Gastric Metastasis of an Extremity Myxofibrosarcoma: A Case Report
Carolina Paredes-Molina, Mazin Al-Kasspooles, John Ashcraft, Joseph Valentino and Peter DiPasco*
Department of General Surgery, The University of Kansas School of Medicine, Kansas City, Kansas, USA
Received date: 10 Jul 2017; Accepted date: 18 Sep 2017; Published
date: 25 Sep 2017.
*Corresponding author: Peter DiPasco, Department of General Surgery,
The University of Kansas School of Medicine, Kansas City, Kansas, USA,
Tel: 9135886065; E-mail: firstname.lastname@example.org
Keywords: Myxofibrosarcoma; Chemotherapy; Soft tissue sarcomas.
Soft tissue sarcomas are rare soft tissue tumors that account for 1%
of all malignancies. The most common primary site for this malignancy
is an extremity, and metastasis most commonly involves the lungs.
Morphologically, myxofibrosarcomas have both fibroblastic and histiocytic
features and are one of the most aggressive tumors of the sarcoma
family. Moreover, because of their ordinary gross appearance, they can
be easily misdiagnosed as benign soft tissue tumors . The average age
at diagnosis ranges from sixth to eighth decade with median age being
approximately 65 years old [2-4].Prognostic factors include size, grade,
location and histologic subtype, with the foremost two determinants most
influential on overall outcome. Lastly, necrosis is also an important factor
for tumor grading . In this report, we present our experience with this
rare extremity tumor metastasizing to the stomach and manifesting as a
gastrointestinal bleed-representing the first occurrence thus described in
the medical literature.
Our patient is a 73-year-old male who initially presented with a
soft tissue tumor involving the posterior aspect of the left thigh. The
patient had noted the mass growing over several months. Clinically and
radiographically, this raised concerns for a soft tissue sarcoma. CT of the
chest showed no distant metastasis. The patient was offered the option to
undergo radical en bloc surgical resection. He underwent this procedure a
few days after and a mass with a size of 12cm x 10cm x 10cm was removed
Histologic evaluation showed high grade sarcoma with myxoid
features, favoring high grade myxofibrosarcoma with histologic grade
3(French Federation of Cancer Centers Sarcoma Group) Moreover,
immunohistochemistry was negative for CAM 5.2, S-100, desmin,
calponin, myogenin and CD31. Non-specific staining is noted with CD34.
The MIB1/Ki67 proliferative index was markedly elevated (greater than
60%) within the high-grade range. Pathologic staging was pT2bNX.
Following surgical resection, the patient received radiation therapy
to the limb which was well tolerated. After a lengthy discussion of risks,
survival, and benefits of adjuvant chemotherapy, the patient elected
against this therapy and chose surveillance alone. Regular 3-4 month
surveillance CT scans were reviewed. Two years later a new 1.1cm
pulmonary nodule consistent with metastatic disease was found. Our
patient underwent radiation therapy with successful response. Another CT
scan the following year showed a pulmonary nodule in the contralateral
side and the patient underwent radiation once again with successful
response. One month later, the patient presented to an outside facility
with anemia and extreme fatigue. Lower and upper endoscopies were
performed and a gastric mass was identified. The patient was transferred
to our facility. During his hospitalization, the patient required multiple
transfusions, esophagogastroduodenoscopy revealed a 6-7 cm nonbleeding
gastric mass. The patient underwent an exploratory laparotomy,
esophagogastroduodenoscopy, and partial gastrectomy, completely
excising the mass (Figures 1 and 2). Pathology showed metastatic highgrade
myxofibrosarcoma which was compared to the previous surgical
pathology of the thigh. The thigh tissue morphologically resembled the
gastric tissue. Immunohistochemical stains for Pan-CK, CK7 and CDX-2
performed were negative which supported the diagnosis. Following results
and further studies, a CT scan showed a new lung nodule and a MRI
showed possible recurrence of the left thigh lesion. The case was discussed
at multidisciplinary tumor board meetings and was recommended to
undergo radiation therapy for the new lung metastasis and surgical biopsy
of potential recurrence on the left thigh. He continues to receive care at
our institution while carrying a relatively normal lifestyle and playing golf
on a regular basis.
Figure 1: Gross image of stomach mass after complete resection
Figure 2: Gross image of stomach mass, sectioned
Myxofibrosarcomas arise in the extremities and metastasize to
lungs and bone. Sarcomas arising from the gastrointestinal system
most commonly metastasize to the liver and peritoneal surfaces. This
patient presented with a unique case in which the myxofibrosarcoma
metastasized to the alimentary tract and presented as a new entity with
anemia and gastrointestinal bleed three years after treatment for the
initial primary extremity tumor. Sanfilippo et al., performed a large
retrospective study to understand the prognostic factors and survival
of patients with myxofibrosarcomas. This study concluded that these
tumors have a slight male predominance, most metastasized to the lungs
at a rate of 14.6%, while median time for development of metastasis was
11 months. The most important prognostic factors are size and grade
which dictate treatment . NCCN guidelines indicate that patients
with local disease of the extremities, superficial trunk, head, and neck
should undergo En bloc surgical resection, which is a large bulky tumor
resection sometimes involving surrounding structures and lymph nodes,
and consider radiation therapy with or without adjuvant chemotherapy.
For metastatic disease, if single organ, options abound including several
permutations of neoadjuvant versus adjuvant chemotherapy as well as
radiation alike. If disseminated metastasis is present, options include
palliative chemotherapy with or without an admixture of radiation,
surgery, observation, or supportive care .
The choice to use neoadjuvant chemotherapy for soft tissue sarcomas is
usually made by a multidisciplinary team of experts. Anthracycline-based
regimens are preferred for the neoadjuvant setting. There are few studies
showing potential benefits of this treatment with slight increases of 5-year
survival rates [7,8].
We reviewed the literature and found case reports of metastasis to the
lung, lymph nodes, brain, bone, mediastinum, mesentery, pancreas, liver,
and colon [4,9-13]. We also found that the different literature reviews and
clinicopathological analysis suggest that tumors with necrosis or larger
than 5cm have significantly higher rates of metastatic relapse [5,14].
Myxofibrosarcomas are rare tumors which show low and high grade
histology. High grade malignancies are associated with higher rates
of metastasis mainly to lungs and lymph nodes . Metastasis to the
gastrointestinal tract is rare and, to our knowledge, this is the only report of
a metastasis to the stomach presenting with anemia from a gastrointestinal
bleed. Thorough surveillance for recurrence and metastasis is imperative
for the best prognosis of patients with myxofibrosarcomas, as well as key
recognition of rare cases like the one presented in this manuscript to avoid
a delayed diagnosis of metastasis once they present.
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