Orbital and myocardial metastases of a primary pulmonary melanotic schwannoma

Abstract

We present the case of a 60-year-old man with a primary pulmonary melanotic schwannoma treated with surgery and who developed an orbital and myocardial relapse 2 years after the initial diagnosis. Melanotic schwannomas are rare pigmented tumours that tend to arise from the peripheral nerves near the midline. A primary lung presentation, as in our case, is extremely rare. In more than half of cases, the Carney triad of myxomas of the heart, skin and breast, spotty pigmentation and endocrine hyperactivity is present. A thorough pathological study is pivotal for a correct diagnosis. The main differential diagnosis is with metastases of malignant melanoma. The biological behaviour is unpredictable. Treatment should include radical surgery if possible; the role of chemotherapy and radiotherapy is uncertain due to the rarity of the tumour.

Intracranial Melanotic Schwannomas

“…In our literature review we only found 17 articles describing 18 cases of intracranial MS (►Table 1). 1,[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Mean age at presentation was 36.3 years (range: 11-77 years), with a strong male predominance (male-to-female ratio: 13:3). MSs mainly occur sporadically, but less frequently they may present as a manifestation of NF II or CC.…”

“…1,5,6,9,17,25,30,53,58,[60][61][62] Radiation therapy may or may not be associated with chemotherapy. 1,8,20,25,36,63 Vallat-Decouvelaere and Zhang observed a lower rate of recurrence and metastasis in patients treated with radiotherapy as adjuvant treatment, suggesting its positive role in the treatment of MS. 1,25 Because of the potential aggressive behavior and the long time to progression, a patient harboring intracranial MS should strictly follow up, even in the case of GTR. 1,4,6,8,9,20,[32][33][34]36,45,54 In the study of Lowman and Livolsi of two spinal MSs, disease control in the case of STR plus radiotherapy was 14 years compared with 17 years in the case of GTR only.…”

“…Radiotherapy is usually taken into consideration as adjuvant therapy in case of malignant histology, regardless of the presence of a remnant, recurrence, and incomplete surgical resection. 1,5,8,20,36,49,53,61 Watson and coworkers suggested, that, without histologic signs of malignancy, adjuvant treatment should not be performed and only strict radiologic and clinical follow-up is required. 30,32 Other authors, because of the potential malignant behavior of these tumors, emphasize the role of adjuvant radiotherapy or radiosurgery, especially in cases of STR, regardless of the histologic features of malignancy.…”

“…1 Craniofacial and intracranial lesions are extremely rare. 1,[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Peak incidence is in young adults, without any sex predilection. 1,19,20 Growing pattern is usually as a single lesion, but multiple nodules have also been described.…”