Amelanotic primary malignant melanoma of the esophagus: report of a case treated with minimally invasive esophagectomy and adjuvant chemotherapy

Amelanotic primary malignant melanoma of the esophagus (PMME) is extremely rare. We report a case of amelanotic PMME that was accurately diagnosed and subsequently treated by radical resection with adjuvant chemotherapy. A 69-year-old woman was admitted to our hospital because of dysphagia. Endoscopic examination revealed a tumor without pigmentation in the upper thoracic esophagus, and melanosis in the lower thoracic esophagus. Tumor biopsy showed proliferation of malignant spindle cells with conspicuous nucleoli. Immunohistochemically, the tumor cells were positive for melanosomes (HMB45). The tumor was diagnosed as PMME. Positron emission tomography and computed tomography showed accumulation of the tracer only in the primary lesion. The preoperative diagnosis according to the extent of the tumor, whether cancer cells have spread to lymph nodes, and whether metastasis has occurred (TNM) classification was malignant melanoma in the upper third of the esophagus, T2N0M0 stage IIA. The patient underwent minimally invasive video assisted esophagectomy in the prone position with three-field lymph node dissection. On the resected specimen, the malignant cells were positive for HMB45, KIT, and melan-A. Masson-Fontana staining demonstrated no melanin pigmentation. The patient was diagnosed with amelanotic PMME and received adjuvant chemotherapy, consisting of dacarbazine, nimustine, cisplatin, and tamoxifen. The patient showed no systemic metastasis, and is alive 18 months after the operation with no evidence of recurrence.     

Primary malignant melanoma of the esophagus: a case report and review of the literature.

SUMMARY. The purpose of this report is to describe a new case of primary malignant melanoma of the esophagus (PMME) and to review the recent literature. A 75‐year‐old man underwent an esophagoscopy for a 3‐month history of dysphagia and weight loss. A pigmented polypoïd mass in the lower third of esophagus was discovered, identified by biopsy as a malignant melanoma. No pigmented lesions of the skin or eyes were observed and a diagnosis of PMME was made. A total transhiatal esophagectomy was carried out and 12 months after the operation the patient is disease‐free. PMME is a rare neoplasm, with only 238 cases having been reported in the literature. Although characterized by an aggressive biological behavior, esophagectomy can result in a 5‐year survival rate of up to 37% of cases, whereas chemotherapy, immunotherapy and radiation therapy currently have no major role in treatment.     

Primary malignant melanoma of the esophagogastric junction: A case report

Rationale:Most gastrointestinal melanomas are metastatic from an oculocutaneous primary lesion; however, primary gastrointestinal melanomas have been found in all levels of the gastrointestinal tract. We present the case of Primary malignant melanoma of the esophagus and discuss the diagnostic methods, differentiation from metastatic lesions and treatment options.Patient concerns:A 78-year-old male patient presented with fresh blood vomiting and tarry stools for 1 day.Diagnoses:Esophagogastroduodenoscopy of this patient revealed a tumor ∼4 cm in size at the cardia side of the esophagogastric junction with dark-red and gray pigmentation. Immunohistochemical stains of the biopsy specimens were positive for S-100 and HMB-45, which are specific markers of melanoma.Interventions:Laparotomy with proximal gastrectomy was performed by the surgeon. Histological examination of the surgical specimen revealed the tumor arose from the distal esophagus with invasion of the proximal stomach. Primary malignant melanoma of the esophagus was diagnosed after a full skin and ophthalmic examination and positron emission tomography, which revealed no lesions elsewhere in the body.Outcomes:No tumor recurrence was noted at the 1-year follow-up.Lessons:Primary malignant melanoma of the esophagus is an extremely rare but highly aggressive tumor. The special pattern of pigmentation should be recognized while performing endoscopy. Early detection and radical resection of the tumor are critical to ensure favorable outcomes.     

Early-stage primary malignant melanoma of the esophagus detected simultaneously with esophageal squamous cell carcinoma

Primary malignant melanoma of the esophagus is a rare disease. The majority of patients are diagnosed at an advanced stage, and only a few are detected at an early stage. We herein describe a case of early-stage primary malignant melanoma of the esophagus that was detected simultaneously with early-stage primary esophageal squamous cell carcinoma. Both tumors were detected during esophagogastroduodenoscopy for heartburn. The malignant melanoma tumor was a nevus-like flat-type lesion in the upper thoracic esophagus, and the squamous cell carcinoma was a slightly depressed lesion in the abdominal esophagus. The tumor was resected by thoracoscopic esophagectomy. Histologically, the invasion of both tumors was limited to the mucosal layer, and no lymph node metastasis was detected. Immunohistochemically, the malignant melanoma cells were strongly positive for HMB-45, melan-A, and S-100 protein. The patient has survived without recurrence for 17?months after the operation.     

