Amelanotic malignant melanoma of the esophagus. Report of a case

Primary malignant melanomas of the esophagus are rare neoplasms with aggressive behavior and tendency to lymphatic and hematogenous spread. We report on a patient affected with a primary amelanotic melanoma of the esophagus synchronous to a squamous cell carcinoma of the epiglottis and treated by subtotal esophagectomy. A 58-year-old woman presented with a three-month history of dysphagia, retrosternal pain and weight loss. An upper gastrointestinal endoscopy revealed a 3 cm, non pigmented, polypoid mass of the middle esophagus and biopsy was consistent with undifferentiated carcinoma. Fiberoptic bronchoscopy revealed a 0.5 cm, polypoid lesion of the right aspect of the epiglottis consistent with a squamous cell carcinoma. The esophageal neoplasm was treated by subtotal esophagectomy while the squamous cell carcinoma of the epiglottis was referred to radiotherapy 1 month after surgery. Microscopy showed diffuse proliferation of anaplastic cells with multiple mitotic figures, marked nuclei and multilobulated nucleoli. The neoplasm stained positive for HBM-45 antigen and S-100 protein. Tumor stage was pT1N0M0. Review of patient's medical history and exploration of skin and mucous membranes failed to reveal malignant lesions and definitive diagnosis was primary amelanotic malignant melanoma of the esophagus. The patient died 16 months after surgery of disseminated disease. In conclusion the present case confirms that primary malignant melanomas of the esophagus have a dismal outcome even in cases of early-stage lesions amenable to surgical resection.     

Primary malignant melanoma of the esophagus: a case report

Primary malignant melanoma of the esophagus is an uncommon tumor associated with a poor survival (5% at 5 years), even when resected at an early stage. Because its symptoms are no different from those of other malignant tumors of the esophagus, histopathologic examination is usually needed to reach a definitive diagnosis. A 57-year-old white nonsmoking nonalcoholic woman was referred to our department after 2 months of increasing dysphagia, odynophagia, and weight loss (5 kg in 2 months). Esophagogastroscopy revealed a dark blue, pediculated, polypoid lesion. Biopsies were taken. Endoscopic ultrasound showed a hyperechoic heterogeneous tumor. Barium esophagogram showed a filling defect of ~ 6 cm in the middle-low esophagus, and thoracic and abdominal computed tomography (CT) scan showed a well-delimited esophageal tumor with no clear lymph node enlargement. The pathology report described a proliferation of small spindle-shaped or stellate cells arranged in a spiral or fascicular structure. All tumor cells were intensively positive for immunoreaction, using HMB45 antimelanoma antibodies. To remove the tumor, distal esophagectomy through a double abdominal and thoracic approach was performed. No postoperative complications were reported and no chemo- or radiotherapy was given. The patients is still alive with no evidence of recurrence at 9 months after the operation.     

Primary Malignant Melanoma of the Small Intestine: Report of a Case

The small intestine is the most common site of gastrointestinal (GI) metastases from cutaneous malignant melanoma; however, primary malignant melanoma originating in the small intestine is extremely rare. We report the case of a 72-year-old man found to have a primary malignant melanoma in the ileum. The patient presented with anorexia, weight loss, diffuse colicky abdominal pain, and episodic rectal bleeding. A preoperative diagnosis of a small intestinal tumor was based on the findings of enteroclysis and computed tomography scanning. This diagnosis was confirmed at laparotomy and an enterectomy was performed. Histopathological examination of the resected specimen clarified the exact nature of the lesion, confirming the diagnosis of melanoma. A thorough postoperative investigation did not reveal a primary lesion in the skin, anus, oculus, or any other location. Thus, we diagnosed this tumor as a primary lesion. One year after his operation, the patient remains well without any evidence of recurrence. Primary malignant melanoma of the small intestine is an extremely rare lesion, which must be differentiated from other intestinal tumors. Received: August 6, 2001 / Accepted: March 5, 2002     

Primary malignant melanoma of the esophagus--a case report

An unusual case of primary esophageal melanoma is reported herein. A 68 year old man who had experienced occasional dysphagia for about one month without suffering any weight loss was admitted to our department. An esophagogram revealed two lobulated masses and esophagoscopy showed a pigmented tumor in one of the masses. Curative surgery was thus performed through a right thoracotomy. The macroscopic appearance of the resected specimen was very unusual and it was subsequently proven to be primary malignant melanoma of the esophagus by histological examination. Postoperatively, cyclophosphamide and interleukin-2 were administered intravenously, followed by lymphokine-activated killer therapy. However, multiple liver metastases were found on a CT scan, 3 months after the operation and he died about 1 month later. The operative indications for primary malignant melanoma of the esophagus are discussed in this report.     

