Glomangiopericytoma of the Infraorbital Region.

Solitary fibrous tumors (SFTs) are well recognized in the head and neck region, but rarely arise in the sinonasal tract (SNT). Six primary SNT SFTs were identified in the files of Southern California Permanente Medical Group between 2006 and 2017. The patients included five males and one female ranging in age from 33 to 72years (mean 52years), most of whom presented clinically with nasal obstruction. Three tumors involved the nasal cavity alone, one involved the paranasal sinuses, and two involved both the nasal cavity and paranasal sinuses. Histologically, the tumors were characterized by a variably cellular proliferation of cytologically bland spindle cells within a collagenous stroma with prominent interspersed branching vessels. Mitotic activity was low (range 0-2 per 10 high power fields) and there was no evidence of pleomorphism or tumor necrosis. Surface ulceration was noted. By immunohistochemistry, the lesional cells were positive for CD34, STAT6 and bcl-2. Clinical follow up information was available for all patients (range 32-102months; mean 72months). There were no recurrences or metastases and all were alive with no evidence of disease at last follow-up. SFTs rarely affect the SNT, but should be considered in the differential diagnosis of SNT mesenchymal lesions. Immunohistochemical expression of STAT6 can aid in diagnosis and separation of SFT from other spindle cell lesions occurring at this anatomic site. In combination with cases reported in the literature, primary SNT SFT behave in an indolent manner with conservative treatment.

Solitary fibrous tumors (SFTs) of the head and neck are uncommon. Lesions previously diagnosed in the head and neck as hemangiopericytomas (HPCs), giant cell angiofibromas (GCAs), and orbital fibrous histiocytomas (OFHs) are now recognized as within the expanded spectrum of SFTs. To better understand the clinicopathologic profile of head and neck SFTs, we performed a multi-institutional study of 88 examples. There was no sex predilection (F:M ratio 1.2), and the median patient age was 52 years (range: 15 to above 89 y). The sinonasal tract and orbit were the most common sites involved (30% and 25%), followed by the oral cavity and salivary glands (15% and 14%). Original diagnoses included HPC (25%), SFT (67%), and OFH (6%), with 1 SFT and 1 OFH noted as showing GCA-like morphology. On review, the predominant histologic pattern was classic SFT-like in 53% and cellular (former HPC-like) in 47%; lipomatous differentiation (8%) and GCA-like pattern (7%) were less prevalent. Subsets demonstrated nuclear atypia (23%), epithelioid morphology (15%), or coagulative necrosis (6%). Infiltrative growth (49%) and osseous invasion (82%) were prevalent among evaluable cases. Of the 48 SFTs with follow-up (median: 43 mo), 19 showed recurrence (40%). Of these, 4 patients were alive with disease and 4 dead of disease. Size and mitotic rate were negative prognosticators using a joint prognostic proportional hazards regression model. Three patients experienced metastasis, to lungs, parotid, bone, and skull base, including one case showing overtly sarcomatous "dedifferentiation." As a group, SFTs present in a wide anatomic and morphologic spectrum in the head and neck. Only rare examples metastasize or cause death from disease. However, the fairly high local recurrence rate underscores their aggressive potential and highlights the importance of prospective recognition.

Solitary fibrous tumor of the middle ear: Immunoexpression of NAB2-STAT6 fusion gene

