VOLUME 3 4
•
NUMBER 15
•
MAY 20, 2016
JOURNAL OF CLINICAL ONCOLOGY
D I A G N O S I S
O N C O L O G Y
primary germ cell tumor and atypical rhabdoid tumor. Computed tomography scanning of the chest, abdomen, and pelvis, MRI of the cervical, thoracic, and lumbar spine, and a bone marrow biopsy failed to demonstrate metastatic disease. While awaiting initiation of adjuvant therapy, the patient presented with progressive disease. She had experienced a rapid decline in her mental status and was found to have hydrocephalus, requiring bilateral ventriculoperitoneal shunt placement. MRI of the brain demonstrated a recurrent 3.3 ⫻ 2.9 ⫻ 5.8 – cm pineal mass (Fig 1C). The patient and her family wanted continued aggressive management, and a repeat craniotomy for additional tumor debulking was performed. Approximately 95% of the mass was resected. Unfortunately, the patient never regained consciousness and succumbed to her disease 1 month later. She survived only 4 months from the time of diagnosis and never recovered enough to be able to receive any adjuvant therapy.
Adult Onset Primary Pineal Rhabdomyosarcoma Introduction Rhabdomyosarcomas (RMSs) represent the largest group of sarcomas in children and are rarely diagnosed in adults.1,2 Less than 1% of all adult solid malignant cancers are sarcomas, and RMSs represent less than 0.001% of adult sarcomas.2 Although rare, when diagnosed in adults, RMS is an aggressive, rapidly recurring, often fatal disease.3 To our knowledge, there are only 21 previously documented cases of primary intracranial RMS in adults, and primary pineal gland RMS has never been reported.4 We report a case of adult pineal gland RMS along with a review of the literature. Case Report A 43-year-old, previously healthy Hispanic woman presented with a 2-week history of ataxia, diplopia, and headaches. Neurologic examination failed to show any focal deficit. Magnetic resonance imaging (MRI) of the brain showed a homogeneous, brightly enhancing, 3.4 cm ⫻ 2.1 cm ⫻ 2.4 – cm lobulated mass within the pineal gland (Fig 1A). Gross total resection with positive margins was performed in a piecemeal resection, as revealed on an MRI of the brain 3 days after surgery (Fig 1B). Pathology revealed a malignant pleomorphic neoplasm with spindle cell proliferation and many large polygonal tumor cells with rhabdomyoblastic differentiation, as seen on hematoxylin and eosin staining (Fig 2A, ⫻100 magnification; Fig 2B, ⫻400 magnification). Immunohistochemical staining showed that the tumor cells were positive for desmin (Fig 2C), muscle-specific actin (Fig 2D), INI1, and myogenin. They stained negative for pankeratin, chromogranin, CAM5.2, synaptophysin, and human placental alkaline phosphatase. The immunoprofile was diagnostic for primary RMS and excluded
A
I N
Discussion This is the first reported case of primary pineal gland RMS in an adult, to our knowledge. Two previous cases of pineal RMS arising from pineal teratomas have been reported, both in children.5,6 Pineal gland tumors are rare, representing less than 0.06% of all primary brain tumors.7 Primary sarcoma of the brain comprises 0.9% to 3.0% of all intracranial tumors, and primary intracranial RMS is even less common.8 The two previously documented cases of teratomas containing RMS were reported in 14and 17-year-old boys; the first underwent subtotal resection and radiation therapy and died within 1 year, whereas the second underwent gross total resection and died 4 days later.5,6 Diagnosis is dependent on immunohistochemistry and exclusion of extracranial disease with intracranial metastasis. This requires whole-body imaging to exclude any extracranial source. Dropcho and Allen9 proposed that pure intracranial RMSs are
B
C
Fig 1.
Journal of Clinical Oncology, Vol 34, No 15 (May 20), 2016: pp e137-e140
© 2014 by American Society of Clinical Oncology e137
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Scull et al
A
B
C
D
Fig 2.