A Case of preoperatively diagnosed mucosal carcinoma of the terminal ileum successfully treated by laparoscopy‐assisted surgery

We report herein a case of primary early ileal adenocarcinoma diagnosed definitively by colonoscopy before surgery and treated by laparoscopy‐assisted ileocecal resection. A 66‐year‐old man visited our hospital because of fecal occult blood. Colonoscopy revealed a relatively clearly demarcated, flat elevation in the terminal ileum; it was slightly red, surrounding a hemorrhagic spot, and had a small depression at its center. The lesion was diagnosed on biopsy as a well‐differentiated adenocarcinoma. Laparoscopy‐assisted ileocecal resection was performed. Macroscopically, the resected specimen appeared as a partially elevated, partially depressed, reddish lesion on Kerckring's fold. It was 1.5 × 1.0 cm and located 3.5 cm from the ileocecal valve. Microscopically, the tumor was diagnosed as a type IIa + IIc well‐differentiated adenocarcinoma; it was confined to the mucosa, and lymph node metastasis was not found. The patient's postoperative course was uneventful, and he is now alive, well and showing no evidence of recurrence 1 year after surgery. Early detection of ileal cancer depends on careful colonoscopic observation of the terminal ileum, where small bowel cancer usually locates, even during routine examination. To our knowledge, this is the first report of laparoscopic surgery for preoperatively diagnosed early ileal carcinoma.     

SYNCHRONOUS MULTICENTRIC SIGNET‐RING CELL CARCINOMA OF THE STOMACH

A case of simultaneous multicentric signet‐ring cell carcinoma (SRC) of stomach is presented. Initially, an early gastric cancer (IIc) was diagnosed and this was cured with distal gastrectomy. Thirty‐eight months after the operation, follow‐up endoscopy revealed a tiny mucosal discoloration lesion, which was diagnosed as a minute SRC focus with biopsy. This was successfully treated with endoscopic mucosal resection (EMR). Nine months later, gastroscopy discovered another small mucosal lesion and it was again diagnosed as a tiny SRC lesion, which was also successfully treated with EMR. After a further 2 months, endoscopy showed three small mucosal lesions (discoloration) similar to previous lesions, two of which proved to be SRC with biopsy. As these lesions distributed widely over the remnant stomach, total gastrectomy of the residual stomach was performed. Pathological examination demonstrated a total of 22 simultaneous multifocal SRC lesions, which were all very small mucosal cancer. These cancers, including previously EMR‐treated ones, seemed to develop in a multicentric manner, as they were diagnosed within 11 months. This case also indicated that even subtle endoscopic findings should vigorously be sought and, if in doubt, be biopsied in order to locate gastric cancer early enough for minimal invasive curative treatment to be feasible.     

Esophageal and multiple gastric cancers treated by endoscopic mucosal resections

A case of successful endoscopic therapy of superficial esophageal and multiple gastric cancers in an 87‐year‐old man is reported. A slightly elevated superficial cancer 10 mm in diameter in the proximal stomach (type 0‐IIa) was detected by esophagogastroduodenoscopy (EGD) and treated by endoscopic mucosal resection (EMR) in September 1999. Four months later, in January 2000, a slightly depressed superficial cancer 10 mm in diameter in the proximal esophagus (type 0‐IIc) was found in follow‐up EGD and treated by EMR. Further, in August 2000, a slightly elevated superficial cancer 10 mm in diameter in the distal stomach (type 0‐IIa) was found in follow‐up EGD and treated by EMR. Microscopic examination of these resected specimens showed that invasion was limited to the mucosal layer, and confirmed that no lymphatic or vascular permeation had occurred. No endoscopic or histological recurrence was detected for 33 months after the most recent EMR for gastric cancer. As multiple primary cancers in the upper gastrointestinal tract are not rare in Japan, periodic endoscopic follow up is important for early detection of recurrence of new primary cancers.     

A case of primary malignant melanoma of the esophagus with long-term survival

Primary malignant melanoma of the esophagus is uncommon, and its prognosis is poor compared to that of cutaneous malignant melanoma. Here we describe a case of primary malignant melanoma of the esophagus with a long-term survival. A 52-year-old woman received an upper gastrointestinal endoscopy and an upper gastrointestinal series for a dull back pain and dysphagia. A pigmented polypoid tumor in the esophagus was discovered and diagnosed pathologically as a malignant melanoma on the biopsied specimen. After effective chemotherapy with cisplatin (CDDP), the patient underwent surgical operation. A subtotal esophagectomy with three-field lymph node dissection was performed through a right thoracotomy. No distant metastasis including liver and lung was found, and histopathological examination revealed no lymph node metastasis. Postoperatively, six courses of chemotherapy with CDDP were performed. The patient has been alive without any problems for more than 11 years postoperatively.     

