Keywords
dermatofibrosarcoma - dermatofibroma - fibrous histiocytoma - tongue neoplasm
Introduction
Fibrous histiocytomas can be classified as benign and malignant. Benign fibrous histiocytomas
(BFHs) can be further sub-divided by tissue of origin, either dermal or deep (subcutaneous).
Cutaneous benign fibrous histiocytoma, or dermatofibroma, is localized to the dermis
and characterized by an assortment of spindle and/or rounded cells.[1] It is a common lesion seen in many age groups, with predominance in the 3rd and
4th decades of life, and gender distribution varying by population.[1] The majority of lesions are found on the extremities, with lesions rarely arising
on the face.[1] Clinically, BFHs present as single, round lesions, appearing reddish early on, and
transitioning to more brown or skin-colored with time. They are moderately well circumscribed,
and produce the characteristic “dimpling” sign when squeezed between the examiners
fingers.[1] Benign fibrous histiocytomas have a variable immunohistochemical profile and multiple
histologic subtypes: aneurysmal, epithelioid, cellular, angiomatoid, etc. These lesions
are considered benign, and tend to recur only with incomplete excision.[1] Deep BFHs generally involve subcutaneous tissue, and affects adults over 25 years,
with a mean age of 40 years.[2]
[3] Similar to BFHs, the majority of deep BFHs are on the extremities, but may also
occur in the head and neck region.[2] Clinically, deep BFH lesions are seen as painless, slowly enlarging masses, and
are more well circumscribed/encapsulated than the cutaneous form.[2] Metastasis has yet to be reported.
Malignant fibrous histiocytoma (MFH) was first described by O'Brien and Stout in 1964.[4] Malignant fibrous histiocytoma was historically thought to be the malignant and
undifferentiated counterpart of the BFH. Recent advances have changed our understanding
of their cell origin, resulting in reclassification as undifferentiated pleomorphic
sarcoma. However, many clinicians continue to use the broader term MFH. In adults,
MFH is the second most common soft tissue sarcoma (STS), with an incidence of 0.88
cases per 100,000 annually.[5] It occurs more commonly in men (2:1, Male:Female), and the incidence increases with
age.[5]
[6] Malignant fibrous histiocytoma is one of the most commonly diagnosed sarcomas in
patients with prior radiation exposure in the head or neck region.[7] The most common locations of these tumors in order are the head and neck, the extremities,
the trunk, and the retroperitoneum.[8] Those occurring on the head and neck might exhibit a more aggressive course; one
study reported a 5-year overall survival of 48% for patients with head and neck tumors
compared with 77% for patients with trunk or extremity tumors.[9]
The clinical presentation typically involves a painless, enlarging nodule that can
become painful if enlarging rapidly.[8] Identification often involves histologic and immunohistochemical evaluations. Treatment
typically involves wide local excision, en bloc resection with 2cm margins of uninvolved
tissue, or Mohs surgery.[8]
[10] However, local recurrence rates are high, ranging from 25 to 75%.[5]
[8] Additional treatment with adjuvant radio- or chemotherapy should be considered on
an individual case basis.[11]
Malignant or benign fibrous histiocytoma involvement of the tongue is considerably
rare, and offers additional symptomatology, such as difficulty speaking or swallowing.[12]
[13] Our review of the available literature identified 20 published cases. Thus, the
present review serves to comprehensively describe previously published cases of BFH
and MFH involving the tongue and aggregate information involving the rare presentation
of this tumor entity.
Review of the Literature
Literature Search
The National Library of Medicine PubMed database was searched for articles discussing
dermatofibromas and dermatofibrosarcomas, or fibrous histiocytomas affecting the tongue.
The following search terms were used: tongue and lingual combined with undifferentiated pleomorphic sarcoma, dermatofibroma, fibrous histiocytoma, and dermatofibrosarcoma. The articles were screened for relevance based on title and abstract. Potentially
relevant articles were subsequently reviewed in full-text for final inclusion decision.
Additionally, the references of each included article were screened for additional
potentially relevant articles. Potentially relevant articles were case reports or
case series or studies discussing fibrous histiocytomas affecting the tongue that
described patient-level data and met the inclusion criteria. The inclusion criteria
were: articles discussing the clinical course of cases of lingual fibrous histiocytoma,
including presentation, diagnostic workup, treatment, and outcome. Articles were excluded
if they did not contain patient-level data and/or original data (that is, literature
reviews), did not pertain to the present topic, or were published in a language other
than English. Of the included articles, patient-level data was extracted and discussed
in the present review.
