Naso-Ethmoidal Phosphaturic Mesenchymal Tumor: A Rare Tumor Site for an Uncommon Paraneoplastic Syndrome

Riminucci, Mara; Colangelo, Luciano; Ungari, Claudio; Cassoni, Andrea; Minisola, Salvatore; Corsi, Alessandro

doi:10.1177/0145561320940869

Cited by 12 publications

(3 citation statements)

References 10 publications

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“…The overlying epithelium was unremarkable. Immunohistochemical analysis (Figure 2), performed as previously, 4 -6 revealed immunoreactivity of the neoplastic cells for Vimentin, EMA, Claudin-1, CD34, and CD99 and their negativity for S100, CD117, Desmin, smooth muscle actin, muscle-specific actin, and progesterone receptor. Many mastocytes were highlighted by CD117.…”

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confidence: 53%

Extraneural Sclerosing Perineurioma of the Tongue

et al. 2021

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Perineurioma (PN) is an uncommon benign peripheral nerve sheath tumor. For the rarity of this tumor in the oral cavity, otolaryngologists and oral surgeons might not be familiar with this entity. Perineuriomas are typically benign and complete excision is deemed adequate management. Thus, their histological recognition is mandatory to avoid unnecessary overtreatment. We report the clinicopathologic findings of an uncommon variant, the Extraneural Sclerosing PN, in an unusual and never described site, the tongue.

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confidence: 53%

Extraneural Sclerosing Perineurioma of the Tongue

et al. 2021

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“…For example, mean serum phosphate was barely in the normal range, fractures persisted after 2 years of treatment, new fractures developed, and the effect on pain was absent in one study [ 3 ] and moderate in the other [ 4 ]. If these findings reflect the need to better define the appropriate dosing regimen [ 5 ] and/or the expression (and secretion) by tumor cells of other phosphatonins (e.g., MEPE, FGF7, sFRP-4) [ 1 , 5 , 16 – 18 ], it remains to be established. Interestingly, in two cases of TIO caused by tumors located in the occipital bone, PRRT 177 Lu-DOTATATE was used [ 8 , 11 ].…”

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confidence: 99%

Occipital bone and tumor-induced osteomalacia: a rare tumor site for an uncommon paraneoplastic syndrome

et al. 2023

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Introduction Tumor-induced osteomalacia (TIO) is an uncommon paraneoplastic syndrome due to the overproduction of fibroblast growth factor 23 (FGF23). It is predominantly caused by mesenchymal tumors and cured upon their complete removal. Non-surgical treatment is an alternative option but limited to specific clinical conditions. Methods We report a challenging case of TIO caused by a tumor involving the occipital bone. We also performed a literature review of TIO caused by tumors localized at this site, focusing on clinical findings, treatment, and outcomes. Results The patient, a 62-year-old male, presented with a long-lasting history of progressive weakness. Biochemical evaluation revealed severe hypophosphatemia due to low renal tubular reabsorption of phosphate with raised intact FGF23 values. A 68 Ga-DOTATATE PET/TC imaging showed a suspicious lesion located in the left occipital bone that MRI and selective venous catheterization confirmed to be the cause of TIO. Stereotactic gamma knife radiosurgery was carried out, but unfortunately, the patient died of acute respiratory failure. To date, only seven additional cases of TIO have been associated to tumors located in the occipital bone. Furthermore, the tumor involved the left side of the occipital bone in all these patients. Conclusion The occipital region is a difficult area to access so a multidisciplinary approach for their treatment is required. If anatomical differences could be the basis for the predilection of the left side of the occipital bone, it remains to be clarified.

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“…The stromal axis was loose, virtually devoid of inflammatory cells and enriched in small nerve bundles that appeared in close proximity to small-size blood vessels (Figure 1 A-D). Immunohistochemical analysis, performed as described previously, [4][5][6][7][8][9] highlighted the vascular and neural components in each individual lesion (Figure 1 E-G). Immunostain for HPV (clone K1H8) was negative.…”

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Squamous cell papilloma-like presentation of multiple neurovascular hamartomas of the oral cavity

et al. 2021

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Significance Statement: Neurovascular hamartomas (NVH) is an uncommon tumor-like developmental anomaly. We hereby report the case of a 28-year-old woman presenting with multiple millimetric excrescences in the oral cavity that were clinically interpreted as squamous cell papilloma and histologically consistent with NVHs. Neurovascular hamartomas is rare in the oral cavity. To the best of our knowledge, multiple NVHs have never been reported at this site.

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Naso-Ethmoidal Phosphaturic Mesenchymal Tumor: A Rare Tumor Site for an Uncommon Paraneoplastic Syndrome

Cited by 12 publications

References 10 publications

Extraneural Sclerosing Perineurioma of the Tongue

Extraneural Sclerosing Perineurioma of the Tongue

Occipital bone and tumor-induced osteomalacia: a rare tumor site for an uncommon paraneoplastic syndrome

Squamous cell papilloma-like presentation of multiple neurovascular hamartomas of the oral cavity

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