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Electron treatment of spindle cell carcinoma of the scalp

Authors: Susan Hunt BA, RT(T), Nishele Lenards MS, CMD, RT(R)(T), FAAMD
Abstract
Introduction:
This case study reviewed the challenges involved in treating the apex of the scalp with
electrons for spindle cell carcinoma (SpCC).
Case Description:
The patient involved in this case study was a 73-year old male who was diagnosed with
spindle cell carcinoma of the apex of the scalp. The patient had an unusual history that indicated
he was at a higher risk of developing such a cancer. Even though SpCC is a highly malignant
type of squamous cell carcinoma, this patients carcinoma was diagnosed early, allowing for a
good prognosis. Since SpCCs involve different cell types, they are difficult to diagnose.
Immunohistochemistry is usually necessary to uncover the various components and provide an
accurate diagnosis.
Conclusion:
It might be prudent to use immunohistochemistry to diagnose certain cancers like SpCC.
Future studies are needed to determine the actual origin and possible causes of SpCC. If the
morphology of SpCC is better understood, more effective treatments could be developed.
Key Words: Spindle cell carcinoma, malignant fibrous histiocytoma, immunohistochemistry

Introduction
This case study involved a 73-year old male who had a history of right vocal cord carcinoma
treated with surgery and radiation therapy in 1996 and basal cell carcinoma of the right forehead
and left medial canthus in 2012. The right vocal cord cancer was treated with surgery and
radiation therapy. Both basal cell carcinomas were removed surgically. Both lesions had deep
margins, but were not an aggressive subtype of basal cell carcinoma. The patient also had a
history of gray plaques near both elbows and about a dozen brown-colored uniform macules on
the trunk, none of which were determined to be cancerous during biopsies performed in 2012. In
April 2014, the patient presented with a lesion on the apex of the scalp diagnosed as spindle cell
carcinoma (SpCC); a highly malignant type of squamous cell carcinoma.1 The patient was

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fortunate since the tumor appeared in a place where it was noticed early. The patients tumor
was small (Stage 1, < 5 cm in diameter), resulting in a better prognosis. Most MFHs are high
grade at diagnosis (Grade 3 or 4), which makes them highly likely to recur. The overall 5-year
survival for a Grade 3 or 4 MFH is 25 70%. This patient was diagnosed with Grade 2 MFH, so
his expected 5-year survival was estimated at 80%.
Spindle cell carcinoma is a type of malignant fibrous histiocytoma (MFH) more common in
Caucasian males in their 60s and 70s.2 It can appear as an exophytic, painless, enlarging,
palpable mass on sun-exposed skin of elderly patients. Certain people have a higher incidence of
MFHs, such as people affected with neurofibromatosis. Neurofibromatosis, a genetic disorder of
the nervous system affecting the growth and development of nerve cell tissue, often presents
with unusual growths on the skin. Of the two types of neurofibromatosis (NF1 and NF2), type 2
is more common. A third type has recently been identified (Schwannomatosis) but it is rare and
not much is known about it. Neurofibromatosis Type 1 is also known as von Recklinghausen NF
or peripheral NF and it can present as multiple patches of tan or light brown skin. Although the
patient was not tested for or diagnosed with neurofibromatosis, the brown spots on the trunk
were suspicious.
Malignant fibrous histiocytomas can also affect the skeletal framework of people with Pagets
disease or dysplasia of bone. There has been recent genetic research into Pagets disease that
has determined the Sequestosome 1 gene on chromosome 5 is involved. People who have these
genes may have a tendency to develop Pagets disease, although infection with a specific virus
may be necessary to trigger the disease. Again, this patient had no known history of either
Pagets disease or dysplasia of bone. Malignant fibrous histiocytoma, also known as
fibrosarcoma and pleomorphic undifferentiated sarcoma (PUS), is known to have an aggressive
biological behavior and a poor prognosis. Malignant fibrous histiocytoma is the most common
soft tissue sarcoma to occur as a result of radiotherapy, and SpCC in general has also been linked
to previous radiation exposure,2 but the patients previous radiation therapy to his right vocal
cord was a small field and did not include the apex of the scalp. Therefore, it is not likely that
the recent malignancy resulted from his previous radiation treatment.
Spindle cell carcinoma commonly occurs on the skin or in the oral cavity. The hallmark of
SpCC is that there are areas of epithelial cells and areas of spindle cells.3 Most SpCCs contain
two cell types: squamous cells and spindle cells (thus they are biphasic),4,5 but they can also be