The search of the PubMed database through September 2016 ([Fig. 1]) returned 234 articles. Screening by title and abstract left 18 potentially relevant
articles for full-text review. Upon full-text review, two studies were excluded for
having diagnoses that did not meet the inclusion criteria (that is, a diagnosis other
than fibrous histiocytoma). Two additional articles were found to meet the inclusion
criteria upon the screening of the article citations. Ultimately, a group of 18 articles,
describing 20 cases, was included in this review. These cases included 15 malignant[12]
[14]
[15]
[16]
[17]
[18]
[19]
[20]
[21]
[22]
[23]
[24]
[25] and 5 benign[13]
[26]
[27]
[28]
[29] fibrous histiocytomas affecting the tongue.
Fig. 1 Systematic search and review strategy of the PubMed database. The initial search
returned 234 articles. After screening the titles and abstracts, 18 articles were
reviewed in full-text for final inclusion. Of these, two articles were excluded for
not meeting the inclusion criteria. Two additional articles were included after screening
the references of the included cases. A final group of 18 articles describing 20 cases
was included in this review.
Clinical Presentation
The demographic profiles of fibrous histiocytomas, summarized in [Table 1], were markedly different between the benign and malignant tumors. The mean patient
age for BFH was 25.8 (range, 8–51) years, whereas MFH appears to affect an older population,
with a mean age of 46.6 (range, 0.4–72) years. Additionally, the benign form reportedly
had a marked predilection for females, with 80.0% of cases involving female patients
versus 40.0% of cases involving female patients in the malignant form.
Table 1
Patient Demographics and Clinical Presentation of Fibrous Histiocytoma of the Tongue
Characteristic
|
Benign Fibrous Histiocytoma, n (%)
|
Malignant Fibrous Histiocytoma, n (%)
|
Demographics
|
Mean, range of age (years)
|
25.8, 8–51
|
46.6, 0.4–72
|
Female
|
4 (80.0)
|
8 (40.0)
|
Male
|
1 (20.0)
|
9 (60.0)
|
Presentation
|
Mean, range of tumor diameter (cm)
|
2.5, 0.4–5.5
|
2.9, 1–5.6
|
Duration of symptoms (months)
|
5.0
|
3.6
|
Painful*
|
0 (0.0)
|
2 (13.3)
|
Firm*
|
4 (80.0)
|
2 (13.3)
|
Epithelial disruption (gray/white, ulcerated, fungating)*
|
0 (0.0)
|
8 (53.3)
|
Location
|
Anterior
|
1 (20.0)
|
2 (13.3)
|
Posterior
|
0 (0.0)
|
3 (20.0)
|
Left lateral
|
2 (40.0)
|
6 (40.0)
|
Right lateral
|
1 (20.0)
|
3 (20.0)
|
Base
|
1 (20.0)
|
2 (13.3)
|
Dorsal
|
2 (40.0)
|
3 (20.0)
|
Ventral
|
1 (20.0)
|
0 (0.0)
|
Unspecified
|
0 (0.0)
|
2 (13.3)
|
Notes: *Presence/absence of each finding not reported in all cases. Percent is out
of total number of cases. Total benign fibrous histiocytoma cases: n = 5. Total malignant fibrous histiocytoma cases: n = 15.
The cases generally presented as a gradually progressive, painless nodule on the tongue
with a firm and/or rubbery, elastic texture. The benign cases demonstrated no overt
epithelial disruption. The malignant tumors were slightly larger than the benign ones,
with an average diameter of 2.92 cm and 2.48 cm respectively. The malignant cases
often presented as gray/white, ulcerated, or fungating lesions (n = 8; 53.3%) that may have affected tongue movement (n = 2; 13.3%). Single cases reported physiologic effects of the tumor, such as dysphagia,
hoarseness, and sore throat. Cervical lymphadenopathy was not reported in any benign
or malignant cases. However, constitutional symptoms such as weight loss and fever
were reported in single cases. The duration of the symptoms was 3.6 (range, 0–12)
months on average for the malignant cases. Benign cases presented later, at 5.0 (range,
2–7) months.
Workup
Blood chemistry values were rarely reported and otherwise generally unremarkable.
Diagnostic imaging studies were reported in two malignant cases, and minimally discussed.