BackgroundA solitary fibrous tumor (SFT) is a rare mesenchymal tumor that occurs mostly in pleural sites, and an SFT occurring in the ischiorectal fossa is extremely rare. Because of the rarity, there are few reports detailing an SFT in the ischiorectal fossa.Case presentationA pararectal tumor was incidentally found in a 42-year-old man during a routine medical examination. The patient had no symptoms and no previous medical history. In the physical examination, a smooth-margined and hard elastic mass was felt, and in a digital rectal examination, the rectal mucosa appeared normal. A computed tomography (CT) scan showed a 5-cm, well-defined, solid mass in the left ischiorectal fossa. Contrast-enhanced CT in the early phase showed intense heterogeneous enhancement that persisted during the delayed phase. T2-weighted images of magnetic resonance imaging yielded heterogeneous intermediate and low signal intensity. Intense arterial enhancement suggested a hypervascular nature, and persistent delayed enhancement and low signal bands on T2-weighted images suggested a fibrous component of the mass. An SFT was suspected. Most SFTs are benign but have malignant potential. Our patient did not hope for surgery if the tumor was benign; therefore, an ultrasound-guided transperineal core needle biopsy was performed to decide on a treatment strategy. Microscopic examination showed tumor cells appearing as spindle and fibroblast-like cells within a collagenous stroma. Immunohistochemistry identified CD34 and vimentin, supporting the diagnosis of an SFT. The patient consented to excision of the mass. He was placed in a prone jackknife position, and the tumor was removed transperineally using a posterior approach (modified Kraske procedure). The levator ani muscle, external sphincter muscles, and rectum were not involved and separated from the tumor. The tumor was successfully resected en bloc with no complications. Five uneventful days post surgery, the patient was discharged. There was no local recurrence during the year following surgery.ConclusionImaging findings reflect the tissue characterization such as hypervascularity and fibrous nature of SFTs. We have presented a rare case of an SFT in the ischiorectal fossa with useful imaging findings for diagnosis, treatment strategy, and successful surgical removal using a posterior approach.

BENIGN PLEURAL SCHWANNOMA WITH PLEURAL EFFUSIONS AND NECROTIZING PNEUMONIA

Purpose To report a rare case of Inverting Papilloma originating from the Lacrimal Sac in Tertiary Hospital. Method Case report. Results Inverting papilloma is uncommon in lacrimal sac. More often presents in Maxillary sinus, Ethmoid sinus and lateral wall of nasal cavity. This case of 69 years old, Filipino male, who presented with a two-year history of gradually enlarging left medial canthal mass, and tearing. There were no ophthalmoplegia, visual disturbances, proptosis or pain but there was globe displacement superolaterally. Other ophthalmologic examinations were unremarkable at that time of examination. Orbital CT-scan revealed: Heterogenously enhancing mass extending to ipsilateral nasolacrimal canal and nasal cavity causing mass effect. There was note of a suspicious area of hyperostosis. Patient was referred to the Department of Otolaryngology Head and Neck Surgery for co-management. Punch biopsy was done and histopathology revealed Inverting papilloma. Combined surgical management of anterior Maxillectomy via lateral Rhinotomy approach and Dacryocystectomy was done. Intraoperatively, there was note of adhesions to bone in the area of the Lacrimal Sac corresponding to the area of hyperostosis on CT-scan. Post-operative recovery was unremarkable with unchanged visual acuity and extraocular muscle movement. Conclusions This is a case of an inverting papilloma involving the lacrimal sac and nasolacrimal duct that was managed with combined anterior maxillectomy via Lateral Rhinotomy approach and dacryocystectomy for complete resection. We recommended complete resection of the tumor to minimize recurrence. Origin in the Lacrimal Sac area was confirmed by intraoperative findings which correlated with CT- scan findings.

Objective: To report a rare case of inverting papilloma originating from the lacrimal sac, and to review the literature of inverting papilloma of the lacrimal sac as main origin. Method: Case report. Results: Inverting papilloma is uncommon in the lacrimal sac. More often it presents in the maxillary sinus, ethmoid sinus and lateral wall of the nasal cavity. This is a case of a 69 year old, Filipino male, who presented with a two-year history of gradually enlarging left medial canthal mass, and tearing. The patient had no history of trauma. There were no ophthalmoplegia, visual disturbances, proptosis or pain but there was globe displacement superolaterally. Orbital CT-scan revealed a heterogenously enhancing mass extending to the ipsilateral nasolacrimal canal and nasal cavity causing mass effect. There was note of a suspicious area of hyperostosis. Patient was referred to the Department of Otolaryngology Head and Neck Surgery for co-management. Punch biopsy was done and histopathology revealed inverting papilloma. Combined surgical management of anterior maxillectomy via lateral rhinotomy approach and dacryocystectomy was done. Intraoperatively, there was note of adhesion to bone in the area of the lacrimal sac corresponding to the area of hyperostosis on CT-scan. Post-operative recovery was unremarkable with unchanged visual acuity and extraocular muscle movement. Conclusion: This is a case of an inverting papilloma involving the lacrimal sac and nasolacrimal duct that was managed with combined anterior maxillectomy via lateral rhinotomy approach and dacryocystectomy for complete resection. We recommended complete resection of the tumor to minimize recurrence. Origin in the lacrimal sac area was confirmed by intraoperative findings which correlated with CT- scan findings.  