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monophasic.4,6 The spindle cell component mimics many other types of tumors, making it an
interesting and challenging lesion to diagnose.4,6 Spindle cell carcinoma is a variation of
squamous cell carcinoma, but the cells are spindled or pleomorphic. Even though they can act
like a sarcoma, they are epithelial in nature. Because of the confusing pathology, SpCC has been
known by other names (carcinosarcoma, pseudosarcoma, pleomorphic carcinoma, sarcomatoid
carcinoma and metaplastic carcinoma).4,6 One of the theories is that there are two separate
neoplastic clones combined to form a single lesion. These types of tumors are called collision
tumors (in this case, a carcinosarcoma).6 Another theory is that SpCC is a pseudosarcoma; a
squamous cell caarcinoma that has a stroma that reacts atypically.6 Yet a third theory has the
epithelial cells transforming into spindle cells (sarcomatoid carcinoma).6 Due to their unusual
makeup, many studies have concentrated on analyzing their morphology, immunohistochemistry
and ultrastructure. As many as one-third of SpCCs are monophasic spindled or pleomorphic
tumors instead of biphasic, making the diagnosis of carcinoma more difficult.4
There has been significant debate in the scientific community as to the actual origin of
SpCC.3 Historically, SpCC has been categorized as a type of squamous cell carcinoma (SCC),
but since the prognosis and response to treatment differs significantly from SCC, SpCC may
need its own category.3 Since the cells show characteristics of both squamous and spindle cells,
it is postulated that rather than being true collision tumors, they are actually tumors where the
cells seem to transition between different cell types.3 According to Torenbeek,7 some studies
show the two types of cells diverge at a very early stage in the cells development, whereas other
studies show cells diverge at various points. Some studies suggest that both carcinosarcomas
and sarcomatoid carcinosarcomas have a monoclonal origin.7
In this case, the diagnosis was more difficult because the malignancy was found on sundamaged skin. Spindle cell malignancies that are poorly differentiated and are found on sun
damaged skin are frequently misdiagnosed as either spindle cell melanoma (SCM), atypical
fibroxanthoma (AFX), spindle cell squamous cell carcinoma (SCSCC), leiomyosarcoma or
angiosarcoma.6 All these types of malignancies have overlapping characteristics. Due to their
unusual histology, immunohistochemistry is usually necessary to accurately diagnose SpCC,
especially when it occurs on sun-damaged skin.6
Immunohistochemistry is the process of using special dyes or stains to identify specific
antigens in tissues. Normally, to diagnose a spindle cell carcinoma, markers will be used to

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identify both epithelial and mesenchymal cells.2 If the epithelial component of the tumor is
poorly differentiated, a transcription factor called p53 can be especially helpful for diagnosis.3
There have been spindle cell carcinomas with highly unusual histology such as the case of the
71-year old Japanese man who presented with a lesion in the oral cavity that also had bone-like
material embedded in between the cancer cells. It was believed that the calcified materials were
actually formed by stromal cells.1 In cases such as these, immunohistochemistry is key to proper
diagnosis. Immunohistochemistry can accurately uncover the various components of cells and
can thus determine the origin and unique morphology of the cells.3 Since spindle cell carcinoma
usually originates in squamous cells, it can morph into mesenchymal or epithelial cells with
characteristics of both.3
Case Report
Surgery:
The patients lesion measured 2.7 cm in diameter. A 5 x 5 cm area was excised to verify all
tumor cells were removed, and a 5 x 10 cm area of skin was removed from his right leg and
grafted onto his scalp in a split-thickness graft. When the lesion was removed and biopsied, the
outer margins (in the scalp) were negative but the deep margin in the skull bone was positive.
The periosteum, or membrane, covering the skull in the area just under the tumor and the
superficial layer of bone underneath were ground down but negative margins were not obtained.
Therefore, the radiation treatment included the bone of the skull underneath where the lesion was
removed.
There were several challenges presented at surgery. First, the patient has an anaphylactic
allergy to all types of local anesthesia, so the lesions were removed under general anesthesia;
therefore Mohs surgery was not an option. Also adding to the risk level was the fact that the
patient was on anti-coagulants for cardiovascular issues. Additionally, the patient was diabetic,
so radiation therapy was delayed until the lesion had sufficiently healed after surgery.
Radiation Therapy:
Simulation:
The patient was obese and had trouble lying on his stomach; however, the lesion was on the
back of his scalp, so he had to be simulated and treated in the prone position. Additionally, he
had poor kidney function, so no contrast was used during CT scans. He was positioned prone on