The diagnosis was made with histopathologic evaluation in all cases. Tissue was usually
procured through excisional or incisional biopsy, though one case employed the use
of fine needle aspiration (FNA). Histopathological evaluation was the primary diagnostic
modality; the most commonly observed histopathologic features are summarized in [Table 2]. On the histopathologic evaluation ([Fig. 2]), BFH lesions were predominantly composed of plump spindle-shaped fibroblasts arranged
in a storiform pattern. Scattered histiocytes were present and demonstrated pale and/or
round nuclei, though occasional mitotic figures were present in two cases. The MFH
cases exhibited pleomorphic cells that were haphazardly arranged with occasional storiform
foci. Mitotic figures were present in the majority of cases (n = 8 of 13, 61.5%). The spindle-shaped fibroblasts cells were reported to have ovoid,
elongated nuclei with dense clumped chromatin. The histiocyte-like cells were hyperchromatic
with lobulated nuclei and a granular or foamy cytoplasm. Multinucleated giant cells
were present in 8 cases (61.5%), and exhibited pleomorphic nuclei.
Fig. 2 Benign fibrous histiocytoma histology (a-b) showing spindle-shaped fibroblasts arranged
in a storiform pattern with scattered histiocytes. Malignant fibrous histiocytoma
histology (c-d) demonstrates spindle-shaped to pleomorphic cells haphazardly arranged
with occasional mitotic figures (Courtesy of Dr. Paul L. Haun, M.D.).
Table 2
Histologic characteristics of fibrous histiocytoma of the tongue
Characteristic
|
Benign Fibrous Histiocytoma, n (%)
|
Malignant Fibrous Histiocytoma, n (%)
|
Histologic finding*
|
Pleomorphic
|
2 (40.0)
|
8 (61.5)
|
Mitotic figures
|
2 (40.0)
|
8 (61.5)
|
Storiform pattern
|
3 (60.0)
|
10 (76.9)
|
Spindle, fibroblast-like cells
|
5 (100.0)
|
13 (100.0)
|
Histiocyte-like cells
|
5 (100.0)
|
12 (92.3)
|
Multinucleated giant cells
|
0 (0.0)
|
8 (61.5)
|
Immunohistochemical expression
|
4 (100.0)†
|
6 (100.0)†
|
α-chymotrypsin
|
1 (25.0)
|
2 (33.3)
|
CD34
|
2 (50.0)
|
0 (0.0)
|
Cytokeratin
|
0 (0.0)
|
0 (0.0)
|
Desmin
|
0 (0.0)
|
0 (0.0)
|
Keratin
|
0 (0.0)
|
0 (0.0)
|
S100
|
0 (0.0)
|
0 (0.0)
|
Vimentin
|
2 (50.0)
|
1 (16.7)
|
Notes: *Malignant fibrous histiocytoma histologic findings are shown as percent out
of 13 cases with available histologic data. †Immunostain not reported in all cases. Percent positivity is out of the total number
of cases with immunostain data available.
The immunohistochemistry results (summarized in [Table 2]) were reported in 4/5 BFH and in 6/15 MFH cases, and were primarily valuable for
diagnostic exclusion. The reported immunostains were as follows: α1-antitrypsin, α1-antichymotrypsin,
CD117, CD31, CD34, CD68, carcinoembryonic antigen (CEA), cytokeratin, desmin, factor
VIII, keratin, Ki-67 protein, Leu7, S100, smooth muscle actin, and vimentin. One to two malignant and
benign cases were reported to have α1-antitrypsin, α1-antichymothrypsin, CD34, CD68,
smooth muscle actin, and vimentin immunoreactivity. Benign and malignant cases were
both consistently negative for desmin and S100 staining.
Treatment and Outcome
The treatment and outcomes of the included cases are summarized in [Table 3]. All BFHs were treated surgically with local excision (n = 4, 80.0%) or CO2 laser excision (n = 1, 20%). The single recurrent case, initially excised with CO2 laser, was treated with subsequent hemiglossectomy. No cases of benign tumors received
radiation or chemotherapy. At an average follow-up of 44.6 months, all benign cases
were reported to be disease free.