Solitary fibrous tumors are rare mesenchymal tumors that typically occur in the pleura. Solitary fibrous tumors of the uterine cervix are uncommon. We report the first case of a patient who underwent total hysterectomy for vaginal wall adenocarcinoma and was found to have a concurrent solitary fibrous tumor in the paracervical-uterus. A 51-year-old woman was admitted to our hospital due to contact bleeding. A gynecological examination revealed nodules of 3.0 × 1.0 cm on the vaginal wall, and a colposcopy with biopsy revealed adenocarcinoma of the vaginal wall. After the recommended staging examinations, the patient underwent total hysterectomy, double adnexectomy, pelvic lymph node dissection, and vaginal wall resection. During surgery, a nodule measuring approximately 2 × 2 cm was found in the middle of the mass in the left paracervical region. Subsequent postoperative histopathological examination confirmed an solitary fibrous tumor of the uterine cervix with adenocarcinoma of the vaginal wall. The patient was followed up for 46 months after hospitalization, and no recurrence or distant metastases were observed. In rare cases, solitary fibrous tumors may form large masses in the cervical or vaginal wall. They can easily be misdiagnosed as benign or malignant cervical tumors before and during surgery. Postoperative pathology and immunohistochemistry are helpful for diagnosis. Most solitary fibrous cervical tumors are benign, occasionally with low malignant potential, and surgical treatment is feasible and effective.

Sinonasal haemangiopericytoma (SN-HPC) is a rare sinonasal mesenchymal neoplasm of perivascular myoid cell origin. Solitary fibrous tumour (SFT) occurs only very rarely in the sinonasal tract. SFT and soft tissue HPC have been considered a single entity. Recently, recurrent gene fusions involving NAB2-STAT6 resulting in differential expression of STAT6 were characterized as central molecular events in SFT. However, no data exist for NAB2-STAT6 status or STAT6 expression in SN-HPC. We examined six SN-HPCs and two sinonasal SFTs by immunohistochemistry and RT-PCR for NAB2-STAT6 fusions. SN-HPC affected three females and three males (mean age: 72years). They expressed smooth muscle actin, lacked strong CD34 reactivity and were negative for nuclear STAT6 expression. RT-PCR analysis confirmed the absence of NAB2-STAT6 fusions in all cases. Conversely, both sinonasal SFTs (in males aged 39 and 52years) displayed classical features of pleuropulmonary and soft-tissue SFTs (uniformly CD34-positive with strong nuclear expression of STAT6). RT-PCR revealed NAB2-STAT6 fusions in both cases. These findings confirm the molecular and phenotypical distinctness of these two entities. While SN-HPC is a site-specific sinonasal neoplasm of as yet unknown molecular pathogenesis, sinonasal SFTs show phenotypical and molecular identity to their pleural/extrapleural counterparts.

The Solitary Fibrous Tumor (SFT) is a rare mesenchymal neoplasm that arises mainly from the pleura. The sinonasal tract is generally not affected by SFT, and less than 100 cases have been reported in the English literature to date. We report an extremely rare SFT of the nasal cavity and paranasal sinuses extending into the anterior frontal fossa through the floor of the anterior skull base. To our knowledge, this case is the fourth SFT of the sinonasal tract involving the anterior frontal fossa in the world. Meanwhile, the tumor, measuring 13 × 6 cm in images, is the largest SFT of the sinonasal tract compared to previously reported cases. Three surgical procedures, including a transcranial one, were performed for the patient to achieve complete removal of the tumor. The diagnosis of SFT was established primarily by immunohistochemical positivity for CD34, STAT6, and negativity for S-100 protein. We emphasize the possibility of recurrence in SFT, and close follow-up is necessary with the help of nasal endoscopy and imaging approaches.

