Medical Care
Peutz-Jeghers syndrome
Medications are directed toward the GI aspects of the disease. No medications are directed specifically for treatment of the oral lesions.
Amalgam tattoos
No medication or treatment is necessary.
Melanoma
Medical therapy is not often beneficial for oral melanoma. Chemotherapeutic medications for the treatment of oral melanoma do not reliably reduce the tumor volume.
Aggressive surgery remains the treatment of choice.
Interferon, dacarbazine, and BCG vaccine have been tried with marginal and unpredictable results.
Drug therapy (dacarbazine), therapeutic radiation, and immunotherapy are used in the treatment of cutaneous melanoma, but they are of questionable benefit to patients with oral melanoma. Dacarbazine is not effective in the treatment of oral melanoma; however, dacarbazine administration in conjunction with interleukin 2 may have therapeutic value.
Experience with oral malignant melanoma is largely derived from single cases. Anecdotal reports have described success with interferon alfa or hyperfractionated radiation therapy. Many cancer centers follow surgical excision with a course of interleukin 2 as adjunctive therapy to prevent or limit recurrence. [31]
Because of the rarity of the lesions, assembling a cohort study group to evaluate the different therapeutic regimens is difficult.
Protocols with interferon and other immunotherapies are being investigated. A good direction for future research would be to incorporate multimodal therapy.
Surgical Care
Peutz-Jeghers syndrome
Push enteroscopy and intraoperative enteroscopy with polypectomy can be used to effectively treat and defer the need for repeated small bowel resections in some patients.
Laparotomy and resection, as indicated, should be performed for repeated or persistent small intestinal intussusception or obstruction or for persistent intestinal bleeding.
Surgical treatment for cancers detected by surveillance and diagnosis is indicated.
Melanoma
Excisional surgery with tumor-free margins remains the treatment of choice. Early surgical intervention when local recurrence is detected enhances survival. Approximately 80% of patients with oral melanoma have local disease, and 5-10% of patients present with grossly involved nodes. After complete surgical excision, the locoregional relapse rate is reportedly 10-20%, and 5-year survival rates are clustered around 10-25%, with a reported range of 4.5-48%. McKinnon et al [32] reported that tertiary care centers have the best results.
Although radiation alone is reported to have questionable benefit (particularly in small fractionated doses), it may be used as an adjuvant when high-fractionated doses are used.
Neither lymphoscintigraphy nor intraoperative blue-dye sentinel node biopsy (eg, selective neck dissection) is useful in predicting drainage patterns in oral melanomas. Anatomic ambiguity appears to preclude consistent assessment of oral lymphatic drainage patterns when this technique is attempted. Surgical lymph node harvesting depends on the identification of positive nodes after clinical or imaging examination. Prophylactic neck dissection (eg, elective neck dissection) is not advocated as a treatment for oral melanoma.
Consultations
For patients with Peutz-Jeghers syndrome, consult a gastroenterologist familiar with Peutz-Jeghers syndrome.
For melanoma, consult the following specialists and facilitate their meeting during head and neck tumor boards to plan the best therapy and aftercare for patients with oral melanoma:
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Ear, nose, and throat surgeon
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Pathologist (eg, dermatopathologist and general surgical, head and neck, or oral and maxillofacial specialist)
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Medical and radiation oncologists
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Maxillofacial prosthodontists
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Speech therapist
The primary concern is to ensure surgical removal; secondary concerns deal with restoring function and cosmesis. If the anatomy restricts ensured removal, medical oncologists and radiation oncologists must provide the most appropriate adjunctive therapy. Maxillofacial prosthodontists can provide advice about the appliances available and the tissue requirements for support and retention. The involved consultants should be aware of the recall schedule to assess patient progress and adaptation.
Long-Term Monitoring
Peutz-Jeghers syndrome
Close follow-up care is needed for the GI aspects of the disease. Genetic counseling should be offered to families trying to have children. Further outpatient care for patients with Peutz-Jeghers syndrome includes the following:
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Annual physical examination that includes evaluation of the breasts, the abdomen, the pelvis, and the testes
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Annual complete blood cell count
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Repeated removal of hemorrhagic or large polyps (>5 mm) by endoscopic polypectomy
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Surveillance for cancer, possibly including (1) Small intestine with small bowel radiography every 2 years; (2) Esophagogastroduodenoscopy and colonoscopy every 2 years; (3) Ultrasonography of the pancreas yearly; (4) Ultrasonography of the pelvis (women) and testes (men) yearly; (5) Mammography (women) at ages 25, 30, 35, and 38 years; every 2 years until age 50 years; then annually; and (6) Papanicolaou (Pap) test every 3 years
Amalgam tattoos
No follow-up care is necessary for amalgam tattoos once the diagnosis is determined.
Melanoma
Patients with melanoma must receive close follow-up care involving oncologists, surgical oncologists, radiologists, and dermatologists. In many instances, psychological assistance and intervention is also necessary.
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Smoker melanosis.
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Smoker melanosis.
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Melanotic macule.
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Melanotic macule.
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Melanotic macule.
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Oral nevi
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Intramucosal nevus.
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Blue nevus.
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Intramucosal nevus.
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Junctional nevus.
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Compound nevus.
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Blue nevus.
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Oral pigmentation of the gingiva due to amalgam tattoo along with photomicrograph.
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Photo of oral pigmented lesion from a patient with Peutz-Jeghers syndrome.
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Photos of patients with amalgam tattoo.
