Limits...
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 1: Computed tomography scan showed a mass with homogeneous destructive pattern that affected the paranasal sinuses and extended into the right orbit.
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC4494484&req=5

Figure 2: Case 1: Computed tomography scan showed a mass with homogeneous destructive pattern that affected the paranasal sinuses and extended into the right orbit.

Mentions: A 24-year-old male patient with a 2-month history of epistaxis, nasal stuffiness, frontal headache, and decrease of sensitivity in the right side of the face complained of a decrease in visual acuity and proptosis of the right eye. Preauricular, submandibular, and laterocervical lymphadenopathies were noticed. Computed tomography scan showed a mass with a homogeneous destructive pattern in the right nasal cavity that affected the ethmoid sinus and right orbit with bone destruction (Fig. 1). A biopsy showed a mass composed of polygonal to round cells with hyperchromatic nuclei and variably eosinophilic cytoplasm (Fig. 2). The cells were arranged in cohesive clusters or noncohesive aggregates, showing an alveolar pattern. A solid pattern of mononuclear cells without alveolar characteristics was focally present. Some degree of pleomorphism was noted, but it was more apparent in the loosely cellular areas where giant tumor cells were evident. The immunohistochemical study was positive for desmin, vimentin, and myosin fast, and negative for cytokeratins AE1 and AE3, smooth muscle actin 1A4, common muscle actin HHF-35, S-100 protein, CD34 antigen, and myoglobin. The diagnosis of RMS was made. Extension study was negative. The pretreatment classification was III (TNM-UICC). The treatment was initiated with chemotherapy and radiotherapy, to the right middle face. The mass recurred 3 times and new chemotherapies were performed, but the disease continued in progression. Two years later, the patient died 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 1: Computed tomography scan showed a mass with homogeneous destructive pattern that affected the paranasal sinuses and extended into the right orbit.
© Copyright Policy
Related In: Results  -  Collection

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

Figure 2: Case 1: Computed tomography scan showed a mass with homogeneous destructive pattern that affected the paranasal sinuses and extended into the right orbit.
Mentions: A 24-year-old male patient with a 2-month history of epistaxis, nasal stuffiness, frontal headache, and decrease of sensitivity in the right side of the face complained of a decrease in visual acuity and proptosis of the right eye. Preauricular, submandibular, and laterocervical lymphadenopathies were noticed. Computed tomography scan showed a mass with a homogeneous destructive pattern in the right nasal cavity that affected the ethmoid sinus and right orbit with bone destruction (Fig. 1). A biopsy showed a mass composed of polygonal to round cells with hyperchromatic nuclei and variably eosinophilic cytoplasm (Fig. 2). The cells were arranged in cohesive clusters or noncohesive aggregates, showing an alveolar pattern. A solid pattern of mononuclear cells without alveolar characteristics was focally present. Some degree of pleomorphism was noted, but it was more apparent in the loosely cellular areas where giant tumor cells were evident. The immunohistochemical study was positive for desmin, vimentin, and myosin fast, and negative for cytokeratins AE1 and AE3, smooth muscle actin 1A4, common muscle actin HHF-35, S-100 protein, CD34 antigen, and myoglobin. The diagnosis of RMS was made. Extension study was negative. The pretreatment classification was III (TNM-UICC). The treatment was initiated with chemotherapy and radiotherapy, to the right middle face. The mass recurred 3 times and new chemotherapies were performed, but the disease continued in progression. Two years later, the patient died 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