Table 3
Treatment and Outcome of Fibrous Histiocytoma of the Tongue
|
Benign Fibrous Histiocytoma, n (%)
|
Malignant Fibrous Histiocytoma, n (%)
|
Treatment
|
Resection/excision
|
4 (80.0)
|
4 (26.7)
|
Partial/hemiglossectomy
|
1 (20.0)
|
6 (40.0)
|
Total glossectomy
|
0 (0.0)
|
1 (6.7)
|
Neck dissection
|
0 (0.0)
|
4 (26.7)
|
Chemotherapy
|
0 (0.0)
|
4 (26.7)
|
Radiotherapy
|
0 (0.0)
|
8 (53.3)
|
CO2 laser excision/debulking
|
1 (20.0)
|
2 (13.3)
|
Outcomes
|
Recurrence
|
1 (20.0)
|
6 (40.0)
|
Disease free
|
5 (100.0)
|
8 (53.3)
|
Death due to the disease
|
0 (0.0)
|
7 (46.6)
|
Mean, range of follow-up (months)
|
44.6, 1–144
|
21.7, 9–37
|
Notes: Percent may add up to more than 100 due to patients receiving multiple treatment
modalities. Reported case numbers (n) may add up to more than the total number of
cases (BFH = 5; MFH = 15) due to patients receiving multiple treatment modalities.
Malignant tumors were primarily treated with surgery. Resection/excision was performed
in 4 cases (n = 4; 26.7%). Partial or hemiglossectomy was performed in 6 cases (n = 6; 40.0%); Total glossectomy was performed in 1 case (n = 1; 6.7%). Neck dissection was performed in 4 (26.7%) cases to evaluate the cervical
lymph nodes for possible metastasis. Adjuvant radiotherapy was administered in 8 (53.3%)
cases, often for recurrent disease or for suspicion of metastasis, with various modalities:
iridium or radium implant/brachytherapy, telecobalt therapy, or unspecified external
beam radiotherapy, and the results varied. Adjuvant chemotherapy was administered
in 4 (26.7%) cases, also often for recurrence or metastasis. Combination chemotherapy
regimens included doxorubicin + dacarbazine, actinomycin D + vincristine sulfate + cyclophosphamide,
or cyclophosphamide + vincristine + doxorubicin + dacarbazine (CYVADIC). Recurrence
occurred in 6 (40.0%) cases. At an average follow-up of 24.1 (range, 9–37) months,
8 (53.3%) cases were disease-free. In 7 (46.6%) cases, the patients succumbed to disease
at an average of 19 (range, 9–34) months. All cases are summarized in [Table 4].
Table 4
Case data
Article
|
Age (years)
Ethnicity
Gender
|
Tumor
|
Size
|
Chief Complaint
|
Duration of Symptoms
|
Prior radiation
|
Treatment
|
Results
|
Follow-up
|
Chen et al 2001
|
16-year-old Chinese Female
|
MFH
|
2 × 2.5cm
|
Enlarging painless swelling
|
5 days
|
None
|
Hemiglossectomy with modified radial neck dissection
|
Negative margins and nodes
|
Free of disease at 3 years
|
Geist et al 1990
|
60-year-old Caucasian Male
|
MFH in thorax, metastasis to tongue
|
1.5 × 0.5cm
|
Progressive weakness and fatigue with painful enlarging mass in tongue
|
2 months of weakness and fatigue, 1–2 weeks of tongue mass
|
None
|
Doxorubicin hydrochloride and DTIC chemotherapy followed by surgical resection with
adjuvant radiation for lung persistence and chemo with fludarabine for metastasis
|
Disease continued to spread
|
Died of disease 16 months after presentation
|
Lin et al 1994
|
57-year-old Taiwanese Male
|
MFH
|
−
|
Painful mass
|
1 month
|
2.5 years ago
|
Total glossectomy and partial mandibulectomy followed by subtotal excision with palliative
chemotherapy for recurrence
|
Recurred 6 months after primary surgery
|
Died at 26mo
|
Lopez et al 2011
|
8-year-old Caucasian Female
|
BFH
|
0.4 × 0.4cm
|
Asymptomatic nodule noticed by parents
|
6 months
|
None
|
Excision
|
Clearance
|
No recurrence at 3 years follow-up
|
Mahajan et al 1989
|
28-year-old German Female
|
MFH
|
−
|
Pain and trismus progressing to hoarseness and decreased tongue mobility
|
7 months
|
None
|
Neoadjuvant chemo, modified neck dissection, partial mandibulectomy, partial glossectomy,
and pharyngectomy with adjuvant radiotherapy and chemotherapy
|
Clearance with negative lymph nodes
|
No recurrence at 15 months
|
Manni et al 1986
|
61-year-old Caucasian Male
|
MFH
|
9 × 5 × 3cm
|
Progressive tongue swelling, bleeding, and swallowing difficulties
|
6 months
|
None
|