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.     

Primary malignant melanoma of the esophagus —A case report—

An unusual case of primary esophageal melanoma is reported herein. A 68 year old man who had experienced occasional dysphagia for about one month without suffering any weight loss was admitted to our department. An esophagogram revealed two lobulated masses and esophagoscopy showed a pigmented tumor in one of the masses. Curative surgery was thus performed through a right thoracotomy. The macroscopic appearance of the resected specimen was very unusual and it was subsequently proven to be primary malignant melanoma of the esophagus by histological examination. Postoperatively, cyclophosphamide and interleukin-2 were administered intravenously, followed by lymphokine-activated killer therapy. However, multiple liver metastases were found on a CT scan, 3 months after the operation and the died about 1 month later. The operative indications for primary malignant melanoma of the esophagus are discussed in this report.     

Primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus is a rare but aggressive tumor that accounts for less than 0.1-0.2% of all esophageal malignancies. The aim of this study was to report a case of primary malignant melanoma of the esophagus in a 72-year-old woman. The diagnosis was histologically proven, but the patient died despite extensive surgical resection.     

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.     

Primary Malignant Melanoma of the Esophagus Arising from a Melanotic Lesion: Report of a Case

We herein report a case of primary esophageal malignant melanoma in which the development from a preceding benign melanotic lesion and the growing process of the tumor were chronologically observed by serial endoscopic examinations. Biopsy specimens repeatedly taken from the tumor failed to identify the presence of malignant melanoma. A positron emission tomography scan and gross changes of the tumor endoscopically observed were useful for detecting the presence of malignant transformation. The patient eventually died of generalized metastatic disease soon after undergoing an esophagectomy. An early diagnosis may therefore be crucial for improving the treatment outcome of esophageal malignant melanoma. Therefore, esophageal melanotic lesions should be carefully followed up even if biopsy specimens repeatedly show no malignancy.     

Primary melanoma of the esophagus

A case of primary malignant melanoma of the esophagus is described. Primary malignant melanoma of the esophagus is rare, reaching only 0.1% of all esophageal tumors. The differential diagnosis is often difficult: the clinical features are similar to those of squamous cell carcinoma and, because of its potentially widespread submucosal growth, the bioptic and/or cytologic examinations are frequently negative. The surgical approach with esophagectomy is actually the treatment of choice. However, the prognosis is poor with a 5-year survival of 4.2%.     

Multiple Synchronous Anorectal Malignant Melanoma Coexisting with Adenocarcinoma of the Sigmoid Colon

Primary anorectal malignant melanoma is an exceptionally rare neoplasm associated with poor prognosis. Anorectal malignant melanoma has been very rarely described with coexisting primary tumors of the colorectum. A 56-year-old female patient was admitted with a history of rectal bleeding. She had experienced increasing constipation and a sense of obstruction in the rectum for 6 months. Flexible rectosigmoidoscopy showed a large, pedinculated polypoid lesion extending from the anal canal to the rectum. She underwent a transanal local excision and was diagnosed with a melanoma of the anorectum with positive margins. Therefore, a formal abdominoperineal resection was performed. In addition to multiple synchronous anorectal malignant melanoma, we incidentally found another primary tumor in the proximal surgical margin of the resected specimen. Histopathologically, the lesion was an intramucosal adenocarcinoma of the sigmoid colon. Postoperatively, the patient received adjuvant chemotherapy of six cycles duration. At present, the patient has completed 18 months of follow-up.     

Primary malignant melanoma of the esophagus

The primary malignant melanoma of the esophagus is a rare tumor. The study aim was to report two cases, one treated by esophagectomy without thoracotomy and the other one by Lewis-Santy type esophagectomy. Both patients had recurrence. One died at the 24th month with liver metastases. The other one who had a cervical invaded lymph node, treated by radio-chemotherapy, is actually in complete remission 9 years after the diagnosis.     