actually neuroectodermal tumors in which neuronal or glial differentiation is missing. Immunohistochemistry diagnostic for RMS includes positive desmin and an absence of nonmesenchymal cell– type markers such as synaptophysin, cytokeratin, or neuronspecific enolase. This specific immunohistochemical pattern is consistent with the theory that RMS is derived from the undifferentiated ectomesenchymal cells along the neuroectoderm; however, the true origin of these tumors is still debated.9 In 1999, Hawkins et al10 proposed that primary CNS RMS arises from mesenchyma, given that some patients have shown response to chemotherapy directed at mesenchymal tissue. Table 1 summarizes the demographic characteristics, treatment, and outcome of the 22 reported patients with primary intracranial rhabdomyosarcoma in adults (including our patient). The patients were 18 to 68 years of age with no difference in sex distribution. The most common locations included frontal lobe,6 parietal lobe,4 and cerebellum.3,11-28 Treatments varied from supportive care, total resection, subtotal resection, radiation, and chemotherapy to multimodality combination therapy. Survival ranged from less than 1 month to 30 months after diagnosis, the longest known period of survival. Follow-up information was available for 19 of 22 patients, 11 of whom had at least one recurrence of tumor in the original location. Local recurrence has been shown to occur as early as 3 days after resection21 and is the most common cause of death, given that distant metastases are rare with primary intracranial RMS.20 Considering the paucity of literature, it is difficult to make standard recommendations about treatment e138 © 2014 by American Society of Clinical Oncology
or the prognosis of adults who are diagnosed with primary intracranial RMS. During the last 40 years, management and treatment of these tumors has changed significantly. Craniospinal irradiation and intrathecal methotrexate were at one time considered the optimal therapy.13,24 The therapy failed to show significant benefit. Current standard treatment consists of initial total resection of the tumor followed by whole-brain radiation and chemotherapy for 2 years.23 Most primary intracranial RMSs adhere to surrounding tissue, making a complete resection with negative margins impossible.29 Pineal region tumors provide an especially difficult surgical challenge in view of the location.29 Current adjuvant radiation is given at a dose similar to that for malignant gliomas, 50 to 60 Gy. The regimen most commonly used in children with RMS involves vincristine, dactinomycin, and cyclophosphamide, and related treatment data are extrapolated to adults.1 The longest surviving adult with primary intracranial RMS was a 46-year-old man who presented with RMS in a right frontoparietal site.23 He underwent a total resection followed by 60 Gy of whole-brain radiation (in 42 fractions), and 10 cycles of systemic chemotherapy with the CYVADIC regimen (cyclophosphamide, vincristine, doxorubicin, and dacarbazine), and was recurrence free at 30 months after diagnosis.23 Four patients survived beyond 1 year, two died at 15 months, one died at 20 months, and one was alive at 30 months (Table 1). These four patients had remarkably different treatments, with varying recurrences, leaving the question of how to best treat adult onset primary intracranial RMS unanswered. JOURNAL OF CLINICAL ONCOLOGY
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Diagnosis in Oncology
Table 1. Demographic Characteristics, Treatment, and Outcome of Reported Cases of Primary Intracranial Rhabdomyosarcoma in Adults First Author and Year
Age (years)
Sex
Site
Treatment
Survival (months)
Recurrence (months)
Lopez11 (1934) Dozic12 (1991) Smith13 (1981) Galatioto14 (1971) Polivka15 (1993) Arita16 (2001) Ozeki17 (1960) Linoli18 (1991) Grebe4 (2008) Mitsuhashi19 (2002) Palta20 (2011) Zhong21 (2007) Hawkins10 (1999) Leedham22 (1972) Celli23 (1998) Min24 (1975) Yagishita25 (1979) Pirillo26 (2011) Chiba27 (1981) Lopes de Faria28 (1957) Hayashi29 (1986) Current patient
18 24 25 26 26 28 29 38 40 42 44 44 44 45 46 48 51 51 51 52 68 43
F M M F M M M M F F M F M F M M F F F F M F
Cerebellopontine angle Lateral ventricle Meningeal Cerebellar Parietal Intrasellar Occipital Cerebellar Frontal Frontal Meningeal Intrasellar Frontotemporal Parietal Frontoparietal Frontal Right cerebrum Parietal Frontal Cerebellar Basal ganglia Pineal gland
None Resection Resection, chemotherapy None Resection Resection, chemotherapy, radiation None Resection Resection, radiation Resection, chemotherapy Resection, chemotherapy Resection Resection, radiation, chemotherapy Resection Resection, radiation, chemotherapy Resection, radiation, chemotherapy Resection Resection, radiation Resection, radiation, chemotherapy Died during angiography Radiation Resection
1
None Unknown None None Unknown 1.5 Unknown None 4 and 15 Unknown Unknown 1 and 2 14 9 None at30 4 and 6 9 11 and 15 8 None 1 3
Primary intracranial RMS usually follows a short and aggressive clinical course in adults. It is often rapidly progressive with local tumor recurrence and a 1-year overall survival rate of only 31.6%. Because of the rarity of the tumor, prospective randomized trials are not feasible; therefore, treatment and prognostic information has to be extrapolated from pediatric data. Every attempt should be made to diagnose the disease early and initiate aggressive multimodality therapy per pediatric protocols.
