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Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1.

Alina B, Sebastian JA, Gerardo C - Case Rep Oncol Med (2015)

Bottom Line: Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement.The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT).The patient was started on chemotherapy and radiation as described above.

View Article: PubMed Central - PubMed

Affiliation: Trinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USA ; Department of Internal Medicine, Trinitas Regional Medical Center, Elizabeth, NJ 67202, USA.

ABSTRACT
Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above.

No MeSH data available.


Related in: MedlinePlus

Arrow pointing to the soft tissue mass in the left lateral chest wall with its measurement.
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Related In: Results  -  Collection


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fig1: Arrow pointing to the soft tissue mass in the left lateral chest wall with its measurement.

Mentions: We describe the presentation of malignant triton tumors in two siblings who are familiar to our cancer center. The first patient is a 36-year-old female with past medical history of asthma who presented to us with complaints of left sided chest wall swelling and mild pain that progressed over a few days. She denied the history of smoking, alcohol, or drugs. Family history was significant for colon cancer in father and maternal aunt. She was not sexually active. On examination, she was found to have axillary freckling, café au lait spots, and mild cognitive impairment. The findings were consistent with NF-1. There was no associated hepatosplenomegaly noted on examination. On imaging, a very large soft tissue mass measuring 15 cm by 15 cm by 13 cm in the left lower anterior lateral chest wall with possible ulceration and axillary lymphadenopathy was seen. The mass infiltrated the adjacent chest wall musculature; however, the ribs and lungs were commented as being intact and clear, respectively (Figure 1). At the time, it was thought to be a possible soft tissue abscess. Thereafter, debridement of the left sided chest mass was undertaken. Due to the rarity of the histologic findings, our pathology department referred this case to Memorial Sloan Kettering for a second opinion. Their findings are quoted as “mainly monotonous undifferentiated spindle cells arranged in intersecting fascicles and vague storiform pattern with high mitotic activity.” Immunohistochemical (IHC) stains revealed tumor cells positive for desmin, vimentin, and myogenin as well as focal reactivity for S-100 (Figure 2). On the other hand, markers CD31, CK, EMA, and CD57 were reported to be negative. These results were, thus, consistent with the diagnosis of a high-grade malignant peripheral nerve sheath tumor with focal divergent skeletal muscle differentiation (triton tumor) in the background of fat and fibrous tissue. After discussion in the multidisciplinary round, we decided to give her adjuvant chemotherapy consisting of ifosfamide and adriamycin for four cycles along with pre- and postchemotherapy radiation. She was started on combination chemotherapy of ifosfamide 2 g/m2 with mesna 2 g/m2 alternating with adriamycin 75 g/m2 every other week for first two cycles four weeks apart and then continued with ifosfamide and mesna for the rest of the two cycles without adriamycin. Concomitant radiation was given after the 2nd cycle and continued till the 3rd chemotherapy cycle. The radiation therapy was applied to the left abdominal wall, with a total dose of 65.4 Gy, with initial photon fields, followed by shrinking fields with electrons with 33 dose fractions during the course of two months. She currently follows up in our cancer center and has no evidence of disease recurrence four years after her treatment.


Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1.

Alina B, Sebastian JA, Gerardo C - Case Rep Oncol Med (2015)

Arrow pointing to the soft tissue mass in the left lateral chest wall with its measurement.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4465693&req=5

fig1: Arrow pointing to the soft tissue mass in the left lateral chest wall with its measurement.
Mentions: We describe the presentation of malignant triton tumors in two siblings who are familiar to our cancer center. The first patient is a 36-year-old female with past medical history of asthma who presented to us with complaints of left sided chest wall swelling and mild pain that progressed over a few days. She denied the history of smoking, alcohol, or drugs. Family history was significant for colon cancer in father and maternal aunt. She was not sexually active. On examination, she was found to have axillary freckling, café au lait spots, and mild cognitive impairment. The findings were consistent with NF-1. There was no associated hepatosplenomegaly noted on examination. On imaging, a very large soft tissue mass measuring 15 cm by 15 cm by 13 cm in the left lower anterior lateral chest wall with possible ulceration and axillary lymphadenopathy was seen. The mass infiltrated the adjacent chest wall musculature; however, the ribs and lungs were commented as being intact and clear, respectively (Figure 1). At the time, it was thought to be a possible soft tissue abscess. Thereafter, debridement of the left sided chest mass was undertaken. Due to the rarity of the histologic findings, our pathology department referred this case to Memorial Sloan Kettering for a second opinion. Their findings are quoted as “mainly monotonous undifferentiated spindle cells arranged in intersecting fascicles and vague storiform pattern with high mitotic activity.” Immunohistochemical (IHC) stains revealed tumor cells positive for desmin, vimentin, and myogenin as well as focal reactivity for S-100 (Figure 2). On the other hand, markers CD31, CK, EMA, and CD57 were reported to be negative. These results were, thus, consistent with the diagnosis of a high-grade malignant peripheral nerve sheath tumor with focal divergent skeletal muscle differentiation (triton tumor) in the background of fat and fibrous tissue. After discussion in the multidisciplinary round, we decided to give her adjuvant chemotherapy consisting of ifosfamide and adriamycin for four cycles along with pre- and postchemotherapy radiation. She was started on combination chemotherapy of ifosfamide 2 g/m2 with mesna 2 g/m2 alternating with adriamycin 75 g/m2 every other week for first two cycles four weeks apart and then continued with ifosfamide and mesna for the rest of the two cycles without adriamycin. Concomitant radiation was given after the 2nd cycle and continued till the 3rd chemotherapy cycle. The radiation therapy was applied to the left abdominal wall, with a total dose of 65.4 Gy, with initial photon fields, followed by shrinking fields with electrons with 33 dose fractions during the course of two months. She currently follows up in our cancer center and has no evidence of disease recurrence four years after her treatment.

Bottom Line: Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement.The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT).The patient was started on chemotherapy and radiation as described above.

View Article: PubMed Central - PubMed

Affiliation: Trinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USA ; Department of Internal Medicine, Trinitas Regional Medical Center, Elizabeth, NJ 67202, USA.

ABSTRACT
Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above.

No MeSH data available.


Related in: MedlinePlus