Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma

Jani, Prashant; Charles, Catney

doi:10.1007/s12098-008-0230-3

Cited by 7 publications

(7 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The natural history of primary alveolar RMS of bone may show individual variations, but our current cases, together with the other four reported ones [ 7 – 10 ], suggest a better overall prognosis as compared to soft tissue ARMS (Table 1 ). Primary alveolar rhabdomyosarcoma of the bone as a subtype of ARMS seems to be a distinct clinico-pathological entity.…”

Section: Discussionsupporting

confidence: 45%

See 1 more Smart Citation

Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature

et al. 2016

View full text Add to dashboard Cite

BackgroundRhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities.Case presentationHere, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma). Biopsies, proved a massive infiltration of the bone marrow cavity with rhabdomyosarcoma. In both cases, the ARMS subtype was confirmed using FOXO1 break-apart probes (FISH). Radiological examination could not identify primary soft tissue component in any localization at the time of diagnosis in either cases.ConclusionsPrimary alveolar rhabdomyosarcoma of the bone as a subtype of ARMS, seems to be a distinct clinico-pathological entity with challenging diagnostic difficulties and different, yet better, biological behavior in comparison to soft tissue ARMS. However, it is difficult to be characterized or predict its prognosis and long-term survival as only sporadic cases (four) were reported so far.

show abstract

Section: Discussionsupporting

confidence: 45%

“…Available data about primary bone ARMS is limited due to the fact that so far only four cases were found in literature reporting fusion-positive alveolar RMS confined to the bone marrow [ 7 – 10 ]. Thus, it is difficult to predict the disease course, the biological behavior and its characteristics.…”

Section: Introductionmentioning

confidence: 99%

Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature

et al. 2016

View full text Add to dashboard Cite

show abstract

“…The poor prognosis of this group of patients is confirmed by a review of the medical literature. We found 16 publications (from 1976 to the present) describing 22 cases of children with RMS and an unknown primary tumor site 3–18 . The characteristics of these patients are summarized in Table 2.…”

Section: Discussionmentioning

confidence: 99%

“…In a few cases, patients present with disseminated disease, but no evidence of a primary tumor mass, sometimes mimicking other cancers such as acute lymphoblastic leukemia. These cases of RMS with unknown primary tumor site have rarely been investigated, and only a few series and case reports are available in the literature 3–18 . A pooled North American–European analysis on metastatic RMS patients included 12 patients with no evident primary tumor site, whose 3‐year event‐free survival (EFS) was reportedly 8% 2 …”

Section: Introductionmentioning

confidence: 99%

“…These cases of RMS with unknown primary tumor site have rarely been investigated, and only a few series and case reports are available in the literature. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] A pooled North American-European analysis on metastatic RMS patients included 12 patients with no evident primary tumor site, whose 3-year event-free survival (EFS) was reportedly 8%. 2 To contribute further information on this rare condition, we report a series of patients with metastatic RMS with unknown primary tumor site registered in the Metastatic (MTS) 2008 protocol coordinated by the European pediatric Soft tissue sarcoma Study Group (EpSSG).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Rhabdomyosarcoma with unknown primary tumor site: A report from European pediatric Soft tissue sarcoma Study Group (EpSSG)

Affinita

Merks

Chisholm

et al. 2022

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Background Rhabdomyosarcoma (RMS) is an aggressive malignancy, and 20% of children present with metastases at diagnosis. Patients presenting with disseminated disease very occasionally have no clear evidence of a primary tumor mass. As these patients have rarely been investigated, we report on a series of patients with RMS and unknown primary tumor site registered in the Metastatic (MTS) RMS 2008 protocol (October 2008 to December 2016) coordinated by the European pediatric Soft tissue sarcoma Study Group. Methods Patients were administered nine cycles of induction chemotherapy, and 48 weeks of maintenance chemotherapy. Surgery and/or radiotherapy were planned after the first assessment of tumor response, and implemented after six cycles of chemotherapy. If feasible, radiotherapy to all sites of metastasis was recommended. Results We identified 10 patients with RMS and unknown primary site, most of them adolescents (median age 15.8 years, range: 4.6–20.4). Nine had fusion‐positive alveolar RMS. Multiple organ involvement was identified in seven patients, two only had bone marrow disease, and one only had leptomeningeal dissemination. All patients were given chemotherapy, four were irradiated, and none had surgery. Three patients underwent allogeneic bone marrow transplantation. At the time of this analysis, only two patients are alive in complete remission: one had received radiotherapy; and one had a bone marrow transplant. Conclusions RMS with unknown primary tumor occurs mainly in adolescents and is typically fusion‐positive alveolar. Radiotherapy may be important, but survival is poor and patients should be offered enrollment in investigational trials.

show abstract

Cranial metastatic alveolar rhabdomyosarcoma mimicking hematological malignancy in an adolescent boy

et al. 2014

View full text Add to dashboard Cite

Background Widespread alveolar rhabdomyosarcoma (ARMS) with bone marrow involvement and with an unknown primary tumor, especially presenting with acute tumor lysis syndrome can be easily misdiagnosed as a hematological malignancy. Furthermore, brain metastasis of ARMS is rare seen in children. Case report Herein, we report a 14-year-old boy presenting with acute tumor lysis syndrome due to bone marrow invasion of ARMS, who was diagnosed after abdominal paraaortic lymph node biopsy. Despite radiological and nuclear medicine imaging, the primary tumor site could not be found. He was treated with vincristine, topotecan, and cyclophosphamide for 42 weeks. Six months after the completion of treatment, he suffered from severe headache, blurred vision, right hemiplegia, and severe bone pain. Cranial magnetic resonance imaging showed multiple hemorrhagic infarctions. Brain biopsy showed brain metastasis with PAX3-FKHR fusion transcript. Conclusion The clinicians must be vigilant about solely brain metastasis in ARMS without additional metastasis.

show abstract

Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma

Cited by 7 publications

References 12 publications

Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature

Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature

Rhabdomyosarcoma with unknown primary tumor site: A report from European pediatric Soft tissue sarcoma Study Group (EpSSG)

Cranial metastatic alveolar rhabdomyosarcoma mimicking hematological malignancy in an adolescent boy

Contact Info

Product

Resources

About