Hemiglossectomy with supraomohyoid neck dissection
|
|
No recurrence at 2 years
|
McMillan et al 1986
|
42-year-old Female
|
MFH
|
2.5cm
|
Enlarging symptomless swelling
|
8 weeks
|
None
|
Excisional biopsy followed by left hemiglossectomy after histologic diagnosis
|
|
No recurrence at 9 months
|
Pandey et al 2013
|
26-year-old Indian Male
|
BFH
|
6 × 5cm
|
Slow growing mass
|
5 months
|
None
|
Excision and blunt dissection
|
|
Disease-free at 1 month f/u
|
Priya et al 2013
|
30-year-old Indian Female
|
BFH
|
3 × 3cm
|
Nodular mass causing speech difficulty
|
7 months
|
None
|
Excision under local anesthesia
|
Tongue function improvement
|
No recurrence at 3 years
|
Rapidis et al 2005
|
24-year-old Caucasian Male
|
MFH
|
3 × 2 × 1cm
|
Slowly enlarging painless swelling
|
5 months
|
None
|
Surgical resection with 1 cm tumor-free margins
|
|
Disease-free at 18 months
|
Restrepo et al 1987
|
5-month-old Caucasian Male
|
MFH
|
−
|
Difficulty bottle feeding
|
−
|
None
|
Partial glossectomy with adjuvant iridium implants and 2 years of actinomycin D, vincristine
sulfate, cyclophosphamide chemo
|
Unremarkable metastatic workup
|
Disease free at 18 months after diagnosis (7 months after therapy completion)
|
Velez et al 1986
|
14-year-old African American Female
|
Atypical FH
|
3.2 × 2 × 0.7cm
|
Rapidly growing non-tender mass
|
2 weeks
|
None
|
Excised with CO2 laser under general anesthesia followed by hemiglossectomy after
recurrence
|
Local recurrence within several weeks. Tumor-free margins on hemiglossectomy
|
Patient died 6 months later due to Cystic Fibrosis
|
Takimoto et al 1990
|
51-year-old Japanese Female
|
FH
|
2 × 1.5 × 1cm
|
Sore throat with globus sensation
|
“several months”
|
None
|
Elliptical excision with 5 mm margins
|
|
Asymptomatic with no evidence of recurrence at 12 years
|
Young et al 1989
|
67-year-old Taiwanese Female
|
MFH
|
−
|
Rapidly growing protruding mass
|
−
|
5 years ago
|
Tumor debulking by CO2 laser followed by excision with tongue flap push back reconstruction
and adjuvant radiation for recurrence
|
|
No evidence of recurrence at 3 years after excision
|
Young et al 1989
|
52-year-old Taiwanese Male
|
MFH
|
−
|
Globus sensation
|
−
|
12 years ago
|
Tumor debulking by CO2 laser
|
Neck metastasis found 1 month after debulking
|
Died of disease 16 months after diagnosis
|
Zapater et al 1995
|
71-year-old Caucasian Male
|
MFH
|
4 × 2cm
|
Painful gum mass
|
3 months
|
None
|
Telecobaltotherapy of the tongue and oral cavity and then curietherapy on the primary
lesion
|
No evidence of tumor macroscopically
|
Recurrence at 8 months, patient died of disease one month later
|
Bras et al 1987
|
72-year-old Caucasian Male
|
MFH
|
−
|
Painless swelling
|
3 months
|
−
|
Radium implants initially followed by local surgery and modified radical neck dissection
for recurrence
|
Partial response, recurrence
|
Died of disease after 12 months
|
Bras et al 1987
|
65-year-old Caucasian Male
|
MFH
|
−
|
Found on follow-up from prior irradiation for SCC of the tongue
|
−
|
10 years ago
|
Initial resection followed by surgery for local recurrence and radiotherapy + CYVADIC
chemo for lung metastasis
|
|
Died of disease after 20 months
|
Barnes et al 1988
|
21-year-old Female
|
MFH
|
3cm
|
Asymptomatic mass
|
12 months
|
None
|
Partial right glossectomy with right modified radical neck dissection
|
Negative lymph nodes
|
No evidence of disease at 37 months
|
Hiasa et al 1986
|
63-year-old Japanese Female
|
Right pleural MFH metastasis to the tongue and other organs
|
5 × 4 × 3cm
|
Swelling of the right edge of the tongue
|
−
|
1 year ago
|
Partial resection with adjuvant radiation to primary tumor. Followed by radiation
to metastases
|
|
Died of disease at 34 months
|
Abbreviations: BFH benign fibrous histiocytoma; CYVADIC, cyclophosphamide + vincristine + doxorubicin + dacarbazine;
DTIC, dacarbazine; FH, fibrous histiocytoma; MFH, malignant fibrous histiocytoma;
SCC, squamous cell carcinoma.