Radiculopathy due to malignant melanoma in the sacrum with unknown primary site

Melanoma is an interesting tumor, showing the appearance of metastasis without any trace of its primary lesion. To report a very rare case of malignant melanoma in the sacrum with unknown primary origin. The authors present a case of a 52-year-old man who was admitted with increasing lower back, left buttock, and left lower extremity pain, and dysuria. Plain radiograph, computed tomography scan, and magnetic resonance imaging revealed a destructive lesion in the sacrum and left ilium, which infiltrated the spinal canal and sacroiliac joint. The tumor cells were immunoreactive for HMB-45. The pathological diagnosis was malignant melanoma. No obvious primary malignant melanoma was detected on the skin surface, on the oral or anal mucosa, or in the fundus oculi. Following radiotherapy and chemotherapy, the severe buttock pain disappeared and the patient was able to walk without impediment. However the patient died nine months after initial diagnosis. Malignant melanoma in the sacrum with an unknown primary site, showing S1 radiculopathy is reported for the first time. The melanoma could have been a metastatic tumor of the sacrum, although the primary site was not detected. The incidence of primary melanoma is increasing faster than any other cancer. Thus treatment of patients with spinal metastasis of melanoma is an important challenge for orthopedic surgeons.

     

Malignant peripheral nerve sheath tumor of the esophagus

BACKGROUND: Sarcomas of the esophagus are rare representing 0.1-1.5% of all esophageal tumors. We report a case of malignant peripheral nerve sheath tumor (MPNST) of the esophagus in a 60-year-old woman. METHODS: The diagnosis was made preoperatively on endoscopic biopsy and confirmed after tumor resection by immunohistochemistry as well as electron microscopy. The patient underwent abdominal-thoracicen bloc esophagectomy with mediastinal lymphadenectomy and intrathoracic esophagogastrostomy. RESULTS: Our therapeutic concept for the first case of a high-grade MPNST (malignant schwannoma) of the esophagus resulted in a recurrence-free interval of 4 years. CONCLUSION: The therapy of choice was abdominal-thoracic en bloc esophagectomy with tumor-free resection margins and esophageal reconstruction with the stomach. After R0-resection we found no indication for adjuvant radio- and/or chemotherapy.     

Malignant melanoma of the chest wall with an unknown primary lesion

We report herein the case of a 66-year-old man who underwent resection and reconstruction of the chest wall due to the presence of a malignant melanoma without a detectable primary lesion. The patient was discharged in good condition after receiving chemotherapy but eventually died of multiple bone metastases 2 years after surgery. Throughout the postoperative course, there were no specific symptoms or findings suggesting the presence of a primary lesion. It was considered likely that the primary tumor was resolved by spontaneous regression after chest wall metastasis had been established.     

Unusual malignant neoplasms of the esophagus. Oat cell carcinoma, melanoma, and sarcoma.

Primary noncarcinomatous malignant neoplasms of the esophagus are uncommon and data concerning treatment and results are sparse. To evaluate the results of therapy in this group, we reviewed the records of 32 patients with primary esophageal malignant tumors of unusual histologic type. Thirteen patients (41%) had sarcoma, eight (25%) melanoma, and 11 (34%) had oat cell carcinoma. Dysphagia was present in 78% (25/32) of the patients for a median of 13 weeks before diagnosis. Location of the esophageal primary tumor was upper third in four patients (12%), middle third in 12 (38%), and lower third in 16 (50%). Treatment consisted of esophagectomy in 10 of 13 patients with sarcoma (77%), seven of eight with melanoma (88%), and three of 11 with oat cell carcinoma (27%). Patients not undergoing resection received chemotherapy or radiation therapy, or both. The 3- and 5-year survival rates were 46% and 23% for sarcoma (median 20 months), 13% and 0% for melanoma (median 5 months), and 0% and 0% for oat cell carcinoma (median 5 months), respectively. Distant disease was the initial form of recurrence in 73% (11/15) of patients undergoing curative therapy. Surgical resection appears indicated for localized primary esophageal sarcoma. Optimum treatment of primary esophageal melanoma is less clear, but surgical resection may be of benefit in selected patients. Esophageal oat cell carcinoma is a systemic disease necessitating systemic therapy with local therapy reserved for palliation of dysphagia.     

Primary malignant melanoma of the esophagus treated with subtotal esophagectomy: a case report

Background

Primary malignant melanoma of the esophagus (PMME) is a rare disease with a poor prognosis. There are few reports of early-stage cases in which tumor invasion reached the lamina propria or muscularis mucosae, as in the present case. A standard treatment for early-stage PMME has not yet been established. The present study aimed to summarize previous reports and to discuss the indications for surgical treatment of early-stage primary malignant melanoma of the esophagus.

