhinosinusitis. Immunohistochemistry was positive for SMA, beta-catenin, and cyclin D1; and negative for STAT6, ERG, pankeratin, SOX10, and S100. CONCLUSION: This diagnosis expands the knowledge around the demographic profile of GPC patients. GPC should be included in the differential diagnosis of sinonasal masses, even in younger patients. The case highlights the importance of collecting the entire pathology specimen in all cases, even of ones that seem routine and benign.

Our Experience with Sinonasal Glomangiopericytoma in North India: A Case Series.

Glomangiopericytoma (GPC) is a rare benign sinonasal tumor originating from Zimmerman's Pericytes surrounding capillaries and accounting for less than 0.05% of all sinonasal tumors. Glomangiopericytoma has low malignant potential (5-10%) and is mostly diagnosed in the 6th or 7th decade of age with slight female preponderance. We presented here a case series of 5 patients with sinonasal GPC. This research was conducted at a tertiary healthcare centre in North India. In our case series, all the patients were evaluated and underwent endoscopic surgical resection. All patients underwent digital subtraction angiography (DSA) and preoperative embolization. The coblation technique used for haemostasis proved very effective and time-saving. All patients exhibited cytoplasmic SMA positivity (a marker of GPC) and CD34 negativity, while one patient exhibited a high Ki-67 index (> 10%), which is a predictor of aggressive tumor behavior. None of the patients showed any recurrence in follow-up. We recommend performing complete endoscopic surgical excision to prevent recurrence. The use of DSA, preoperative embolization, and intraoperative use of the coblation technique provides a cleaner surgical field and reduced operating time.

Sinonasal Glomangiopericytoma with Skull Base Involvement: A Diagnostic Dilemma.

Introduction: Glomangiopericytoma (GPC) is a rare type of neoplasm with hemangiopericytoma-like vasculature and perivascular hyalinization of capillary-sized veins. CD34 and S100 protein staining might be positive in a small percentage of GPC. Solitary fibrous tumors (SFTs) present clinically like GPC. However, challenges remain when differentiating GPC from SFT. Case Presentation: A 37-year-old male, smoker, presented with 3 years history of right-sided epistaxis and nasal congestion. He was also complaining of hyposmia but no headaches or visual complaints. On nasal endoscopy, he was found to have a right-sided nasal mass occupying the ethmoid cavity. Computed tomography showed a right sinonasal mass abutting the anterior skull base and magnetic resonance imaging demonstrated a hyperintense, enhancing mass within the right ethmoid cavity, superior septum, and anterior skull base. The patient underwent endoscopic resection with gross total resection and skull base reconstruction. Postoperative pathology initially was thought to be an SFT; however, subsequent STAT6 expression was negative, and therefore GPC diagnosis was confirmed. At the most recent follow-up (6 months), the patient remained free of local disease. Discussion: SFT, unlike GPC, stains strongly for CD34, in this case, due to strong CD34, the diagnosis of SFT was initially made. Nuclear STAT6 expression is highly specific and sensitive for SFT. This later returned as negative; therefore, GPC was confirmed. Conclusion: We present a case of sinonasal GPC with skull base involvement that was treated with endoscopic resection. At the most recent follow-up (6 months), the patient remained free of local disease.

CD34-Positive Spindle Cell Tumor With CTNNB1 Mutation: An Unusual Spindle Cell Variant of Sinonasal Glomangiopericytoma.

Sinonasal glomangiopericytoma is an uncommon mesenchymal tumor with a perivascular myoid phenotype, which is categorized as a borderline/low-grade malignant soft tissue tumor by the current World Health Organization Classification of Head and Neck tumors. Here, we present the case of a 53-year-old woman with an unusual spindle cell morphology of sinonasal glomangiopericytoma arising in the nasal cavity, mimicking solitary fibrous tumor. Microscopically, the tumor showed a cellular proliferation of spindle cells in fascicles including a focal long sweeping arrangement or whorls, or with a storiform growth pattern, associated with hemangiopericytoma-like gaping blood vessels embedded in a fibrous stroma. This arrangement of the spindle cells faintly indicated a solitary fibrous tumor rather than sinonasal glomangiopericytoma. Immunohistochemically, the tumor was positively reactive to not only beta-catenin (in the nuclei) but also CD34, although signal transducers and activators of transcription 6 was negative. Mutational analysis using Sanger sequencing detected a CTNNB1 mutation. We finally diagnosed the tumor as a sinonasal glomangiopericytoma, showing an unusual spindle cell variant. Such unusual spindle cell morphology with CD34-immunoreactivity potentially leads to an incorrect diagnosis of solitary fibrous tumor because such prominent fascicles including long sweeping structures, reminiscent of desmoid-type fibromatosis, have scarcely been described in the literature. Hence, careful morphological scrutiny using appropriate diagnostic adjuncts is necessary for correct diagnosis.

