Primary tonsillar and soft palate mucosal melanoma
REEM ISSA, SANAD BALOUM, MOHAMMED ATAYA
INTRODUCTION
Melanoma manifests as a malignant tumor originating from
melanocytes, which are neural crest-derived cells
responsible for melanin pigment synthesis in the basal and
suprabasal layers of the epithelium. While cutaneous
surfaces are the main loci, mucosal melanomas (MM) are
not uncommon. 1
Primary oral malignant melanoma is rare, constituting 0.2–
8% of all melanomas, and its etiology remains unclear.
Initiating as a black macule, it evolves into a nodule or
ulcerative lesion, marked by diagnostic asymmetry and
irregular borders. MM is aggressive due to its invasive
nature, with vertical growth and a high index of metastasis.
The preferred treatment modality is surgery, either alone or
together with radiotherapy. The prognosis is poor, with 5-
year survival rates ranging from 0% to 55%. 2
Here, we described a case of a 44-year-old male with
tonsillar and soft palate mucosal melanoma after
complaining of non-tender discoloration in the oral cavity.
As this disease is aggressive and rare to be a primary
tumor, we emphasize the necessity for early detection and
treatment of this tumor.
CASE PRESENTATION
A 44-year-old male who is a farmer with long hours
working under the sun and has a 50-pack-year history of
smoking has no history of alcohol use or occupational
exposure. In addition to the absence of a familial cancer
background, He was referred to the oncology department
in June 2022 after discovering a black discoloration in his
soft palate and the left tonsil. Which is not painful and did
not cause chewing or swallowing discomfort. Intraoral
examination showed non-tender pigmented soft tissue
present at the left tonsil, left posterior soft palate, and hard
palate. The soft palate lesion measured approximately 1*1
with irregular margins (Fig. 1). No palpable cervical or
regional lymph nodes were detected, and there were no
cranial nerve deficits. In addition to an incidental anterior
scalp lesion, an excisional biopsy was done and showed
evidence of basal cell carcinoma with free margins.
Computed tomography (CT) of the maxillary region, upper
and lower endoscopy, and nuclear medicine evaluation,
including brain, bone, and liver-spleen scans, all of which
were negative. Then an incisional biopsy was performed
by the oncologist from the soft and hard palate under local
anesthesia. The specimens were sent for histopathological
examination, and the results showed features consistent
with superficial invasive melanoma, Breslow depth of
nearly 1 mm, and a mitosis score of 3 (Fig. 2)..
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After 9 months, the patient was treated by total excision
of all lesions with lymph node dissection (Fig. 3), in
addition to adjuvant radiotherapy. After excision, the soft
palate specimen was sent to histopathology, which
revealed a tumor size of 1*1 cm2, a depth of invasion of 2
mm, free margins, deep margins of 0.6 cm, no
lymphovascular invasion, LN 0/53, and PT3N0M0.
Unfortunately, the total radiotherapy sessions are
unknown and the real effectiveness of the treatment is
not clear due to missed follow-up from the patient.
Figure 1: intra oral view, left soft Figure 2: Histopathologic
and hard palate discoloration evaluation,
with left tonsillar involvement Hyperpigmentation reveals
atypical melanocytes and
melanin pigmentation
Figure 3: Post excision
of all three lesions.
DISCUSSION
Primary oral mucosal melanoma (MM) is a rare
malignancy with a poor prognosis, developing from
melanocytes found in the basal layer of the oral
mucosa. It affects between 0.2% and 8% of all
melanoma cases [1] and 0.5% of all oral malignancies.
Black, Japanese, and Asian patients tend to be
disproportionately more affected than whites. [2] Unlike
cutaneous melanoma, oral lesions are more common in
men with a male-to-female ratio of roughly 2:1; they can
affect patients of any age with an average age of 56
years. [3].
MM is most commonly found in the palate and maxilla
in around 80% of all oral melanomas. The clinical
presentation of oral melanoma is variable, presenting
as a dark brown, bluish black, or black mucosal
discoloration, and it may be presented as asymptomatic
swelling and occasional bleeding.