Chelsi Scull, Surabhi Amar, Iman Feiz-Erfan, Harikrishna Dave, and Daniel Gridley Maricopa Integrated Health System, Phoenix, AZ
ACKNOWLEDGMENT
Presented in part as a poster presentation for Academic Excellence Day, Maricopa Integrated Health System, Phoenix, AZ, May 1, 2013. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST
The author(s) indicated no potential conflicts of interest. REFERENCES 1. Wexler LH, Helman LJ: Pediatric soft tissue sarcomas. CA Cancer J Clin 44:211-247, 1994 2. Burningham Z, Hashibe M, Spector L, et al: The epidemiology of sarcoma. Clin Sarcoma Res 2:14, 2012 3. Guilcher GM, Hendson G, Goddard K, et al: Successful treatment of a child with a primary intracranial rhabdomyosarcoma with chemotherapy and radiation therapy. J Neurooncol 86:79-82, 2008 4. Grebe HP, Steube D: Primary cerebral rhabdomyosarcoma presenting as haemorrhagic stroke. Zentralbl Neurochir 69:93-95, 2008 5. Preissig SH, Smith MT, Huntington HW: Rhabdomyosarcoma arising in a pineal teratoma. Cancer 44:281-284, 1979 6. Glass RL, Culbertson CG: Teratoma of pineal gland with choriocarcinoma and rhabdomyosarcoma. Arch Pathol (Chic) 41:552-555, 1946 7. Al-Hussaini M, Sultan I, Abuirmileh N, et al: Pineal gland tumors: Experience from the SEER database. J Neurooncol 94:351-358, 2009 www.jco.org
Unknown 4 ⬍1 15 Alive at 5 1.5 Died postoperatively Alive at 15 Unknown Unknown 5 14 10 Alive at 30 8 Alive at 10 20 12 ⬍1 5 4
8. Masuzawa T, Shimabukuro H, Kamoshita S, et al: The ultrastructure of primary cerebral rhabdomyosarcoma. Acta Neuropathol 56:307-310, 1982 9. Dropcho EJ, Allen JC: Primary intracranial rhabdomyosarcoma: Case report and review of the literature. J Neurooncol 5:139-150, 1987 10. Hawkins C, Muller P, Bilbao JM: April 1999: 44 year old man with a bleeding intracerebral tumor. Brain Pathol 9:741-742, 1999 11. Lopez F: Rhabdomyosarcoma as primary tumor of the cerebellopontine angle [in German]. Z Gesamte Neurol Psychiatr 150:242-251, 1934 12. Dozic S, Cvetkovic D, Samardzic M, et al: Primary rhabdomyosarcoma of the lateral ventricle. Clin Neuropathol 10:245, 1991 13. Smith MT, Armbrustmacher VW, Violett TW: Diffuse meningeal rhabdomyosarcoma. Cancer 47:2081-2086, 1981 14. Galatioto S, Gaddoni G: Primary myosarcomas of the CNS [in Italian]. Acta Neurol (Napoli) 26:297-302, 1971 15. Polivka M, Lot G, Woimant F, et al: Report of a case of rhabdomyosarcoma of the central nervous system: Histologic, immunohistochemical and ultrastructural study [in French]. Ann Pathol 13:118-122, 1993 16. Arita K, Sugiyama K, Tominaga A, et al: Intrasellar rhabdomyosarcoma: Case report. Neurosurgery 48:677-680, 2001 17. Ozeki T: A necropsy case report of primary rhabdomyosarcoma in the cerebrum. Trans Soc Pathol Jpn 49:750, 1960 18. Linoli G: Primary rhabdomyosarcoma of the cerebellum: Histopathological and immunohistochemical study of an autopsy case [in Italian]. Pathologica 83:201-215, 1991 19. Mitsuhashi