Solitary fibrous tumor of the retropharynx causing obstructive sleep apnea

Locally aggressive solitary fibrous tumor in the infraorbital region: A case report and review of the literature

We present a retrospective study of 65 cases of solitary fibrous tumors (SFTs) of several localizations including the most common site of origin in the pleura and lungs. SFTs are mesenchymal fibroblastic tumors with an unpredictable biological potential ranging from benign to malignant. We investigated morphologic characteristics, proliferation activity evaluated by immunohistochemical expression of Ki-67 antigen, and the existence of NAB2-STAT6 fusion gene together with Ki-67, TPX2, and TERT mRNA expression levels. The aim was to define relationships between proliferation activity and biological potential and progression of the disease. We measured Ki-67, TPX2, and TERT mRNA levels using quantitative real-time reverse transcription PCR (RQ-RT-PCR). We observed a significant association between increased Ki-67 and TERT mRNA levels and the SFTs with malignant potential. Also, we investigated the effect of TERT promoter mutation on telomerase activation and patient outcome in our SFT cohort. We verified that TERT promoter mutation was frequent (36.6%) and present in a majority of malignant SFTs and SFTs with uncertain biological behavior. TERT promoter mutation alone predicted the disease recurrence.

Solitary fibrous tumor (SFT) of the pleura is a rare mesenchymal neoplasm arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura and accounting for less than 5% of primary pleural tumors. SFTs are generally benign and asymptomatic, with 10-year survival rates of up to 98%. Unfortunately, approximately 10% have malignant potential, leading to local recurrence after radical surgery and/or metastatic spread. Of note, giant pleural SFT, which consists of a tumor occupying at least 40% of the affected hemithorax, is even less common with only anecdotal cases reported in the medical literature. We describe a unique case of giant SFT of the pleura that metastasized to the thyroid gland 1 year after complete resection, focusing on its clinical and pathological features of presentation. En bloc resection remains the mainstay of therapy with curative intent. Patients with large tumors may undergo preoperative angiography with percutaneous embolization of the tumor, which allows to reduce perioperative bleeding. In case of local recurrence, surgery still remains the best treatment option. However, surgery can also be considered in patients with isolated metastatic spread. Every suspected and proven SFT of the pleura should undergo surgical resection, as clinical and radiological criteria cannot accurately distinguish benign from malignant forms. Moreover, the peculiar histological features of SFT should not be neglected when planning clinicoradiological follow-up. Additionally, suspicious clinical findings during follow-up should always be thoroughly investigated in order to exclude or confirm the diagnosis of recurrent disease.

Several benign and malignant mesenchymal and meningothelial lesions may preferentially affect or extend into the sinonasal tract. Glomangiopericytoma (GPC, formerly sinonasal-type hemangiopericytoma) is a specific tumor with a predilection to the sinonasal tract. Sinonasal tract polyps with stromal atypia (antrochoanal polyp) demonstrate unique histologic findings in the sinonasal tract. Juvenile nasopharyngeal angiofibroma (JNA) arises from specialized tissue in this location. Meningioma may develop as direct extension from its intracranial counterpart or as an ectopic tumor. Selected benign mesenchymal tumors may arise in the sinonasal tract and pose a unique differential diagnostic consideration, such as solitary fibrous tumor and GPC or lobular capillary hemangioma and JNA. Although benign and malignant vascular, fibrous, fatty, skeletal muscle, and nerve sheath tumors may occur in this location, this paper focuses on a highly select group of rare benign sinonasal tract tumors with their clinicopathological and molecular findings, and differential diagnosis.