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a table pad with a blue prone pillow, arms up, encircling the superior edge of the pillow. A
small, rounded sponge was placed under his ankles for comfort. Radiopaque markers were taped
to his scalp around the entire area of interest during CT simulation (Figures 2 and 3).
Treatment Planning:
The medical dosimetrist contoured the radiopaque wires around the area of interest, the skin
(scalp), and the brain. The radiation oncologist wanted to fuse the pre-surgery CT with the CT
simulation to help identify where the tumor was removed, but the pre-surgery CT scan was done
with the gantry tilted, which made fusion more difficult. Since the two data sets were not in the
same plane, fusion was not exact. Figure 1 demonstrates a fusion image of the tumor on the presurgery CT and the CT simulation.
There were several challenges that presented during the treatment planning. First there was
the curved surface of the patients scalp. It is more difficult to obtain a good dose distribution on
a curved surface due to the way the radiation interacts with matter. There was a large, crescentshaped area where the split-thickness skin graft did not cover. This resulted in a 5 mm deep
divot (Figure 3) that had to be accounted for by creating a 5 mm custom bolus. The bolus was
designed in the treatment planning software to fill the depression so that the 1 cm bolus could be
laid over the scalp as an even surface. Finally, the pre-surgery CT was taken at an oblique angle,
which made fusion with the planning CT difficult. The fused pre-surgery and planning CT data
sets were used to identify the area where the tumor had been excised.
After the 5 mm bolus was added in the treatment planning software to fill the divot, a 1 cm
thick bolus was custom created to cover the entire area. The area of the skull treated was near
the apex where the curvature was severe, so adding bolus at this location was difficult since
bolus comes in a flat sheet. The goal was to treat the underlying skull where there was disease,
but not penetrate to the depth of the brain. The gray matter covering the surface of the brain is
composed of dendrite and axon terminals of neurons. It is where all the synapses are located.
The white matter of the brain (deeper into the center of the brain) contains the axons, which
connect all the gray matter together. Electrons were chosen as the beam modality due to the
quick rise in maximum dose, region of uniform dose, and relatively rapid fall-off. Electrons
could treat the scalp and underlying skull and allow the dose to fall-off rapidly to avoid the
underlying brain. A 9 MeV electron beam was chosen for its penetration factors with an
effective depth of 1.8 cm. The bolus was added to bring the dose to the skin surface. The

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practical range of a 9 MeV beam is 3 cm (1/3 the energy). The thickness of the skull along the
central axis at the isocenter is 2.2 cm, so the beam penetrated into the skull bone without going
entirely through the bone and into the brain. The gross tumor volume (GTV) was covered by the
90% isodose line. Figures 5, 6 and 7 demonstrate the dose distribution in the sagittal and coronal
planes at different points.
Once the beam modality and energy were chosen, the gantry and couch angles were placed en
face with the surface of the scalp. The angles used for treatment planning determined the dose
distribution, even though the treatment was a clinical setup. In order to distribute the dose
correctly, the isocenter was placed on the surface of the outer bolus and the gantry was angled
until the beam was en face with the skin surface. A couch angle of 270o and a gantry angle of
312o provided the best beam arrangement and dose distribution for the plan. The field size
dictated that a 15 X 15 cm cone be used with a custom cutout. Figure 4 demonstrates a BeamsEye View (BEV) showing the electron cutout, which determined the exact treatment area. The
final challenge was determining where to position the calculation point. The point of maximum
dose was in the skull bone, which would cause tissue inhomogeneity problems with dose
calculations; therefore, an off-axis point was used for the prescription. The prescription was 200
cGy per fraction for 25 fractions, resulting in a total dose of 6000 cGy. The 90% isodose line was
chosen as the normalization point.
The dose volume histogram (DVH) shows the entire tumor bed receiving a dose of 5000 cGy
with minimal dose to the outer layer of the brain (Figure 7). Side effects were expected to be
minimal. During a follow-up visit after 1400 2400 cGy of radiation dose had been delivered,
the patient developed mild skin erythema (Grade I dermatitis), which was expected and not
concerning.
Conclusion
Spindle cell carcinomas may result from injury to the skin or, more accurately, as a result of
inflammation. Sun damage causes inflammation, so if the patient had a genetic predisposition to
develop neurofibromatosis, the sun damage to his balding scalp may have been the cause of the
recent cancer. Genetic testing might be appropriate for this patient. The previous cancers (vocal
cord and basal cell) in addition to the brown macules on his trunk indicate a high probability that
he has a genetic predisposition to developing certain unusual cancers. Additionally,
chemotherapy might be considered in this type of case due to the aggressive nature of the