T, Mori K, Wada R, et al: Primary rhabdomyosarcoma associated with tumoral hemorrhage: Case report. Neurol Med Chir (Tokyo) 42:73-77, 2002 20. Palta M, Riedel RF, Vredenburgh JJ, et al: Primary meningeal rhabdomyosarcoma. Sarcoma 2011:312802, 2011 21. Zhong J, Li ST, Yao XH, et al: An intrasellar rhabdomyosarcoma misdiagnosed as pituitary adenoma. Surg Neurol 68:S29-S33, 2007 (suppl 2) 22. Leedham PW: Primary cerebral rhabdomyosarcoma and the problem of medullomyoblastoma. J Neurol Neurosurg Psychiatry 35:551-559, 1972 23. Celli P, Cervoni L, Maraglino C: Primary rhabdomyosarcoma of the brain: Observations on a case with clinical and radiological evidence of cure. J Neurooncol 36:259-267, 1998 24. Min K-W, Gyorkey F, Halpert B: Primary rhabdomyosarcoma of the cerebrum. Cancer 35:1405-1411, 1975 25. Yagishita S, Itoh Y, Chiba Y, et al: Primary rhabdomyosarcoma of the cerebrum: An ultrastructural study. Acta Neuropathol 45:111-115, 1979 © 2014 by American Society of Clinical Oncology e139
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Scull et al
26. Pirillo V, Cipriano Cecchi P, Tripodi M, et al: Primary cerebral alveolar rhabdomyosarcoma in adult. Rare Tumors 3:e26, 2011 27. Chiba Y, Fujino H, Yagishita S, et al: A case of primary cerebral rhabdomyosarcoma (author’s transl) [in Japanese]. No Shinkei Geka 9:1067-1072, 1981 28. Lopes de Faria J: Rhabdomyosarcoma of cerebellum. AMA Arch Pathol 63:234-238, 1957
29. Hayashi K, Ohtsuki Y, Ikehara I, et al: Primary rhabdomyosarcoma combined with chronic paragonimiasis in the cerebrum: A necropsy case and review of the literature. Acta Neuropathol 72:170-177, 1986
DOI: 10.1200/JCO.2013.50.8036; published online ahead of print at www.jco.org on April 21, 2014
■ ■ ■
e140 © 2014 by American Society of Clinical Oncology
JOURNAL OF CLINICAL ONCOLOGY
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
•
NUMBER 15
•
MAY 20, 2016
JOURNAL OF CLINICAL ONCOLOGY
D I A G N O S I S
O N C O L O G Y
primary germ cell tumor and atypical rhabdoid tumor. Computed tomography scanning of the chest, abdomen, and pelvis, MRI of the cervical, thoracic, and lumbar spine, and a bone marrow biopsy failed to demonstrate metastatic disease. While awaiting initiation of adjuvant therapy, the patient presented with progressive disease. She had experienced a rapid decline in her mental status and was found to have hydrocephalus, requiring bilateral ventriculoperitoneal shunt placement. MRI of the brain demonstrated a recurrent 3.3 ⫻ 2.9 ⫻ 5.8 – cm pineal mass (Fig 1C). The patient and her family wanted continued aggressive management, and a repeat craniotomy for additional tumor debulking was performed. Approximately 95% of the mass was resected. Unfortunately, the patient never regained consciousness and succumbed to her disease 1 month later. She survived only 4 months from the time of diagnosis and never recovered enough to be able to receive any adjuvant therapy.