Spindle Cell Tumors of the Sinonasal Tract: A Diagnostic Update with Focus on Ancillary Workup.

Spindle cell neoplasms arising in the head and neck may be challenging to recognize due to their relative rarity. While underlying molecular alterations are increasingly elucidated, testing for these features may not be readily available. In most cases, combinations of key morphologic features and diagnostic immunohistochemical markers can be used to replace molecular diagnostics. Conversely, some molecular alterations and expression of their surrogate biomarkers are not specific for any one entity, and it is important to recognize these to avoid diagnostic pitfalls. In this review, we discuss both old and new spindle cell tumors of the sinonasal tract, with an emphasis on histologic features and clinically relevant immunohistochemical markers serving as surrogate markers for underlying genomic alterations.

Single Institution Experience With Sinonasal Glomangiopericytoma: A Case Series.

Introduction: Sinonasal glomangiopericytoma (GPC) is an uncommon neoplasm, accounting for less than 0.5% of all sinonasal tumors. It is characterized as having low malignant potential, with complete surgical excision representing optimal treatment. Presenting symptoms are typically due to mass effect and vascularity of this tumor, often including unilateral nasal obstruction or epistaxis. Accounts of this tumor in the literature remain sparse. Methods: Single institution retrospective review. Results: Six cases of sinonasal GPC were identified from a review of the electronic medical records spanning from 2009 through 2021. Age at diagnosis ranged from 48 to 67 years, with a gender distribution of 5 males and 1 female. Most subjects presented with unilateral sinonasal obstruction of variable duration. Each underwent endoscopic resection of the mass with negative margins, and no adjuvant therapy was pursued. Pathologic specimens demonstrated a vascular patterned tumor with spindled cells surrounding vessels and were positive for smooth muscle actin and negative for cytokeratin. Active post-surgical follow-up ranged from 11 months to 10 years. All patients were without endoscopic evidence of recurrence, and 2 had post-operative imaging which showed no evidence of disease. Conclusions: This review of 6 cases of sinonasal GPC represents the largest known series of this rare pathology in the literature to date. Based on our experience, and in agreement with the available literature, this disease is reliably managed with complete surgical excision. Adjuvant therapy can be avoided in otherwise uncomplicated cases. Although rare, GPC should be considered in the differential diagnosis of all vascular sinonasal tumors.

Unveiling a Rarity: A Case Report on Glomangiopericytoma in the nasal cavity.

Sino-nasal glomangiopericytoma is a rare benign tumour comprising only about 0.5% of all sino-nasal tumours. Presenting as a bleeding nasal mass, it is among the myriad of differential diagnoses for the same. Clinical characterisation of mass becomes difficult; hence, histopathology and immunohistocytochemistry play an essential role in clenching the diagnosis. Optimal treatment includes complete tumour excision with endoscopic or open approaches with or without preoperative embolization and a long post-operative follow-up period. Here we report such a case treated with endoscopic approach. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03975-z.

Top 10 Significant Spindled Head and Neck Lesions to Scrutinze.

BACKGROUND: Spindled lesions are a challenging area in head and neck pathology. This is particularly true in the sinonasal tract, where several uncommon entities with both unique and overlapping morphologic, immunophenotypic, and/or molecular features can occur. METHODS: Review. RESULTS: The clinicopathologic characteristics of biphenotypic sinonasal sarcoma and nine important differential diagnostic considerations with one or more overlapping feature are summarized to establish a practical framework for approaching spindled lesions of the sinonasal tract. CONCLUSION: Morphologic evaluation is central to the work up of sinonasal spindle cell lesions-in particular, cellular morphology, tumor architecture and growth pattern, and the presence of admixed epithelial elements - however, focused immunohistochemical analysis of neural, myogenic, rhabdomyoblastic, epithelial, and/or melanocytic marker expression and/or ancillary tests for tumor-specific molecular alterations may be necessary for definitive diagnosis.

Primary intranasal perivascular wall tumors in 2 cats.

