Sinonasal Hamartomas: From Nasal Chondromesenchymal Hamartoma to Respiratory Epithelial Adenomatoid Hamartoma. Report of six Cases and Review of the Literature

Sinonasal hamartomas are uncommon lesions of nasal and sinus cavities. Based on indigenous cellular components and characteristic histologic features, they are further classified into four entities: respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma (SH), chondro-osseous and respiratory epithelial hamartoma (CORE), and nasal chondromesenchymal hamartoma (NCH). REAH, SH, and CORE are seen in adult patients, while NCH predominantly occurs in newborns and infants. Morphologically REAH and SH are composed of respiratory epithelium and seromucinous glands, CORE is related to REAH but with additional feature of chondroid and/or osseous tissue, and NCH is composed of chondroid and stromal elements but devoid of epithelial component. All four lesions can present as sinonasal mass lesions and with associated obstructive symptoms. Given the rarity of these lesions, diagnosis can be challenging, especially in unusual clinical scenario. In this study, we report six cases of sinonasal hamartoma, including one case of NCH, one case of CORE, two cases of SH, and two cases of REAH. All cases were from adult patients including four men and two women. We also review the literature of the clinical and pathologic features of these rare lesions.

Sinonasal Plasmablastic Lymphoma Arising in the Setting of Recurrent Nasal Polyposis in an Immunocompetent Individual

Plasmablastic lymphoma (PBL) is an aggressive, rare variant of B-cell lymphoma typically associated with human immunodeficiency virus and other immunocompromised populations. Most commonly found in the oral cavity, PBL can occasionally originate in the sinonasal tract. Diagnosis of PBL is difficult due to overlapping features with other malignancies; however, early detection and treatment are imperative given its aggressive clinical course. When in the sinonasal tract, the diagnostic process can be further complicated if the patient has a history of recurrent nasal polyposis. Described is the case of a 57-year-old immunocompetent male who initially presented with benign nasal polyposis, only to return a year after sinus surgery with a unilateral sinonasal mass consistent with PBL. As literature has yet to characterize this phenomenon, this article presents the first case reported of sinonasal PBL arising in the setting of recurrent nasal polyposis. This case emphasizes the importance of investigating sinonasal masses showing laterality, maintaining a high index of suspicion for malignancy, and keeping close surveillance of the patient after treatment of PBL.

HPV-Related Multi-Phenotypic Sinonasal Carcinoma with Aggressive Clinical Behavior; A Rare Case

Introduction/Objective Human Papilloma Virus (HPV)-related multiphenotypic sinonasal carcinoma, previously known as HPV-related sinonasal carcinoma with adenoid cystic carcinoma-like features, is a rare type of sinonasal carcinoma with both epithelial-derived and salivary gland-type morphologic features. It is associated with high-risk HPV, but lacks MYB gene rearrangements. Methods/Case Report We report a case of a 59-year-old male who presented with a rapidly growing sinonasal mass. On MRI, a left nasal cavity lesion was identified growing laterally along the frontal process of the maxilla, extending into the middle meatus and into the maxillary sinus. Patient underwent a complex left medial maxillectomy, spheno- ethmoidectomy, and sinusotomy. On gross evaluation, the left inferior turbinate and sidewall demonstrated a 4 cm unremarkable turbinate with attached friable soft tissue. Microscopic examination revealed sections of carcinoma with various architectural patterns comprised of foci with adenoid cystic carcinoma-like morphology, basaloid squamous cell carcinoma and adenocarcinoma. The tumor showed positive immunostaining for P40, but focal reactivity to S100 and rare scattered reactivity with CD117. INI-1 immunostain was retained in tumor cells. P16 immunostain was strong and diffuse and high-risk cocktail HPV RNA ISH was positive. However, MYB FISH testing was equivocal. Morphologic and immunophenotypic findings were consistent with HPV-related multiphenotypic sinonasal carcinoma. The tumor involved the olfactory nerve fibers requiring a skull base resection and showed extension into the dura mater. Results (if a Case Study enter NA) NA Conclusion HPV related multiphenotypic sinonasal carcinoma is a recently described entity that can pose significant diagnostic challenge. It typically has an indolent clinical course with potential for late recurrences. This case study highlights the potential aggressive nature of this type of sinonasal carcinoma, despite association with high-risk HPV, and use of ancillary testing in aiding diagnosis.