Case presentation

A 70-year-old woman with PMME was referred to our hospital. She underwent thoracoscopic and laparoscopic subtotal esophagectomy with lymphadenectomy. The resected specimen showed melanocytosis and junctional activity. Melanoma-specific antigens melan-A, S-100, and HMB45 were detected by immunohistochemical staining. The pathological diagnosis was pT1a-MM, pN0, pM0, and pStage IA. She remains alive without evidence of recurrence 39 months later.

Conclusion

Subtotal esophagectomy with regional radical lymphadenectomy could be recommended to patients with early-stage primary malignant melanoma of the esophagus, and curative surgical resection could improve their prognosis.

     

Differential diagnosis of occult primary malignant melanoma]

Controversy exists in literature about therapy and prognosis of malignant melanoma of unknown primary site. We investigated frequency, differential diagnosis and follow-up of patients with occult primary malignant melanoma treated at the University of Leipzig in 1996. Among 135 patients with malignant melanoma (MM) seven were found without known primary. In two of seven cases the medical history pointed to regression of primary lesion of skin. In another two cases the diagnosis "melanoma" was changed to "lung cancer" after autopsy and in one case there was a relationship to a naevus blue resected nine months before. Recurrences or metastases occurred within six months after therapy. Two patients are still alive free of disease after a follow up of 33 and 24 months. Five patients died from tumor progression between 2 to 14 months postoperatively. Pitfalls in differential diagnosis and ways to find out the primary are discussed. Patients with unknown primary malignant melanoma should be treated similar to those with known primary. Radical surgery is indicated because it's impossible to determine the prognosis of patients with unknown primary malignant melanoma.     

A rare primary sellar melanoma. Case report

The authors report on the case of a 37-year-old woman in whom a primary sellar malignant melanoma mimicking a hemorrhagic pituitary macroadenoma was treated. This entity is exceedingly rare; only five cases are described in the literature. The patient presented with rapid deterioration of vision within a 2-week period. After an ophthalmological diagnosis of chiasmal syndrome was made, magnetic resonance (MR) imaging of the head revealed an intra- and suprasellar mass that was elevating and compressing the optic chiasm. Because of the signal heterogeneity of the lesion a hemorrhagic pituitary macroadenoma was assumed; the lesion was transsphenoidally resected. Histological examination of the specimen showed a malignant melanocytic tumor with immunopositivity for S100 protein and HMB-45. Despite extensive staging no other primary melanotic tumor was found. Thus, a primary sellar melanoma was diagnosed. Postoperative MR images demonstrated no residual tumor. For adjuvant therapy the region around the sella turcica received 40.4 Gy stereotactically guided radiation. A 24-month follow-up examination revealed no tumor recurrence. This represents the sixth case of such a lesion reported in the literature, the third case evaluated using MR imaging, and the first case with a progression-free survival of 24 months. Thus, the authors advocate that management of primary sellar melanoma should include gross-total removal and postoperative stereotactic radiotherapy.     

Transition from meningeal melanocytoma to primary cerebral melanoma. Case report

The authors describe the first case of an intracranial transition of a melanocytoma into a primary malignant melanoma within a short time. A 37-year-old woman presented with progressive brainstem syndrome due to a tumor, originally diagnosed and treated 12 years earlier, that extended from the petroclival area to the anterior craniocervical junction. The histological workup following subtotal tumor resection of the initial tumor had revealed the typical features of a fibrous melanocytic meningioma without increased proliferation. Ten years after the patient had completed treatment for the melanocytic meningioma, control neuroimaging demonstrated growth of the residual tumor with compression of the brainstem. Another neurosurgical intervention revealed a dark tumor of hard consistency. At this time immunohistochemical examinations demonstrated melanocytic features (expression of vimentin, S100 protein, and melan A) of the lesion with focally increased proliferation (5% of Ki-67-positive cells) but no higher mitotic activity. Clinical signs of deterioration along with imaging-confirmed tumor progression precipitated another operation within 7 months. A neuropathological examination revealed epithelial and anaplastic changes and indicated that the MIB-1 indices were greater than 25%. Pleomorphic changes and a focal high mitotic activity led to the diagnosis of a primary cerebral malignant melanoma. The patient's later clinical course consisted of a rapid diffuse meningeal spread of the lesion throughout the entire brain and spine. Despite whole-brain and stereotactic radiation therapy as well as chemotherapy, the patient died 4 months after the last neuropathological diagnosis. Although grossly resembling a meningioma, melanocytomas lack the former's histological and immunohistochemical features. The biological behavior of a melanocytoma is variable and recurrence may happen after subtotal resection, but intracranial transition into a malignant melanoma has not been observed previously.