Adult Onset Primary Pineal Rhabdomyosarcoma Introduction Rhabdomyosarcomas (RMSs) represent the largest group of sarcomas in children and are rarely diagnosed in adults.1,2 Less than 1% of all adult solid malignant cancers are sarcomas, and RMSs represent less than 0.001% of adult sarcomas.2 Although rare, when diagnosed in adults, RMS is an aggressive, rapidly recurring, often fatal disease.3 To our knowledge, there are only 21 previously documented cases of primary intracranial RMS in adults, and primary pineal gland RMS has never been reported.4 We report a case of adult pineal gland RMS along with a review of the literature. Case Report A 43-year-old, previously healthy Hispanic woman presented with a 2-week history of ataxia, diplopia, and headaches. Neurologic examination failed to show any focal deficit. Magnetic resonance imaging (MRI) of the brain showed a homogeneous, brightly enhancing, 3.4 cm ⫻ 2.1 cm ⫻ 2.4 – cm lobulated mass within the pineal gland (Fig 1A). Gross total resection with positive margins was performed in a piecemeal resection, as revealed on an MRI of the brain 3 days after surgery (Fig 1B). Pathology revealed a malignant pleomorphic neoplasm with spindle cell proliferation and many large polygonal tumor cells with rhabdomyoblastic differentiation, as seen on hematoxylin and eosin staining (Fig 2A, ⫻100 magnification; Fig 2B, ⫻400 magnification). Immunohistochemical staining showed that the tumor cells were positive for desmin (Fig 2C), muscle-specific actin (Fig 2D), INI1, and myogenin. They stained negative for pankeratin, chromogranin, CAM5.2, synaptophysin, and human placental alkaline phosphatase. The immunoprofile was diagnostic for primary RMS and excluded
A
I N
Discussion This is the first reported case of primary pineal gland RMS in an adult, to our knowledge. Two previous cases of pineal RMS arising from pineal teratomas have been reported, both in children.5,6 Pineal gland tumors are rare, representing less than 0.06% of all primary brain tumors.7 Primary sarcoma of the brain comprises 0.9% to 3.0% of all intracranial tumors, and primary intracranial RMS is even less common.8 The two previously documented cases of teratomas containing RMS were reported in 14and 17-year-old boys; the first underwent subtotal resection and radiation therapy and died within 1 year, whereas the second underwent gross total resection and died 4 days later.5,6 Diagnosis is dependent on immunohistochemistry and exclusion of extracranial disease with intracranial metastasis. This requires whole-body imaging to exclude any extracranial source. Dropcho and Allen9 proposed that pure intracranial RMSs are
B
C
Fig 1.
Journal of Clinical Oncology, Vol 34, No 15 (May 20), 2016: pp e137-e140
© 2014 by American Society of Clinical Oncology e137
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Scull et al
A
B
C
D
Fig 2.
actually neuroectodermal tumors in which neuronal or glial differentiation is missing. Immunohistochemistry diagnostic for RMS includes positive desmin and an absence of nonmesenchymal cell– type markers such as synaptophysin, cytokeratin, or neuronspecific enolase. This specific immunohistochemical pattern is consistent with the theory that RMS is derived from the undifferentiated ectomesenchymal cells along the neuroectoderm; however, the true origin of these tumors is still debated.9 In 1999, Hawkins et al10 proposed that primary CNS RMS arises from mesenchyma, given that some patients have shown response to chemotherapy directed at mesenchymal tissue. Table 1 summarizes the demographic characteristics, treatment, and outcome of the 22 reported patients with primary intracranial rhabdomyosarcoma in adults (including our patient). The patients were 18 to 68 years of age with no difference in sex distribution. The most common locations included frontal lobe,6 parietal lobe,4 and cerebellum.3,11-28 Treatments varied from supportive care, total resection, subtotal resection, radiation, and chemotherapy to multimodality combination therapy. Survival ranged from less than 1 month to 30 months after diagnosis, the longest known period of survival. Follow-up information was available for 19 of 22 patients, 11 of whom had at least one recurrence of tumor in the original location. Local recurrence has been shown to occur as early as 3 days after resection21 and is the most common cause of death, given that distant metastases are rare with primary intracranial RMS.20 Considering the paucity of literature, it is difficult to make standard recommendations about treatment e138 © 2014 by American Society of Clinical Oncology
