Melanoma arising in a spinal nerve root. Case report

Melanotic neoplasms of the spinal nerve root are rare but well-documented occurrences. The authors report the case of a 68-year-old woman with a malignant melanotic neoplasm containing premelanosomes and melanosomes and lacking a basal lamina. This tumor was found in a lumbar nerve root and is believed to represent an instance of a primary melanoma arising in a spinal nerve root. The differential diagnosis of pigmented lesions of spinal nerve root includes melanoma, pigmented nerve sheath tumor, blue nevus, melanotic clear-cell sarcoma, and meningeal melanocytoma. Surgeons should be aware of the potential for encountering unexpected malignant lesions involving spinal nerve roots.     

Curatively resected primary malignant melanoma of the esophagus: Report of a case

We report herein a rare case of primary esophageal malignant melanoma in a 49-year-old Japanese woman who presented with a 3-week history of dysphagia. Esophagogram and esophagoscopy demonstrated a polypoid tumor in the upper to mid-thoracic portion of the esophagus, and a radical subtotal esophagectomy was performed through a right thoracotomy. Histological and immunohistochemical studies proved this tumor to be a primary esophageal malignant melanoma. She received no postoperative adjuvant therapy and no signs of recurrence have been clinically observed for the 22 months since her operation.     

Penile malignant melanoma in a hemodialysis patient

Abstract:   A patient who was receiving hemodialysis treatment developed melanotic macules on the surface of the penis in 2002 and showed a tumor-like mass in the same region in July 2006. The patient presented with a pedunculated tumor of 3 cm in diameter on the right side of his penis. The tumor was resected for biopsy and was diagnosed as malignant melanoma. The melanoma was in stage IIIB with pT4 N1 M0. The patient received interferon-β for a total of three courses. A computed tomography scan in the 10th postoperative month did not find any additional metastatic foci or recurrence of the tumor. In the present case, side effects caused by interferon were not observed. Therefore, particularly in dialysis patients, immune therapy might be favored over anticancer drug treatment.     

Malignant transformation of spinal meningeal melanocytoma. Case report and review of the literature

Meningeal melanocytomas are rare tumors and malignant transformation of these lesions is even rarer. The authors report on a case of a 57-year-old man who presented with a melanocytoma located at the L5-S1 level. After gross-total resection of the tumor, it recurred 1 year later as a malignant melanoma. In addition, multiple subcutaneous metastases were found at that time. The patient was treated with radiation therapy (5000 cGy). Five months later metastases in the liver and the left ninth rib were discovered. The pertinent literature is reviewed and disease criteria are presented to distinguish meningeal melanocytoma from malignant melanoma and from meningiomas or schwannomas containing melanotic pigment. Patients and investigators should be cautioned that a meningeal melanocytoma may recur and transform into a malignant melanoma.     

Transformation of a benign oral pigmentation to primary oral melanoma.

OBJECTIVE: The objective is to report the serendipitous 7-year follow-up and transformation of a melanotic palatal lesion, which was initially diagnosed histologically as a benign oral melanotic macule, into primary oral melanoma and to provide long-term follow-up of a case of oral malignant melanoma. STUDY DESIGN: Nine formalin-fixed paraffin-embedded tissue blocks from several different facilities and microscopic slides of the patient's lesions were reviewed to study the transformation of a benign, oral, pigmented lesion into melanoma. RESULTS: Review of blocks and slides of the patient's lesions suggest that the onset of melanocytic hyperplasia (increased clear cell activity) heralded the transformation of the melanotic macule into melanoma. The histology of the first biopsies was totally benign, but retrospectively, the clinical appearance was not typical and was somewhat worrisome because of the size of the lesion and feathering of the pigmentation at the periphery. This clinical presentation, however, was unknown to the pathologists. CONCLUSIONS: This study documents a case of primary oral melanoma arising from an apparent oral melanotic macule and suggests that even histologically benign-appearing oral melanotic lesions should perhaps be viewed with caution if increased numbers of melanocytes (melanocytic hyperplasia or clear cell activity) are present. Careful correlation of clinical appearance and histology is necessary to arrive at an appropriate diagnosis and prognosis for oral pigmented lesions.     

Malignant melanoma in the thymus

A case of malignant melanoma in the thymus is reported. Diagnostic imaging demonstrated a left anterior mediastinal mass in a patient with giant pigmented nevus without malignant change. Histologic and cytologic specimens obtained from the tumor revealed that the tumor was malignant melanoma. Surgery revealed malignant melanoma in the left lobe of the thymus. Many cell nests of pigmented nevi were observed throughout the thymus. The malignant melanoma was thought to have originated from the nevocellular nevus in the thymus. This is the first report of malignant melanoma in the thymus.     

