Pseudotumor fibroso calcificante de cuello en una paciente adolescente: reporte de caso / Calcifying fibrous pseudotumor of the neck in an teenager female patient: case report

RESUMEN: Introducción: El pseudotumor fibroso calcificante es un tumor benigno de tejidos blandos que aparece principalmente en niños y adultos jóvenes entre 20 y 30 años de edad, aún sin una etiología clara y definida. De distribución corporal variable, siendo relativamente poco común en el cuello. Caso clínico: Se presenta el caso de una paciente adolescente de 17 años con tumor en el cuello de rápido crecimiento que se manifestó con dolor moderado localizado, ronquido excesivo y dificultad respiratoria progresiva. Tratamiento: Se realiza resección completa del tumor localizado en hipofaringe, la cual confirma el diagnóstico histopatológico e inmunohistoquímico de pseudotumor fibroso calcificante. Conclusión: El pseudotumor fibroso calcificante de cuello es una patología benigna rara, con síntomas inespecíficos y muy probablemente, inducida porque el tumor comprime los tejidos circundantes. La resolución quirúrgica sigue siendo el Gold estándar (GS) en cuanto al tratamiento. El pronóstico postresección es bueno a largo plazo, con tasas bajas de recurrencias. Se discute la aproximación diagnóstica y terapéutica en un paciente adolescente comparado con lo descrito en la literatura.

ABSTRACT Introduction: Calcifying fibrous pseudotumor is a benign soft tissue tumor, appearing mainly in children and young adults between 20 and 30 years of age, still without a clear and defined etiology. It has a variable body distribution, being relatively uncommon in the neck. Case report: We present the case of a 17-year-old teenager female patient with a rapidly growing neck tumor, which manifested with moderate localized pain, excessive snoring and progressive respiratory distress. Treatment: Complete resection of the tumor located in the hypopharynx was performed, which confirmed the histopathological and immunohistochemical diagnosis of calcifying fibrous pseudotumor. Conclusion: Calcifying fibrous pseudotumor of the neck is a benign pathology, rare with non-specific symptoms and most probably induced by the tumor compressing the surrounding tissues. Surgical resolution remains the gold standard for treatment. The prognosis after resection is good in the long term, with low recurrence rates. The diagnostic and therapeutic approach in this teenager patient is discussed compared to that described in the literature

Sclerosing angiomatoid nodular transformation of spleen with calcifying fibrous pseudotumor: A rare case with unusual association

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.

Calcifying Fibrous Pseudotumor of the Stomach That Was Diagnosed by Endoscopic Submucosal Dissection / 대한소화기내시경학회지

Calcifying fibrous pseudotumor (CFPT) is very rare and especially at an intrinsic visceral location such as the stomach. The CFPT is generally located in the subcutaneous or deep soft tissue and the subserosal area of organs and it is mainly diagnosed during childhood or young adulthood. The etiology and pathophysiology of CFPT are unclear. Herein we describe a case of gastric CFPT and we review the related literature. A thirty-year-old asymptomatic female patient visited our hospital for the evaluation of a submucosal tumor that was seen on previous endoscopy. On the endoscopic ultrasonography (EUS), a subepithelial lesion was found at the gastric angle and it was a slightly hypoechoic, homogenous mass located at the submucosal layer of the gastric wall. Endoscopic submucosal dissection was performed under the suspicion of carcinoid. On the microscopic findings, most of the tissue was composed of hyalinized collagenous tissue. Mild lymphocytic infiltration and several dystrophic calcifications were also seen. Immunohistochemically, bcl-2 and CD34 staining was negative, so we diagnosed the mass as CFPT.