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disease. Persons who have been diagnosed with either Pagets or neurofibromatosis probably
should be given additional information on MFHs so they can be extra vigilant. If a patient
develops a MFH and receives early treatment, a better outcome may be expected. Finally, it
might be prudent to use immunohistochemistry to help correctly diagnose certain cancers.
Future studies need to be done to determine the actual origin and possible causes of SpCC. If the
morphology of SpCC is better understood, more effective treatments could be developed.

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References
1. Katase N, Tamamura R, Gunduz M, et al. A spindle cell carcinoma presenting with osseous
metaplasia in the gingiva: a case report with immunohistochemical analysis. Head & Face
Med. 2008;4(28):1-6. http://doi:10.1186/1746-160X-4-28
2. Jain M, Singh S, Agarwal K. Spindle cell neoplasm of skin: diagnostic dilemma. Indian J
Pathol Microbiol. 2007;50(4):814-816. http://www.ncbi.nlm.nih.gov/pubmed/18306566
3. Gupta R, Singh S, Hedau S, et al. Spindle cell carcinoma of head and neck: an
immunohistochemical and molecular approach to its pathogenesis. J Clin Pathol.
2007;60(5):472-475. http://doi:10.1136/jcp2005.033589
4. Lewis, Jr JS. Spindle cell carcinoma and other atypical spindle cell lesions of the head and
neck. Head Neck Pathol. 2008;2(2):103-110. http://doi:10.1007/s12105-008-0055-4
5. Boamah H, Ballard B. A case report of spindle cell (sarcomatoid) carcinoma of the larynx.
Case Reports in Med. 2012;(2012):1-4. http://doi:10.1155/2012/370204
6. Lewis JE, Olsen KD, Sebo TJ. Spindle cell carcinoma of the larynx: review of 26 cases
including DNA content and immunohistochemistry. Human Pathol. 1997;28(6):664-673.
http://dx.doi.org/10.1016/S0046-8177(97)90175-1
7. Torenbeek R, Hermsen MA, Meijer GA, Baak J, Meijer CJ. Analysis by comparative
genomic hybridization of epithelial and spindle cell components in sarcomatoid carcinoma
and carcinosarcoma: histogenetic aspects. J Pathol. 1999;189(3):338348.
http://doi:10.1002/(SICI)1096-9896(199911)189:3<338::AID-PATH429>3.0.CO;2-Q

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Figures

Figure 1. Fusion of pre-surgery CT with CT simulation demonstrating original site of tumor and
corresponding area of treatment planning CT (purple).

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Figure 2. Simulation position demonstrating tumor excision and radiopaque marker placement
to area of interest.

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Figure 3. CT simulation showing patient position and 5 mm crescent-shaped divot from skin
graft.

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Figure 4. Beams-Eye View (BEV) Digitally Reconstructed Radiograph (DRR) with cutout
showing Gross Tumor Volume (pink).

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Figure 5. Sagittal view of isodose lines at top of skull, shown in absolute value. The lavender
line shows the 5400 cGy line (90%), yellow is 4800 cGy (80%), orange is 3000 cGy (50%) and
pale blue is 1200 cGy (20%). The GTV is shown in pink colorwash.

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Figure 6. Coronal view of isodose lines at side of skull (at the level of isocenter), shown in
absolute value. The lavender line shows the 5400 cGy line (90%), yellow is 4800 cGy (80%),
orange is 3000 cGy (50%) and pale blue is 1200 cGy (20%). The GTV is shown in pink
colorwash.

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Figure 7. Coronal view of isodose lines at side of skull (at level of calculation point), shown in
absolute value. The lavender line shows the 5400 cGy line (90%), yellow is 4800 cGy (80%),
orange is 3000 cGy (50%) and pale blue is 1200 cGy (20%). The GTV is shown in pink
colorwash.

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Figure 8. Dose Volume Histogram showing dose of 5040 cGy to 90% of the GTV with minimal
dose to the brain.