颅内恶性黑色素瘤的诊断(附10例报告)

目的 分析颅内恶性黑色素瘤的诊断要点。方法 报告10例无明颅外原发灶的黑色素瘤,并对其流行病学特点、临床症状、体征和影像资料进行分析。结果 青年男性常见;临床症状和体征表现类似脑肿瘤和脑出血,但病程短,其中2例患者伴有皮肤黑痣;病理均为恶性黑色素瘤;术前误诊8例（80%）。结论 要综合分析该病的流行病学、临床表现、颅外体征和影像学特点以提高术前的确诊率。     

Simultaneous resection of metastatic melanoma in the esophagus and primary cutaneous melanoma showing partial regression: report of a case

We herein describe a case of melanoma that metastasized to the esophagus from a primary melanoma of the abdominal skin in a 40-year-old female. Esophagography and endoscopy demonstrated a 30-mm protruding mass in the proximal third of the esophagus, and this was diagnosed as malignant melanoma by mucosal biopsy. The patient also had a pigmented lesion on her abdominal skin, which was diagnosed immunohistochemically as a primary malignant melanoma from the resected specimen. The esophageal tumor was resected by transthoracic esophagectomy. Histopathologically, the radial growth phase of the tumor cells was not present in the esophageal lesion, which was diagnosed as melanoma metastatic to the esophagus. Postoperatively, the patient received 5 courses of DAV-Feron chemotherapy. Eight months after the chemotherapy, multiple metastases developed, including to the subcutis, bronchus, liver, adrenal gland and mediastinum. Chemotherapy was not effective at this stage. The patient died of multiple organ failure 21 months after initial esophagectomy.     

Primary malignant melanoma of the adrenal gland. A report of two cases and review of the literature

Primary malignant melanoma of the adrenal gland is an established entity despite early doubts. It originates in the adrenal medulla from cells derivative of the neural crest. Because of the high frequency of metastatic involvement of the adrenal by cutaneous and ocular melanomas, rigid diagnostic criteria should be followed. Only four cases of this lesion have been reported since 1946. Review of these four together with the two described in this article shows that primary adrenal melanoma is a highly malignant tumor of middle age that often manifests as a painful flank mass. Distant lymph node metastases can be seen as a presenting sign. Treatment is not effective with a mortality rate approaching 100 per cent within two years. Since the true melanocytic origin of primary adrenal melanoma has not been established and because of the similarity of its pathologic findings with the pheochromocytomas, we believe that adrenal melanoma arises from the pheochromocytes and should be called "melanotic malignant pheochromocytoma."     

Human papilloma virus in melanoma biopsy specimens and its relation to melanoma progression

OBJECTIVES: To evaluate melanoma biopsy specimens for human papilloma virus (HPV) and determine the relation between the presence of HPV, in vitro growth, and clinical progression of melanoma in the patients from whom the biopsy specimens were derived. SUMMARY BACKGROUND DATA: Ultraviolet radiation from sun exposure appears to be the primary causal agent in the development of cutaneous melanoma. However, other agents, including HPV, as observed in different epithelial carcinomas, may also play a role in melanoma development and progression. METHODS: Twelve melanoma biopsy specimens obtained from 12 patients with AJCC stage III and IV melanoma were stained with antibodies against gp-100 (HMB-45) and S-100 protein to confirm melanoma diagnosis and with a polyclonal HPV antibody. After mechanical dissociation, the melanoma specimen cells' ability to grow in vitro was assessed. Patients were evaluated for melanoma progression with physical examination, complete blood count, and liver function tests every 3 months and a chest radiograph every 6 months. RESULTS: All biopsy specimens were positive for S-100, and nine (75%) were positive for gp-100. Seven of 12 (58%) were positive for HPV by immunohistochemistry. In vitro, none of the HPV-negative tumor cells grew from the tumor biopsies, whereas five of seven (71%) of the HPV-positive melanoma tumor cells grew very well. All patients with HPV-positive tumor cells had recurrences and died of melanoma progression, whereas four of five (80%) patients with HPV-negative tumor cells remained alive and without melanoma recurrence. CONCLUSIONS: The presence of HPV was found in 58% of the biopsy specimens obtained from patients with stage III and IV melanoma and correlated with rapid melanoma progression. HPV may serve as a cofactor in the development of melanoma and may modulate a more aggressive phenotype in HPV-containing melanoma cells.     

Isolated pancreatic metastasis from melanoma. Case report

Pancreas is frequently site of isolated metastasis, approximately in the 40% of cases in patient with previous history of malignant neoplasia, more frequently from renal cell carcinoma. The melanoma metastasis can also interest the pancreas in case of disseminated disease (50% of the cases); more rarely the pancreas is site of isolated metastases from melanoma. The treatment of the pancreatic metastases from melanoma is controversial: the therapeutic choices are few and the role of surgery is not well defined. If the metastasis are confined to the pancreas, the surgical treatment can be useful for better long time survival. We report a rare case of melanoma with pancreatic isolated metastasi in a patient with a previous melanotic metastasis to the inguinal lymph nodes without evidence of primitive tumor.     

Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature

The authors present the case of a dumbbell-shaped malignant psammomatous melanotic schwannoma of the upper cervical spine involving the C-2 sensory root. The family of the patient had a history of other malignant stromal tumors, without the Carney complex genetic pattern. The 30-year-old female patient complained of experiencing cervical pain and cervical muscle contractions for 6 months, and was admitted to the hospital. The cervical T1-weighted magnetic resonance (MR) images revealed the presence of a slightly hyperintense C2-3 intra-extradural lesion, moderately enhancing, which had eroded and enlarged the intervertebral foramen. The patient workup also included computed tomography scans and angiography. A posterior approach was used to perform a C2-3 hemilaminectomy, including opening of the dura mater and gross-total removal of the lesion. Histopathological examination of the lesion revealed it to be a malignant psammomatous melanotic schwannoma. The cerebrospinal MR image of the patient obtained at the 12-month follow-up examination demonstrated the presence of tumor progression into the subarachnoid space at the C-3 level. The strong malignancy potential of the lesion must be considered in the future management of the patient, especially due to the presence in the family of other stromal tumors such as gastrointestinal-stromal tumors and malignant melanomas. The authors review all the literature concerning melanotic schwannomas and report 105 cases of melanotic schwannoma that were not related to the Carney complex. The particular focus of their review is on the characteristics of the malignant progression of melanotic schwannoma, such as local recurrences, metastasis, and survival rate.     

Melanoma-specific cytotoxic T cells generated from peripheral blood lymphocytes. Implications of a renewable source of precursors for adoptive cellular immunotherapy.

Cytotoxic T lymphocytes (CTL) specific for autologous human melanoma have been generated in vitro from peripheral blood lymphocytes (PBL) of five patients with resectable stage II malignant melanoma. The PBL were cultured with 5u/ml recombinant IL-2 and were repeatedly stimulated with irradiated fresh or cultured autologous tumor cells. Cytotoxicity was determined by four-hour chromium release assays. Specific cytotoxicity developed in 30 to 40 days, after three or four stimulations with tumor. The PBL-derived CTL are CD3+ and are mixed for CD4+ and CD8+ phenotypes. They lysed autologous melanoma and failed to lyse allogeneic melanoma, K562, or autologous lymphocytes. The lysis of autologous tumor was maintained for more than 4 months. The cells proliferated in response to autologous, but not allogeneic melanoma cells, in a dose-dependent manner. Lysis of the autologous tumor target was inhibited with w6/32, a monoclonal antibody to HLA Class I antigens. It is concluded that PBL may serve as a plentiful and renewable source of precursor cells for the generation of autologous tumor-specific CTL, which may be useful in specific adoptive cellular immunotherapy of melanoma.     

Pigmentierte Läsionen der Mundschleimhaut

Primary malignant melanoma of the oral cavity is an exceedingly rare event. The most frequently affected sites are the palate and the gingiva of the maxilla. The lack of symptoms often causes diagnostic delay. We report on a 78-year-old man with a history of melanotic pigmentation on the hard palate for many years. Due to tumor growth an incisional biopsy was performed which yielded the diagnosis of mucosal melanoma arising in a preexisting blue nevus. Mucosal melanomas of the upper airway are not classified as other melanomas but with their own TNM classification due to their special behavior. Although the classification by Clark cannot be applied, thickness and size of the tumor are known prognostic indicators.     

Radical resection of primary malignant melanoma of the gallbladder with multiple metastases: Report of a case

We present herein an usual case of primary malignant melanoma of the gallbladder in a 51-year-old man in whom an exploratory laparotomy for melena revealed six malignant melanoma lesions located in the gallbladder, main pancreatic duct, stomach, duodenum, jejunum, and a mesenteric lymph node. Total pancreatectomy was performed and histologically, junctional activity was seen only in the gall-bladder, suggesting that this was the primary site. No melanotic lesions were found on the skin or eyes. The metastases to the main pancreatic duct and gastrointestinal tract appeared likely to have occurred as a consequence of the mucosal dissemination of the tumor cells shed into the bile. The post-operative course was uneventful and combined chemotherapy was administered for 16 months. No new metastatic lesions were found until 21 months postoperatively, when metastases were detected in the brain and thoracic spinal cord. These metastatic tumors were removed surgically, but the patient died from cerebral disturbance 26 months after the initial operation. Thus, we consider that aggressive surgical therapy was effective for extending the survival time and improving the quality of life of this patient.     