or the prognosis of adults who are diagnosed with primary intracranial RMS. During the last 40 years, management and treatment of these tumors has changed significantly. Craniospinal irradiation and intrathecal methotrexate were at one time considered the optimal therapy.13,24 The therapy failed to show significant benefit. Current standard treatment consists of initial total resection of the tumor followed by whole-brain radiation and chemotherapy for 2 years.23 Most primary intracranial RMSs adhere to surrounding tissue, making a complete resection with negative margins impossible.29 Pineal region tumors provide an especially difficult surgical challenge in view of the location.29 Current adjuvant radiation is given at a dose similar to that for malignant gliomas, 50 to 60 Gy. The regimen most commonly used in children with RMS involves vincristine, dactinomycin, and cyclophosphamide, and related treatment data are extrapolated to adults.1 The longest surviving adult with primary intracranial RMS was a 46-year-old man who presented with RMS in a right frontoparietal site.23 He underwent a total resection followed by 60 Gy of whole-brain radiation (in 42 fractions), and 10 cycles of systemic chemotherapy with the CYVADIC regimen (cyclophosphamide, vincristine, doxorubicin, and dacarbazine), and was recurrence free at 30 months after diagnosis.23 Four patients survived beyond 1 year, two died at 15 months, one died at 20 months, and one was alive at 30 months (Table 1). These four patients had remarkably different treatments, with varying recurrences, leaving the question of how to best treat adult onset primary intracranial RMS unanswered. JOURNAL OF CLINICAL ONCOLOGY
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Diagnosis in Oncology
Table 1. Demographic Characteristics, Treatment, and Outcome of Reported Cases of Primary Intracranial Rhabdomyosarcoma in Adults First Author and Year
Age (years)
Sex
Site
Treatment
Survival (months)
Recurrence (months)
Lopez11 (1934) Dozic12 (1991) Smith13 (1981) Galatioto14 (1971) Polivka15 (1993) Arita16 (2001) Ozeki17 (1960) Linoli18 (1991) Grebe4 (2008) Mitsuhashi19 (2002) Palta20 (2011) Zhong21 (2007) Hawkins10 (1999) Leedham22 (1972) Celli23 (1998) Min24 (1975) Yagishita25 (1979) Pirillo26 (2011) Chiba27 (1981) Lopes de Faria28 (1957) Hayashi29 (1986) Current patient
18 24 25 26 26 28 29 38 40 42 44 44 44 45 46 48 51 51 51 52 68 43
F M M F M M M M F F M F M F M M F F F F M F
Cerebellopontine angle Lateral ventricle Meningeal Cerebellar Parietal Intrasellar Occipital Cerebellar Frontal Frontal Meningeal Intrasellar Frontotemporal Parietal Frontoparietal Frontal Right cerebrum Parietal Frontal Cerebellar Basal ganglia Pineal gland
None Resection Resection, chemotherapy None Resection Resection, chemotherapy, radiation None Resection Resection, radiation Resection, chemotherapy Resection, chemotherapy Resection Resection, radiation, chemotherapy Resection Resection, radiation, chemotherapy Resection, radiation, chemotherapy Resection Resection, radiation Resection, radiation, chemotherapy Died during angiography Radiation Resection
1
None Unknown None None Unknown 1.5 Unknown None 4 and 15 Unknown Unknown 1 and 2 14 9 None at30 4 and 6 9 11 and 15 8 None 1 3
Primary intracranial RMS usually follows a short and aggressive clinical course in adults. It is often rapidly progressive with local tumor recurrence and a 1-year overall survival rate of only 31.6%. Because of the rarity of the tumor, prospective randomized trials are not feasible; therefore, treatment and prognostic information has to be extrapolated from pediatric data. Every attempt should be made to diagnose the disease early and initiate aggressive multimodality therapy per pediatric protocols.
Chelsi Scull, Surabhi Amar, Iman Feiz-Erfan, Harikrishna Dave, and Daniel Gridley Maricopa Integrated Health System, Phoenix, AZ
ACKNOWLEDGMENT
Presented in part as a poster presentation for Academic Excellence Day, Maricopa Integrated Health System, Phoenix, AZ, May 1, 2013. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST
The author(s) indicated no potential conflicts of interest. REFERENCES 1. Wexler LH, Helman LJ: Pediatric soft tissue sarcomas. CA Cancer J Clin 44:211-247, 1994 2. Burningham Z, Hashibe M, Spector L, et al: The epidemiology of sarcoma. Clin Sarcoma Res 2:14, 2012 3. Guilcher GM, Hendson G, Goddard K, et al: Successful treatment of a child with a primary intracranial rhabdomyosarcoma with chemotherapy and radiation therapy. J Neurooncol 86:79-82, 2008 4. Grebe HP, Steube D: Primary cerebral rhabdomyosarcoma presenting as haemorrhagic stroke. Zentralbl Neurochir 69:93-95, 2008 5. Preissig SH, Smith MT, Huntington HW: Rhabdomyosarcoma arising in a pineal teratoma. Cancer 44:281-284, 1979 6. Glass RL, Culbertson CG: Teratoma of pineal gland with choriocarcinoma and rhabdomyosarcoma. Arch Pathol (Chic) 41:552-555, 1946 7. Al-Hussaini M, Sultan I, Abuirmileh N, et al: Pineal gland tumors: Experience from the SEER database. J Neurooncol 94:351-358, 2009 www.jco.org
Unknown 4 ⬍1 15 Alive at 5 1.5 Died postoperatively Alive at 15 Unknown Unknown 5 14 10 Alive at 30 8 Alive at 10 20 12 ⬍1 5 4
8. Masuzawa T, Shimabukuro H, Kamoshita S, et al: The ultrastructure of primary cerebral rhabdomyosarcoma. Acta Neuropathol 56:307-310, 1982 9. Dropcho EJ, Allen JC: Primary intracranial rhabdomyosarcoma: Case report and review of the literature. J Neurooncol 5:139-150, 1987 10. Hawkins C, Muller P, Bilbao JM: April 1999: 44 year old man with a bleeding intracerebral tumor. Brain Pathol 9:741-742, 1999 11. Lopez F: Rhabdomyosarcoma as primary tumor of the cerebellopontine angle [in German]. Z Gesamte Neurol Psychiatr 150:242-251, 1934 12. Dozic S, Cvetkovic D, Samardzic M, et al: Primary rhabdomyosarcoma of the lateral ventricle. Clin Neuropathol 10:245, 1991 13. Smith MT, Armbrustmacher VW, Violett TW: Diffuse meningeal rhabdomyosarcoma. Cancer 47:2081-2086, 1981 14. Galatioto S, Gaddoni G: Primary myosarcomas of the CNS [in Italian]. Acta Neurol (Napoli) 26:297-302, 1971 15. Polivka M, Lot G, Woimant F, et al: Report of a case of rhabdomyosarcoma of the central nervous system: Histologic, immunohistochemical and ultrastructural study [in French]. Ann Pathol 13:118-122, 1993 16. Arita K, Sugiyama K, Tominaga A, et al: Intrasellar rhabdomyosarcoma: Case report. Neurosurgery 48:677-680, 2001 17. Ozeki T: A necropsy case report of primary rhabdomyosarcoma in the cerebrum. Trans Soc Pathol Jpn 49:750, 1960 18. Linoli G: Primary rhabdomyosarcoma of the cerebellum: Histopathological and immunohistochemical study of an autopsy case [in Italian]. Pathologica 83:201-215, 1991 19. Mitsuhashi T, Mori K, Wada R, et al: Primary rhabdomyosarcoma associated with tumoral hemorrhage: Case report. Neurol Med Chir (Tokyo) 42:73-77, 2002 20. Palta M, Riedel RF, Vredenburgh JJ, et al: Primary meningeal rhabdomyosarcoma. Sarcoma 2011:312802, 2011 21. Zhong J, Li ST, Yao XH, et al: An intrasellar rhabdomyosarcoma misdiagnosed as pituitary adenoma. Surg Neurol 68:S29-S33, 2007 (suppl 2) 22. Leedham PW: Primary cerebral rhabdomyosarcoma and the problem of medullomyoblastoma. J Neurol Neurosurg Psychiatry 35:551-559, 1972 23. Celli P, Cervoni L, Maraglino C: Primary rhabdomyosarcoma of the brain: Observations on a case with clinical and radiological evidence of cure. J Neurooncol 36:259-267, 1998 24. Min K-W, Gyorkey F, Halpert B: Primary rhabdomyosarcoma of the cerebrum. Cancer 35:1405-1411, 1975 25. Yagishita S, Itoh Y, Chiba Y, et al: Primary rhabdomyosarcoma of the cerebrum: An ultrastructural study. Acta Neuropathol 45:111-115, 1979 © 2014 by American Society of Clinical Oncology e139
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
Scull et al
26. Pirillo V, Cipriano Cecchi P, Tripodi M, et al: Primary cerebral alveolar rhabdomyosarcoma in adult. Rare Tumors 3:e26, 2011 27. Chiba Y, Fujino H, Yagishita S, et al: A case of primary cerebral rhabdomyosarcoma (author’s transl) [in Japanese]. No Shinkei Geka 9:1067-1072, 1981 28. Lopes de Faria J: Rhabdomyosarcoma of cerebellum. AMA Arch Pathol 63:234-238, 1957
29. Hayashi K, Ohtsuki Y, Ikehara I, et al: Primary rhabdomyosarcoma combined with chronic paragonimiasis in the cerebrum: A necropsy case and review of the literature. Acta Neuropathol 72:170-177, 1986
DOI: 10.1200/JCO.2013.50.8036; published online ahead of print at www.jco.org on April 21, 2014
■ ■ ■
e140 © 2014 by American Society of Clinical Oncology
JOURNAL OF CLINICAL ONCOLOGY
Downloaded from jco.ascopubs.org on May 27, 2016. For personal use only. No other uses without permission. Copyright © 2016 American Society of Clinical Oncology. All rights reserved.