Perivascular wall tumors (PWTs) are common well-known canine mesenchymal tumors. The term PWT has not yet been applied to cats; only 2 cases of feline soft tissue hemangiopericytomas (HEPs) are available. In human medicine, sinonasal HEP-like tumor/glomangiopericytoma (SHPCL/GP) and intranasal solitary fibrous tumor (SFT) are well-known mesenchymal tumors with staghorn vasculature and low malignant potential; however, these entities have not been described in small animals. We describe here the pathologic and immunohistochemical features of 2 cases of feline intranasal mesenchymal tumors consistent with PWTs and resembling human SHPCL/GP (case 1), and human intranasal SFT (case 2). Both cats developed intranasal, unilateral, polypoid, expansile neoplasms with a mostly patternless growth of spindle cells, minimal stroma, and prominent staghorn vessels. The stroma was PAS negative, which excludes a glomus tumor. Immunohistochemistry identified diffuse vimentin and PDGFRß expression. Case 1 was α-SMA positive (as is human SHPCL/GP); case 2 was negative (as is human intranasal SFT). Both tumors were incompletely excised, leading to recurrence in case 1. Case 2 was lost to follow up. To our knowledge, intranasal PWTs have not been reported previously in cats. The frequency of the lesions is not known, but awareness of these entities may assist in their recognition and better characterization in the future.

A rare case of glomangiopericytoma in the nasal cavity: A case report in light of recent literature.

Introduction and importance: Glomangiopericytoma (GPC) is a rare sinonasal tumor that behaves benignly with a long overall survival rate. It accounts for fewer than 0.5% of all sinonasal tumors. Case presentation: We report the case of a 64-year-old man who presented with recurrent episodes of epistaxis. Rhinoscopy revealed a left posterior nasal septal mass with active oozing. Computed tomography (CT) showed a well-defined soft tissue lesion in the left nasal cavity measuring 1.95 × 1.51 cm. Complete endoscopic resection was successfully performed. Histopathological findings favored the diagnosis of GPC as it revealed tumor cells positive for smooth muscle actin and ß-catenin with immunopositivity for CD34. Clinical discussion: Presenting symptoms of GPC are predominated by epistaxis and nasal obstruction. Since CT and MRI merely lead to a presumptive diagnosis, histopathological findings are indispensable. Complete surgical excision of GPC remains the treatment of choice with excellent prognosis, especially when immunohistochemistry is positive for actin and CD34 immunostaining is negative. Conclusion: GPC is a rare indolent tumor of pericytes that has a macroscopic appearance of a nasal polyp, which may result in uncertainty in the initial diagnosis. In most cases, GPC warrants only local excision. This case report adds to the literature and helps galvanize the developing clinical guidelines for diagnosis and treatment.

Sinonasal Glomangiopericytoma with Prolonged Postsurgical Follow-Up.

Sinonasal glomangiopericytoma is a rare vascular tumor of the respiratory epithelium. Treatment consists mainly of surgical resection, though there is no consensus regarding the use of adjuvant therapies or preoperative endovascular embolization. The postsurgical prognosis is favorable, though there is a high risk of delayed recurrence. Here, we present the case of a patient who underwent endoscopic resection of a sinonasal glomangiopericytoma and a review of the literature.

Glomangiopericytoma of the inferior nasal turbinate: A case report.

INTRODUCTION: Glomangiopericytoma is defined as a sinonasal tumor with perivascular myoid phenotype, which was first described in 1942 by Stout and Murray as a soft tissue tumor with characteristic proliferation including branching vessels and small vessel perivascular hyalinization. The tumor accounts for less than 0.5 % of all sinonasal neoplasms. The World Health Organization (WHO) classified this tumor as glomangiopericytoma in 2005. CASE REPORT: A 47-year-women presented with two years history of permanent left nasal obstruction and frequent epistaxis. Rhinoscopy revealed a friable grayish pink polypoidal mass, fully occupying the left anterior naris. Computed tomography showed a lesion involving the left nasal cavity, with a soft tissue density (70 UH) measuring 50 × 16 mm, widely infiltrative. Endoscopic surgery was performed to remove the mass, considering the size, limited expansion and the accessible location of the tumor. The immunohistochemistry examination showed positive staining b-catenin tumor cells which confirmed the diagnostic of glomangiopericytoma. After a 2 years follow-up, the patient showed no signs of recurrence. CONCLUSION: Glomangiopericytomas generally arises in the nasal cavity and may extend into the paranasal sinuses. It is categorized as a borderline low malignancy tumor by the WHO classification. Complete transnasal endoscopic excision is the optimal treatment.