Oncogenic osteomalacia secondary to glomus tumor

Summary Oncogenic osteomalacia secondary to glomus tumor is extremely rare. Localization of causative tumors is critical as surgical resection can lead to a complete biochemical and clinical cure. We present a case of oncogenic osteomalacia treated with resection of glomus tumor. A 39-year-old woman with a history of chronic sinusitis presented with chronic body ache and muscle weakness. Biochemical evaluation revealed elevated alkaline phosphatase hypophosphatemia, increased urinary phosphate excretion, low calcitriol, and FGF23 was unsuppressed suggestive of oncogenic osteomalacia. Diagnostic studies showed increase uptake in multiple bones. Localization with MRI of paranasal sinuses revealed a sinonasal mass with concurrent uptake in the same area on the octreotide scan. Surgical resection of the sinonasal mass was consistent with the glomus tumor. The patient improved both clinically and biochemically postoperatively. Along with the case of oncogenic osteomalacia secondary to a glomus tumor, we have also discussed in detail the recent development in the diagnosis and management of oncogenic osteomalacia. Learning points Tumor-induced osteomalacia is a rare cause of osteomalacia caused by the secretion of FGF23 from mesenchymal tumors. Mesenchymal tumors causing TIO are often difficult to localize and treat. Resection of the tumor can result in complete resolution of biochemical and clinical manifestations in a very short span of time. Glomus tumor can lead to tumor induced osteomalacia and should be surgically treated.

Glomus Tumor: An Unusual Cause of Hypophosphatemic Osteomalacia

Background: Tumor-induced osteomalacia (TIO) is a rare condition resulting in hypophosphatemic osteomalacia. We present a rare case of TIO secondary to glomus tumor. Clinical Case: A 39-year-old woman with history of chronic sinusitis presented with progressively worsening generalized body pain and muscle weakness of eight months duration. Examination showed decreased muscle strength in bilateral upper and lower extremities and congenital cleft palate. Laboratory work up revealed elevated alkaline phosphatase 603 U/L (44–147 U/L), low serum phosphorus 1.5 mg/dL (3.5–5.0 mg/dL), normal serum calcium 8.9 mg/dL (8.3–10.4 mg/dL), normal 25-hydroxyvitamin D 32 ng/dL (30–100 ng/dL), elevated 1,25-dihydroxyvitamin D 62 g/mL (20–45 pg/mL), elevated intact PTH level 99.01 pg/mL (8–74 pg/mL), high normal 24 hour urinary phosphate levels 1100 mg/dl, and elevated FGF23 level 128 RU/mL (<108 RU/mL). Fractional excretion of filtered phosphate (FEPO4) [FEPO4 = (Urine phosphate / Serum phosphate) / (Urine creatinine / Serum creatinine) * 100] was 107.54 % (normal range <20%). Tubular reabsorption of phosphate (TRP) [Percentage TRP = 100 * {1- (Urine phosphate / Serum phosphate) / (Urine creatinine / Serum creatinine)}] was -7.54 %. Tubular maximum reabsorption of phosphate to glomerular filtration rate (TmP/GFR) calculated using formula [TmP/GFR = TRP x serum phosphate] was -0.1131 mg /dL (2.8–4.2 mg/dL). Bone densitometry revealed normal bone density and parathyroid scintigraphy was negative for adenoma. Clinical symptoms along with biochemical evidence of phosphate wasting confirmed by low TRP and an unsuppressed FGF23 levels suggested oncogenic osteomalacia as underlying etiology. Whole body bone scan demonstrated increased radiotracer uptake in the elbows, spine, sacroiliac joints, knees, ankles and multiple ribs suggestive of inflammatory process. MRI of paranasal sinuses revealed large soft tissue mass occupying right frontal, ethmoid, maxillary and sphenoid sinuses suggestive of sinonasal polyposis. 99mTc-Octreotide scan confirmed increased uptake in the nasopharynx. The sinonasal mass was resected and pathology revealed the mass to be a glomus tumor confirmed on immune histochemical studies with positive staining for vimentin and SMA and Ki67 of 5–10 %. Postoperatively, phosphorus levels normalized to 3 mg/dl on day 7. Conclusion: Glomus tumor as cause of TIO is extremely rare however localization and surgical resection of tumor can result in complete resolution of biochemical and clinical manifestations. References: 1. Minisola, S., et al., Tumour-induced osteomalacia. Nat Rev Dis Primers, 2017. 3: p. 17044.

Fibrolipoma presenting as a posterior nasal septum mass: a report of a unique case and review of the literature

Unilateral sinonasal mass presenting as nasal block is a common presentation but poses a diagnostic challenge. The differential diagnosis includes inflammatory polyps, benign and malignant sinonasal neoplasm. Fibrolipoma presenting in the sinonasal area is an uncommon pathology. As it is uncommon, there is potential for a misdiagnosis, unnecessary investigation and delay in the definitive treatment. We report a patient with a fibrolipoma originating from the posterior nasal septum. The diagnostic and surgical features of this unique case are discussed.