Note: - = not reported.
Discussion
Benign fibrous histiocytomas of the tongue are consistent in clinical presentation
and course. The mean patient age is 25.8 years, with 80% of cases affecting females.
The cases presented as painless, gradually growing, firm masses. Surgical excision
of benign lesions demonstrated excellent prognosis. The single case using CO2 laser required subsequent hemiglossectomy due to recurrence. In early oral cancers,
this modality is potentially an effective and functionally advantageous approach to
therapy.[30] However, in the case of BFH of the tongue, incomplete resection, and thus subsequent
recurrence, is of concern. Further study is warranted to validate the efficacy of
the CO2 laser as monotherapy for BFH of the tongue. This modality could also have use in
tumor debulking, or may be indicated for smaller lesions.
Accurate histopathological diagnosis of these tumors is critical, considering the
large differences in treatment and outcome, based on tumor pathology and, subsequently,
malignant potential. The differential diagnosis for BFH and MFH is broad, and includes
the following: fibroma, fibrosarcoma, dermatofibrosarcoma protuberans, neurofibroma,
Kaposi sarcoma, among others.
Considering the high innervation, functional importance, and cosmetically sensitive
nature of the tongue, tissue-preserving technique could be considered for excision
of tumors in this location. Mohs micrographic surgery techniques may be of interest
for BFH or MFH of the tongue. A case series of Mohs surgical excision of squamous
cell carcinomas affecting the tongue demonstrated promising results, with no recurrence
evident at a follow-up of 12–34 months.[31] If appropriate, application of this technique could reduce the need for disfiguring
glossectomies. Further evaluation of its efficacy is warranted.
The current World Health Organization guidelines for tumors historically called MFH
now classifies them as undifferentiated pleomorphic sarcoma. Tumors should be classified
as such only after all recognizable lines of differentiation have been excluded.[32] The nomenclature of MFH subtypes was also changed to the following: storiform-pleomorphic
MFH/undifferentiated high-grade pleomorphic sarcoma, giant cell MFH/undifferentiated
pleomorphic sarcoma with giant cells, and inflammatory MFH/undifferentiated pleomorphic
sarcoma with prominent inflammation. These 3 subtypes remained within the “so-called
fibrohistiocytic tumors” category, while myxoid MFH/myxofibrosarcoma, now a separate
entity, has been moved to the myofibroblastic tumor category.[2]
Final Comments
The present review of the available literature returned 5 cases of BFH and 15 cases
of MFH. The BFH cases demonstrated an apparent predilection for female (80.0% versus
40.0% in MFH) and younger (25.8 versus 46.6 in MFH) patients than MFH. The benign
cases presented as a slowly growing, asymptomatic nodule with a firm, rubbery and
elastic texture. The lesions were diagnosed by histopathologic evaluation with immunostaining
used primarily for diagnostic exclusion. Of the BFH cases treated with first-line
surgical excision, no recurrences were reported, whereas the case treated with initial
CO2 laser required subsequent hemiglossectomy for tumor recurrence.
Malignant tumors presented as gray/white, ulcerated, fungating lesions with an average
diameter of 2.92 cm. A minority of cases reported physiologic effects of the tumor,
including affected tongue motility, dysphagia, hoarseness, and sore throat. The histopathologic
workup demonstrated pleomorphic cells with occasional storiform patterns. Mitotic
figures were present, with a mix of spindle-shaped fibroblast-like cells, histiocyte-like
cells, and multinucleated giant cells. The surgical treatment is first-line, and adjuvant
radiotherapy may be considered. Combination chemotherapy regimens vary. Recurrence
occurred in 40.0% of cases (follow-up of 24.1 months), with death due to disease in
46.6% of patients (follow-up of 19.0 months).