A Case of Alveolar Rhabdomyosarcoma of the Ethmoid Sinus Invading the Orbit in an Adult

Moon, Hyun Seung; Kwon, Sang Won; Lee, Jong Hyeok

doi:10.3341/kjo.2006.20.1.70

Cited by 25 publications

(26 citation statements)

References 16 publications

(29 reference statements)

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“…It is a common soft-tissue malignancy in children and adolescents but infrequent in adults. 1 The orbit is the primary site in approximately 10% of children with RMS 2,4 , and in adults orbital involvement is very rare. 2 The histopathologic types of RMS include embryonal, alveolar, pleomorphic and botryoide.…”

Section: Discussionmentioning

confidence: 99%

“…1 The orbit is the primary site in approximately 10% of children with RMS 2,4 , and in adults orbital involvement is very rare. 2 The histopathologic types of RMS include embryonal, alveolar, pleomorphic and botryoide. Alveolar RMS is an aggressive subtype with a distinct histology, characterized by small round cells and with a poorer prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…The patient died after seven months secondary to metastasis to the spine. 2 Torres-Peña et al, described two adults with alveolar RMS, one patient had involvement of ethmoid sinus and right orbit; and the other patient presented tumor on the nasal cavity that infiltrates the orbit and the fronto-ethmoidal sinuses. Both patients died of systemic complications associated with the invasion.…”

Section: Discussionmentioning

confidence: 99%

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Orbital Rhabdomyosarcoma in an Adult, Case Report

2017

ARC Journal of Surgery

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Orbital Rhabdomyosarcoma in an Adult, Case Report

2017

ARC Journal of Surgery

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“…Moon et al, described a patient with alveolar RMS of the ethmoid sinus with extending to the orbit and cervical lymph node involvement, who was treated with surgery and chemoradiotherapy, but died after seven months because of metastasis that progressed to the spine [2]. Similarly, Torres-Peña et al, described two adults with alveolar RMS, one patient showed the involvement of ethmoid sinus and right orbit; in the other patient the mass was centered at the nasal cavity and infiltrated the orbit and the ethmoidal and frontal sinuses [3].…”

Section: Discussionmentioning

confidence: 99%

Adult Rhabdomyosarcoma of Ethmoid Sinus Recurring as an Orbital Mass

Eshraghi

Ameli

Anvari

2016

JCDR

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A 23-year-old man first presented to an outlying hospital with a 3-week history of anosmia and mild proptosis in his left eye. Computed tomography (CT), revealed an isodense and wellcircumscribed mass arising from the left ethmoid sinus . Erosions of the lamina papyracea was observed that was extending into the maxillary sinus and the left orbit while compressing the left medial rectus muscle. Extension to the crista was noted. Subsequent T2-weighted magnetic resonance imaging (MRI) showed a mass in the left ethmoid sinus . Alveolar rhabdomyosarcoma (RMS) was diagnosed following endoscopic biopsy by an ENT specialist, and immunohistochemistry of the mass. The patient underwent chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamaide plus etoposide (VEC+IE), followed by 25 fractions of radiotherapy.Maintenance chemotherapy achieved one-year remission but eventually new symptoms emerged. He was referred to our ophthalmology department. A reddish medial canthal mass was present in the left eye with periorbital puffiness, injection of the nasal conjunctiva and diffuse punctate epithelial erosions. The mass in the medial orbital wall was relatively large (30 mm in diameter), palpable, and non-mobile. The globe was displaced laterally. There was slight left gaze diplopia and limitation of abduction and adduction. Other ocular examinations were normal and his corrected visual acuity was 20/20 OU. Spiral axial and coronal CT of the orbit [Table /Fig-1c,d] revealed an extraconal mass in the medial orbit of the left eye, extending posteriorly, causing dystopia of the globe, and compressing the medial rectus muscle. Remarkably, the ethmoid and maxillary sinuses were clear. An extensive preoperative metastatic workup was negative. Inferior transconjunctival orbitotomy was performed to obtain an incisional biopsy. A whitish avascular tumour was found. Immunohistochemical staining for actin and myogenin was positive [Table/ Fig-2]. Round myoblasts with abundant eosinophilic cytoplasm were identified. With a diagnosis of recurrence of alveolar RMS, the patient received his second round of chemoradiotherapy. At the 3-month follow-up visit, the mass was reduced in size by 80% and disappeared completely in the weeks following the treatment. However, he had developed dry eye Alveolar rhabdomyosarcoma (RMS) is a primitive, malignant, round cell neoplasm derived from mesenchymal tissue that exhibits partial skeletal muscle differentiation. We describe a rare case of alveolar RMS of ethmoid sinus, recurring as an orbital mass. A 23-year-old man with the chief complaint of anosmia and mild proptosis was diagnosed with RMS of the left ethmoid sinus and orbit following an endoscopic biopsy of the mass. He was treated with chemotherapy and radiotherapy. At 12 months after diagnosis, while still on maintenance chemotherapy, he presented to our eye hospital with a large medial canthal mass and lateral globe displacement. Orbital computed tomography revealed an extraconal mass in the medial orbit of the ...

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“…While some cases of alveolar RMS have been described in young adults, the occurrence of this neoplasm in adults over the age of 45 years is extremely uncommon, being pleomorphic RMS, characterized by different microscopic features and biological behavior, the most common histotype in this category of patients [8][9][10]16]. For this reason, the inclusion of alveolar RMS in the differential diagnosis of small round cell tumors of the head and neck region is often neglected.…”

Section: Discussionmentioning

confidence: 99%

Alveolar Rhabdomyosarcoma of the Paranasal Cavity in an Adult Woman

Covello

2014

J Med Cases

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Alveolar rhabdomyosarcoma (RMS) is extremely rare in adults older than 45 years and may be very difficult to distinguish from other primitive round cell neoplasms without immunohistochemistry and/or genetic studies. In adults, the differential diagnosis of small round cell tumors of the head and neck region includes small cell carcinoma, lymphomas and neuroepithelial tumors, such as esthesioneuroblastoma and, as last choice, rhabdomyosarcoma. Initial immunoprofiling may not include myogenic markers. Moreover, aberrant expression of epithelial and neuroendocrine markers in alveolar RMS may be a potentially serious diagnostic pitfall. In this report, we describe the clinico-pathological features of a case of paranasal alveolar RMS in a 61-year-old woman with no previous history of neoplasia and discuss the differential diagnosis of small round cell tumor in the nasal/paranasal cavity.

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A Case of Alveolar Rhabdomyosarcoma of the Ethmoid Sinus Invading the Orbit in an Adult

Cited by 25 publications

References 16 publications

Orbital Rhabdomyosarcoma in an Adult, Case Report

Orbital Rhabdomyosarcoma in an Adult, Case Report

Adult Rhabdomyosarcoma of Ethmoid Sinus Recurring as an Orbital Mass

Alveolar Rhabdomyosarcoma of the Paranasal Cavity in an Adult Woman

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