But most melanotic mucosal lesions are not painful in the
early stages; identification is often delayed until the ulcer
or growth leads to symptoms. [2, 3] The lesion may be
solitary or multiple, flat or elevated, usually with irregular
borders, and either pigmented or rarely not, which is
termed amelanotic melanoma. [2, 4]
The etiology of oral mucosal melanoma is unclear.
Possible risk factors may include irritation from ill-fitting
dentures, pre-existing melanotic benign oral lesions,
tobacco use, and factors associated with environmental
pollution. Ultraviolet radiation is unlikely to be a risk factor
for MM, in contrast to cutaneous melanoma. [5, 6]
However, in our case, the patient's disease may be related
to a chronic history of smoking. Oral pigmentations can
precede malignant melanoma in approximately 30% of
cases, while the majority of them arise de novo. [7] Recent
data indicates that c-KIT (CD117) is overexpressed in
many mucosal melanoma cases. [8] Bioinformatics
Resources and Applications Facility (BRAF) protein
mutations are uncommon in MM and found in less than
10% of cases. [9]
The clinical diagnosis is quite difficult due to the absence
of specific clinical symptoms of MM. In addition to notable
variations in morphological features, developmental
processes, and biological behavior, Furthermore,
distinguishing mucosal melanoma from metastatic
melanoma is challenging. The differential diagnosis
includes melanotic macule, post-inflammatory
pigmentation, melanoplakia, nevi, Peutz-Jeghur syndrome,
Addison's disease, smoking-associated melanosis, and
Kaposi's sarcoma. [10].
Three main patterns can be observed in mucosal
melanomas. An in situ melanoma remains within the
epithelium without crossing the epithelial-connective tissue
interface. A nodular or deeply invasive melanoma
penetrates the connective tissue underneath. A combined
pattern is characterized by a nodular component with an in
situ or radially growing pattern. [11].
Early diagnosis is crucial since oral melanomas exhibit a
very aggressive evolution with a poor prognosis. The most
reliable method for diagnosing oral melanomas is a biopsy.
It is indicated to do an excisional biopsy of small lesions
and an incisional biopsy of the thickest portion of large
lesions.
[12] Staging may be done using simple tumor, node, and
metastasis (TNM) classifications of malignant tumors and
radiologic methods such as CT, MRI, and positron emission
tomography. [2,13] The histopathological features of
malignant atypical melanocytes of varying phenotypes
(epithelioid, spindle-cell, and plasmacytoid) combined with
positive S-100 and HMB-45 markers confirm mucosal
melanoma. [5, 12]
Surgery is the mainstay of treatment in MM. The first
objective of the surgery is a radical excision with disease-
free margins. Radiation, immunotherapy, and chemotherapy
may be used as adjunctive therapies. [9] Despite the fact
that melanoma is typically not radiosensitive, several
authors have reported improved outcomes following
radiation therapy, particularly in terms of better local control
and survival. [12]
CONCLUSIONS
Early diagnosis and treatment are crucial for
primary tonsillar and soft palate mucosal melanoma
due to its aggressive nature, diversity in clinical
presentation, and difficulties differentiating it from
other oral lesions. This case raises important
questions regarding the complexities of treating this
tumor and highlights the need for more study to
enhance diagnostic techniques and treatment
strategies in the hopes of improving the prognosis.
REFERENCES
1. Sen S, Sen S, Kumari MG, Khan S, Singh S. Oral Malignant Melanoma:
A Case Report. Prague Med Rep. 2021;122(3):222-227.
doi:10.14712/23362936.2021.20
2. Ram H, Mohammad S, Husain N, Devi S, Gupta PN. Metastatic
malignant melanoma of palate: A review of literature and report of an
unusual case. Natl J Maxillofac Surg. 2010;1(1):63. doi:10.4103/0975-
5950.69165
MM : Mucosal melanoma
1- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3448307/
2- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3304187/
3-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10124672/#REF21
4- https://pubmed.ncbi.nlm.nih.gov/5086000/
5- https://www.ncbi.nlm.nih.gov/books/NBK564515/
6- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3469906/
7-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3469906/#ref9
8-https://pubmed.ncbi.nlm.nih.gov/18066592/
9-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4195501/
10-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4147826/
11-https://pubmed.ncbi.nlm.nih.gov/9195622/
12- https://www.ncbi.nlm.nih.gov/books/NBK513276/
13-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4147826/#ref7