Histologic distinction between subungual lentigo and melanoma

The distinction between a benign subungual pigmented macule (lentigo) and an early lesion of melanoma in situ can be difficult. To identify histologic parameters of potential diagnostic value, we retrospectively reviewed biopsies and excisions of 35 pigmented nail lesions. We studied 20 melanomas (10 invasive and 10 noninvasive) and 15 benign subungual melanotic lentigines. Ten specimens of normal nail apparatus obtained for reasons other than melanonychia were also examined as controls. The parameters, which were analyzed, included the density of melanocytes, the presence of multinucleated cells, pagetoid spread, cytologic atypia, inflammation, and the distribution of melanin pigment. The density of melanocytes was measured as the number of cells per 1 mm stretch of subungual dermo-epithelial junction [=melanocyte count (MC)]. The MC for invasive melanomas was as follows: mean=102, median=92.5, and range 52 to 212. For noninvasive (only in situ) melanoma, the mean MC was 58.9, median 51, and range 39 to 136. For benign subungual melanotic macules, the mean MC was 15.3, median 14, and range 5 to 31. In normal controls, the mean MC was 7.7, median 7.5, and range 4 to 9. Qualitative features associated with in situ melanoma and useful for its distinction from benign subungual melanotic macules included the presence of confluent stretches of solitary units of melanocytes, multinucleated melanocytes, lichenoid inflammatory reaction, and florid pagetoid spread of melanocytes.     

Malignant melanoma presenting as a mediastinal mass

A case of malignant melanoma presenting as a mediastinal mass without an extrathoracic primary is reported. Microscopically the tumor appeared consistent with malignant melanoma, with the presence of focal melanin pigment in large epithelioid cells. Fontana stain confirmed the presence of melanin pigment. Immunohistochemical staining further suggested melanoma, with the tumor cells expressing a HMB45+, S100+ and cytokeratin-phenotype. Electron microscopy showed an abundance of melanosomes confirming the diagnosis of malignant melanoma.     

Oral malignant melanoma detected after resection of amelanotic pulmonary metastasis

INTRODUCTIONSolitary pulmonary metastasis from oral malignant melanoma is very rare.PRESENTATION OF CASEWe demonstrated a 84-year-old patient with a lung nodule that was diagnosed as malignant melanoma by video-assisted thoracoscopic resection. Because primary pulmonary malignant melanoma was extremely rare, the tumor was thought to be a metastasized from an occult primary lesion. A detailed physical examination revealed a black tumor in the oral cavity, and this was suspected to have been the primary. Resection of the hard palate tumor and dissection of the cervical lymph nodes were performed. The patient was simply followed up without further therapy at his request, and he died one year after surgery due to bleeding from a pleural metastasis of malignant melanoma.DISCUSSIONPrimary melanoma of the oral cavity is rare, accounts for 0.5% of all oral cancers, and 0.8–1.8% of all melanomas. Because of absence of symptoms in the early stage of the disease and the presence of the tumor in relatively obscure areas of the oral cavity, the diagnosis is unfortunately often delayed. In view of the rarity of primary lung melanoma, when lung tumor was diagnosed as malignant melanoma, detailed physical examination of the entire skin and mucosa including the oral cavity was necessary.CONCLUSIONOral malignant melanoma was very rare, but oral cavity should be examined when the pulmonary nodule was diagnosed as malignant melanoma.     

Malignant melanoma showing ganglioneuroblastic differentiation: report of a unique case.

We report a case of metastatic malignant melanoma in an inguinal lymph node, expressing ganglioneuroblastic differentiation. This was characterized by the presence of discrete nests and islands of large ganglion cells with abundant cytoplasm and eccentric nuclei with prominent nucleoli admixed with smaller primitive neuroblasts. The cells were separated by pale pink fibrillar material representing neuritic cell processes. These foci of ganglioneuroblastoma were seen over a background of an otherwise typical metastatic epithelioid, focally melanotic, malignant melanoma. Immunohistochemistry showed positivity for neurofilament, synaptophysin, chromogranin, vasoactive intestinal peptide, and glial fibrillary acidic protein in the areas with ganglioneuroblastic differentiation, but not in the melanocytic component. Conversely, HMB45 positivity was expressed by the melanocytic cells only. S-100 protein and Melan-A, a putative melanocytic marker, showed positivity in both melanocytic and ganglioneuroblastic components. Ultrastructurally, neuritic cell processes and dense core neurosecretory granules were identified in the ganglionic and neuroblastic cells. A subsequent nodal metastasis in the same region showed focal neuroblastic differentiation without the ganglionic element. No evidence of neuronal or ganglionic differentiation was seen in the primary skin melanoma.