Chondromyxoid fibroma of skull base: a tumour prone to local recurrence

Abstract

Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of followup. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.

Chondromyxoid fibroma of the skull base: Differential diagnosis and radiotherapy. Two case reports and a review of the literature

“…Many investigators have advocated that surgery should be performed according to an ''en bloc'' procedure, but this approach is associated with considerably increased morbidity [1,10,12,16,17]. Curettage alone is not recommended because of the probability of leaving small tumour lobules in the spongiosa in the vicinity of the tumour mass that would contribute to recurrence [16].…”

“…Shek et al described an uncommon case of skull base chondromyxoid fibroma in a teenage girl with multiple recurrences over a 10-year period after the initial operation [17]. In another study, a thoracic spine chondromyxoid fibroma recurrence was described 30 years after initial curettage [19].…”

“…English language publications are rare, and are limited in terms of the number of cases and followup. Among 44 cases of skull chondromyxoid fibroma (Table I), Inoue and Shek reported only two cases, one of which was irradiated, followed for 10 years [17,20].…”

“…The authors consequently concluded that radiation therapy is not indicated except for very rare surgically inaccessible lesions [1,2,12]. However, some authors have used radiotherapy for local relapses following surgical excision or for unresectable tumour without malignant transformation with follow-ups ranging from 11 months to 10 years [1,8,17]. The definition for so-called radiation-induced tumours as proposed by Cahan et al includes the following criteria: it arises in the zone of irradiation, at least 5 years after irradiation, with a different histological type from that of the initial tumour [24,25].…”

Fibroblast Phenotype Plasticity: Relevance for Understanding Heterogeneity in “Fibroblastic” Tumors

“…or low levels of aSMA in the course of our routine diagnostic work, including, for example, otherwise unambiguously non-smooth-muscle tumors such as chondromyxoid fibroma [54][55][56], chondroblastoma [57], osteosarcoma [58,59], and rhabdomyosarcoma [60]. Table 2 reveals a wide range of cells expressing aSMA or myofilaments, but also shows a few examples of nonmyofibroblastic cells also expressing fibronexuses-for example, endothelium, in vitro cultured fibroblasts, some rare variants of epithelioid sarcoma, and spindle-cell carcinoma.…”