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Nasal Cavity or Alveolar Paranasal Sinus Rhabdomyosarcoma with Orbital Extension in Adults: 2 Cases.

Torres-Peña JL, Ramos Castrillo AI, Mencía-Gutiérrez E, Gutiérrez-Díaz E, Rodríguez-Peralto JL, Bengoa-González Á - Plast Reconstr Surg Glob Open (2015)

Bottom Line: These patients were evaluated with radiological studies.Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy.Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, 12 de Octubre Hospital, Complutense University, Madrid, Spain; Department of Ophthalmology, La Paz Hospital, Autónoma University, Madrid, Spain; and Department of Pathology, 12 de Octubre Hospital, Complutense University, Madrid, Spain.

ABSTRACT
Alveolar rhabdomyosarcoma (RMS) has a predilection for the deep soft tissues of the extremities and mainly occurs in children. Although the tumor may originate in other sites, such as the nasal cavity or paranasal sinus, invasion of the orbit is unusual. We describe the clinicopathological features of 2 cases of alveolar RMS of the nasal cavity or paranasal sinus in adult patients with orbital extension. These cases of alveolar RMS of the nasal cavity or paranasal sinuses are described in 2 men, both in the third decade of life. These patients were evaluated with radiological studies. The histological diagnosis was confirmed by immunohistochemical methods. Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy. Alveolar subtype RMS is an extremely aggressive neoplasm that rarely presents in the orbit or paranasal sinuses of adults but should be considered in the differential diagnosis of tumors with this localization. Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

No MeSH data available.


Related in: MedlinePlus

Case 2: Magnetic resonance imaging showing a large mass in the right sinuses with orbital invasion and extension into anterior cranial fossa.
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Figure 3: Case 2: Magnetic resonance imaging showing a large mass in the right sinuses with orbital invasion and extension into anterior cranial fossa.

Mentions: A 26-year-old man with a 3-month history of nasal stuffiness and epistaxis also complained of orbital pain and decrease of visual acuity in the right eye. The physical examination discovered 2 submandibular lymph nodes of 2 cm in diameter, severe proptosis, inferior and lateral displacement of the eyeball, and complete ophthalmoplegia. The right fundus showed optic nerve avulsion. The magnetic resonance imaging documented a large mass of the right nasal cavity with infiltration into the orbit, ethmoidal and frontal sinuses. The extraconal component of the mass showed extradural extension into the anterior cranial fossa. However, there were no intraparenchymal cerebral lesions (Fig. 3). The pretreatment classification was III (TNM-UICC). A biopsy of the neoplasm in the nasal cavity was performed. Grossly, the tumor tissue showed a pale red soft-tissue tumor. Hematoxylin and eosin–stained section demonstrated a tumor composed of solid sheets of small, round cells with scant cytoplasm and hyperchromatic nuclei. The centers of the nests were solid and filled with polygonal cells with more abundant eosinophilic cytoplasm. Immunohistochemical studies showed that the tumor cells were negative to cytokeratins and S-100 protein, and they were positive for markers of skeletal muscle differentiation, desmin (Fig. 4), vimentin, and antibodies against Myo D1, which are highly specific and sensitive for RMS. The diagnosis of RMS was made. The extension study was negative. The treatment was initiated with chemotherapy and radiotherapy to the right middle face. The patient died 5 months later due to systemic complications associated with the invasion.


Nasal Cavity or Alveolar Paranasal Sinus Rhabdomyosarcoma with Orbital Extension in Adults: 2 Cases.

Torres-Peña JL, Ramos Castrillo AI, Mencía-Gutiérrez E, Gutiérrez-Díaz E, Rodríguez-Peralto JL, Bengoa-González Á - Plast Reconstr Surg Glob Open (2015)

Case 2: Magnetic resonance imaging showing a large mass in the right sinuses with orbital invasion and extension into anterior cranial fossa.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4494484&req=5

Figure 3: Case 2: Magnetic resonance imaging showing a large mass in the right sinuses with orbital invasion and extension into anterior cranial fossa.
Mentions: A 26-year-old man with a 3-month history of nasal stuffiness and epistaxis also complained of orbital pain and decrease of visual acuity in the right eye. The physical examination discovered 2 submandibular lymph nodes of 2 cm in diameter, severe proptosis, inferior and lateral displacement of the eyeball, and complete ophthalmoplegia. The right fundus showed optic nerve avulsion. The magnetic resonance imaging documented a large mass of the right nasal cavity with infiltration into the orbit, ethmoidal and frontal sinuses. The extraconal component of the mass showed extradural extension into the anterior cranial fossa. However, there were no intraparenchymal cerebral lesions (Fig. 3). The pretreatment classification was III (TNM-UICC). A biopsy of the neoplasm in the nasal cavity was performed. Grossly, the tumor tissue showed a pale red soft-tissue tumor. Hematoxylin and eosin–stained section demonstrated a tumor composed of solid sheets of small, round cells with scant cytoplasm and hyperchromatic nuclei. The centers of the nests were solid and filled with polygonal cells with more abundant eosinophilic cytoplasm. Immunohistochemical studies showed that the tumor cells were negative to cytokeratins and S-100 protein, and they were positive for markers of skeletal muscle differentiation, desmin (Fig. 4), vimentin, and antibodies against Myo D1, which are highly specific and sensitive for RMS. The diagnosis of RMS was made. The extension study was negative. The treatment was initiated with chemotherapy and radiotherapy to the right middle face. The patient died 5 months later due to systemic complications associated with the invasion.

Bottom Line: These patients were evaluated with radiological studies.Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy.Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, 12 de Octubre Hospital, Complutense University, Madrid, Spain; Department of Ophthalmology, La Paz Hospital, Autónoma University, Madrid, Spain; and Department of Pathology, 12 de Octubre Hospital, Complutense University, Madrid, Spain.

ABSTRACT
Alveolar rhabdomyosarcoma (RMS) has a predilection for the deep soft tissues of the extremities and mainly occurs in children. Although the tumor may originate in other sites, such as the nasal cavity or paranasal sinus, invasion of the orbit is unusual. We describe the clinicopathological features of 2 cases of alveolar RMS of the nasal cavity or paranasal sinus in adult patients with orbital extension. These cases of alveolar RMS of the nasal cavity or paranasal sinuses are described in 2 men, both in the third decade of life. These patients were evaluated with radiological studies. The histological diagnosis was confirmed by immunohistochemical methods. Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy. Alveolar subtype RMS is an extremely aggressive neoplasm that rarely presents in the orbit or paranasal sinuses of adults but should be considered in the differential diagnosis of tumors with this localization. Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

No MeSH data available.


Related in: MedlinePlus