Keywords
Anemia - blood loss - cell cancer - squamous - ulcer
INTRODUCTION
Cutaneous squamous cell carcinoma (cSCC) is the second most common cancer in humans
after basal cell carcinoma (BCC). Due to the shared lineage with epidermal keratinocytes,
“keratinocyte carcinoma” is becoming the preferred term to refer to BCC and SCC instead
of the traditional term “nonmelanoma skin cancer.”[1] As most primary keratinocyte carcinomas have low metastatic potential, most of the
identified lesions are localized and minimal risk. Metastatic cSCC is uncommon and
comprises approximately 4%–5% of the cases.[1] Only a small fraction (0.4%) may present with giant tumors with a diameter of ≥5
cm and these are considered high-risk tumors with high morbidity and mortality.[2] Traditionally, the “Marjolin’s ulcer” term is used to refer to SCC arising from
burns’ scars; however, currently, it encompasses all malignant tumors that primarily
occur in body surface ulcers that include squamous cell carcinoma, BCC, melanoma,
sarcoma, and others.[3] Although perioperative anemia is a very common finding reported in head and neck
SCCs (HNSCC),[4] it is not reported with facial cSCC. We describe an interesting case of giant-neglected
facial Marjolin’s ulcer identified as cSCC associated with perioperative blood loss
anemia, its staging workup, and the treatment options provided.
CASE REPORT
A 63-year-old Caucasian male presented with a 3-year history of ulcerating lesion
involving the left cheek and parotid gland. Three years ago, he started to have slow-growing
lesion arising from a nonhealing wound that occurred after a left cheek traumatic
injury by a tree branch. The lesion was neglected until it started to increase rapidly
in size over the last 5 months when he sought treatment. He reported associated left-sided
hearing loss, weakness in eye closing, and mandibular weakness, all on the left side.
He denied weight loss, trismus, or any lymphadenopathy.
The patient lived in California and had excessive sunlight exposure before he moved
to Arkansas, where he received treatment. He has a past medical history of asthma,
and cSCC of the right cheek required Mohs surgery. He reported no medications use
or allergies. He denied tobacco, alcohol, or recreational drug use or any occupational
chemical exposure. No history of immunocompromised status or immunosuppressive therapy
reported. The patient’s mother died from colon cancer at age 46, and his brother had
a history of melanoma.
On physical examination, there was an extensive bleeding fungating growth overlying
the left cheek, extended to the left helix root and tragus [Figure 1A]–[1]. The lesion hung over the external auditory meatus, which was intact. The tympanic
membrane was intact. There was no significant submandibular or cervical lymphadenopathy.
He had pale conjunctivae. The remainder of his physical examination was unremarkable.
Figure 1: Giant-neglected facial Marjolin’s ulcer. (A–B–C) Clinical presentation. (D) Defect
after complex excision before flap application. (E) Intraoperative picture after the
reconstruction of the left facial defect with an anterolateral thigh free flap with
microvascular anastomosis. (F) Two days after surgery
The patient was evaluated initially by a dermatologist, and his lesion biopsy showed
poorly differentiated SCC. Given his extensive and high-risk SCC, he was referred
to a head and neck surgeon. The computed tomography (CT) scan of the head and neck
showed an extracapsular invasion in the soft tissues of the muscles of mastication
and the zygomatic arch [Figure 2A]. For the staging workup, the patient underwent a positron emission tomography/computed
tomography (PET/CT) scan, which showed the giant invasive facial mass [Figure 2B] and interestingly showed an incidental finding of a 2.1 cm left cortical renal mass
and bladder thickening [Figure 2B] and [D]. The patient reported no history of gross hematuria or flank pain. On the basis
of his staging workup, he was considered to have a T3, N0, and MX poorly differentiated
SCC.
Figure 2: (A–C) computed tomography (CT) and positron emission tomography (PET) scan of head
and neck showing the giant invasive facial mass. (B–D) CT scan and PET scan of the
abdomen and pelvis showing the incidental left renal mass
The patient was scheduled for an elective surgical resection and a reconstruction
flap. His preoperative evaluation revealed increased fatigue and dyspnea with exertion,
palpitations, dizziness, and lightheadedness without syncope. His preoperative laboratory
data showed hemoglobin of 5.3g/dL, mean corpuscular volume (MCV) 97.1, with normal
B12, folic acid, and iron studies. He reported a long-term intermittent low-volume
oozing from the facial lesion, which increased to an intermittent frank bleeding over
the last 3 months. He denied hematochezia, hematemesis, hematuria, or coffee-ground
emesis. Despite blood transfusion of two packed red blood cells (RBCs) units in the
outpatient settings, his hemoglobin improved only to 6.5g/dL. Due to his blood loss
anemia, he was hospitalized preoperatively and received another unit of packed RBCs
for medical optimization before his planned surgery.
He underwent an excision of the left zygomatic bone, left total parotidectomy with
facial nerve sacrifice, and selective left neck dissection levels of I–IV [Figure 1D]. The reconstruction of the left facial defect was performed with an anterolateral
thigh free flap with microvascular anastomosis [Figure 1E] and [F]. Pathology studies showed deeply invasive squamous cell carcinoma invading the parotid
gland. All the margins and excised lymph nodes were negative for cancer.
Upon his postoperative follow-up, he was, expectedly, noted to have iatrogenic Bell’s
palsy. His ophthalmology examination revealed no corneal epithelial defect and he
was started on high-viscosity artificial tears. The radiation oncology team recommended
adjuvant radiation therapy to the deeper structures of the operative site and he is
scheduled to undergo an intensity-modulated radiation therapy (IMRT) to preserve salivary
function and minimize the risk of flap failure. For his renal mass, the patient was
evaluated by urology and based on the peripheral location, renal cell carcinoma was
suspected. He is scheduled for percutaneous CT-guided cryoablation after recovery
from the facial reconstructive surgery.
DISCUSSION
Marjolin’s ulcers are form of cutaneous malignancy, and mostly represent cSCC, arising
from chronic wounds. The malignant transformation rate was reported as 1%–2%.[2],[3] Ultraviolet radiation exposure among fair-skinned individuals is the primary etiology
for keratinocyte carcinoma with higher incidence among individuals who have male gender,
older age (>65 years), and lighter skin color,[1] which seems to fit the patient’s presentation based on his demographics and location.
Other risk factors include ionizing radiation, immunosuppression, chronic inflammation,
arsenic exposure, human papillomavirus, and genetic disorders.[5] In his case, chronic inflammation occurred due to his neglected facial nonhealing
wound. His presentation was consistent with high-risk cSCC,[6] which usually requires multidisciplinary approach. His management required collaboration
among dermatologist, head and neck surgeon, plastic surgeon, internist, ophthalmologist,
urologist, and radiation oncologist.
The lungs, liver, brain, and bone are the most frequent sites for distant metastases
of cSCC.[7] Although it is not entirely excluded, the patient’s kidney tumor was felt to be
incidental finding and not directly related to his cSCC. Although anemia is very common
in HNSCC and has prognostic implication,[4] symptomatic blood loss anemia has not been reported in cSCC.
As the population ages, keratinocyte carcinoma incidence is expected to increase dramatically
over the next couple of decades. Focus on preventive measures and more public awareness
of the carcinogenic effect of sunlight exposure is crucial to tackle the rising number
of skin cancers.[1]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