Challenges in Medicine: The Odyssey of a Patient with Isolated IgG4-Related Eosinophilic Angiocentric Fibrosis Presenting as a Locally Destructive Sinonasal Mass

Eosinophilic angiocentric fibrosis (EAF) is an exceeding rare clinical entity and is considered a part of the spectrum of IgG4-related disease (IgG4RD). We hereby present such an unusual case of a 60-year-old female who presented to us with recurrent sinonasal mass, after a decade long haul of multiple clinical evaluations, biopsies, and debulking surgery without a definitive diagnosis. Over this period, the mass eroded through the ethmoid cells along with nasal septal destruction leading to saddle nose deformity, extended superiorly through the cribriform plates to right frontal lobe, and compressed the optic nerve leading to visual loss. Although initial biopsy was negative, repeat biopsy was performed owing to high clinical suspicion due to all the classic histopathological findings compatible with the diagnosis of eosinophilic angiocentric fibrosis IgG4-related disease (EAF-IgG4RD). Steroids are the recommended first-line therapy; however, our case was resistant to steroids needing rituximab to halt the disease progression. Our case interestingly also had T-cell clonality and isolated isocitrate dehydrogenase 2 enzyme mutation on next-generation sequencing, suggesting a possible role of novel molecular-targeted therapies in this rare disease. This case highlights the clinical challenges physicians face towards diagnosing and treating EAF-IgG4RD, emphasizing the need for high clinical suspicion and the possible role of targeted therapies for this rare disease.

Different Faces of Sinonasal Mass Lesions

Introduction Mass in the nasal cavity presents with a wide range of symptoms, when a presumptive diagnosis is often made with the help of imaging and endoscopy. This study focussed on correlating clinical diagnosis with the histopathological diagnosis so that appropriate treatment can be offered to improve the quality of life of the patient. Materials and Methods The study included 120 cases who presented with symptoms and signs of mass in the nasal cavity, undergoing surgery or diagnostic biopsy. They were evaluated with a detailed history and clinical examination, diagnostic nasal endoscopy, and relevant radiological investigations. Histopathological examination of the biopsy of the excised specimen was performed by Haematoxylin and Eosin stain. Special stains and Immunohistochemistry (IHC) were performed whenever indicated. The clinical diagnosis was correlated with histopathological diagnosis. Results Nasal obstruction was the most frequent symptom followed by nasal discharge. Non-neoplastic lesions made up 85% of cases, while16% of cases were proved as neoplastic lesions. Among neoplastic lesions, 7% were benign, and 9% were malignant. The inflammatory polyp was the most common non-neoplastic lesion. Fischer's exact test showed a correlation between clinical diagnosis and histopathological diagnosis. Non-neoplastic lesions were common in the 4th decade of life; benign lesions were common in the 3rd decade of life, while malignant lesions were common in the 5th decade of life. Conclusion Sinonasal masses present with overlapping clinical features, and sometimes the definite diagnosis is possible only by histopathological examination of the specimen. However, in the presence of characteristic clinical features, accurate clinical diagnosis is possible in most cases, and appropriate treatment can be performed without delay, pending histopathological examination.

Nasal Myiasis: A Neglect State

Background: Nasal myiasis is a parasitic condition of human being and animal species in which nose and paranasal sinuses infested by Diptera Larvae of Chrysomya albiceps and Oestrus ovis group of flies. It is rare and sporadic, usually occurs in adults, the elderly, debilitated poor, and neglected patients suffering from chronic Sinonasal diseases. Methods: A cohort retrospective study of 11 cases in the Department of Otolaryngology and Head-Neck Surgery, Comilla Medical College Hospital, Cumilla, Bangladesh from 01 July 2016 to 31 June 2020. Results: Incidence of nasal myiasis out of total admitted in the inpatient department was 0.03%. Of them, the female was 10 (90.91%), the male 01 (9.09%) (P-value <0.001), age range 35-70 years, the adult was 09 (81.82%), and the elderly 02 (18.18%) (P-value <0.001). The left nostril exhibited 08 (72.73%), and right nostril 03 (27.27%) (P-value <0.05). Social class showed poor was 08 (72.73%), and lower middle class to working 03 (27.27%) (P value< 0.05), villagers was 09 (81.82%), and slum dwellers 02 (18.18%) (P-value <0.001), Sinonasal mass was 07 (63.64%), and atrophic rhinitis 04 (36.36%) (P-value <0.001), Sinonasal malignancy was 06 (85.71%), and benign 01 (14.29%) (P-value <0.001). Conservative traditional treatment was 07 (63.64%), and endoscopic removal 04 (36.36%) (P-value <0.001), recovery without complication was 10 (90.91%), and complication occurred in 01 (9.09%) (P value<.001). Conclusion: Nasal myiasis is a progression of other disease processes of the nose and paranasal sinuses of adults and the elderly in a low socioeconomic group of people. They need extra care medical services to overcome it.