Non‐transplant surgical approach to liver‐based hereditary haemorrhagic telangiectasia: a first report

A 55‐year‐old woman with hereditary haemorrhagic telangiectasia (HHT) underwent a left lateral liver bisegmentectomy (removal of segments 2 and 3) for hepatic‐based arteriovenous malformations. This lesion determined a progressive fatigue and invalidating effort dyspnoea. The postoperative course was uneventful and the patient is currently doing well at 4 years after surgery. To our knowledge, this is the first case of hepatic‐based HHT treated with liver resection. This anecdotal report should promote the evaluation of this approach in order to define its role in the treatment of liver involvement in this rare disease.     

Gastric vascular ectasia treated by endoscopic mucosal resection

An 80‐year‐old woman consulted our hospital complaining of general weakness. She had iron deficiency anemia, and upper gastrointestinal endoscopy revealed a small lesion accompanying a small amount of fresh bleeding in the stomach. Close observation of the lesion revealed that it was composed of a local assembly of dilated microvessels. The diagnosis of this patient was gastric vascular ectasia causing anemia. Endoscopic ultrasonography demonstrated that the lesion involved the mucosal and submucosal layers of the stomach, and that there were no large vessels inflowing to or outflowing from the lesion. In the present case, we attempted endoscopic mucosal resection (EMR). The lesion was completely resected by only one procedure of EMR without complications such as bleeding. After the endoscopic treatment, iron deficiency anemia improved. Follow‐up endoscopy performed 1 year later revealed that there was no residual or recurrent lesion. Although there have not been any published reports describing the use of EMR for gastric vascular ectasia, EMR may be a useful endoscopic treatment for this condition.     

Endoscopically managed superficial carcinoma overlying esophageal lipoma

The occurrence of superficial carcinoma over a benign tumor of the esophagus is considered to be rare. Only a few reports have been reported and all of them were treated surgically. We now report one case of superficial carcinoma overlying an esophageal lipoma that was successfully resected endoscopically. The patient was a 61‐year‐old man who had no symptoms. A submucosal tumor was found at the thoracic esophagus by upper gastrointestinal endoscopy. The top of the tumor was slightly depressed with mild redness and its surface was irregular. This depressed lesion was not stained by iodine. Histological examination of endoscopic biopsy revealed squamous cell carcinoma. To completely remove this tumor in a single fragment, we used an insulation‐tipped electrosurgical knife. An en bloc resection of the tumor was completed without complications.     

Esophageal carcinoid tumor treated by endoscopic resection

The present report describes a rare case of esophageal carcinoid tumor that was treated by endoscopic resection. A 43‐year‐old woman underwent esophagogastroduodenoscopy at her family clinic for screening of the upper digestive tract and a small lesion resembling a submucosal tumor was detected in the lower esophagus. A biopsy sample from the lesion was diagnosed as esophageal carcinoid tumor and the patient visited our hospital for detailed examination. The tumor was approximately 3 mm in diameter and its surface appeared to be covered with normal squamous epithelium. The tumor had a shiny reddish surface without ulceration or erosion. Magnifying endoscopy with narrow‐band imaging showed structures resembling reticular vessels under the epithelium. Endoscopic ultrasonography depicted the tumor as a low‐echoic mass within the lamina propria. Computed tomography did not detect the tumor and no metastatic lesions were evident in other organs. With the patient's informed consent, the tumor was resected using endoscopic submucosal dissection, with a sufficient free margin in both the vertical and horizontal directions. Magnifying endoscopic examination showed the resected tumor to have abundant reticular vessels. Finally, the tumor was diagnosed immunopathologically as an esophageal carcinoid tumor (neuroendocrine cell tumor, grade 1), without lymphatic or vascular invasion.     

A case of anorectal malignant melanoma appearing 1 year after endoscopic mucosal resection of a rectal adenocarcinoma

We present a case of a 61-year-old woman who underwent endoscopic mucosal resection (EMR) for early-stage colorectal cancer. However, because the condition of the horizontal margin of the resected tumor was unknown, she further underwent local transanal excision. Lower gastrointestinal endoscopy performed 1 year later showed protruding lesions both on the scar tissue and in the vicinity. Biopsy revealed malignant melanoma. She then underwent laparoscopic abdominoperineal resection and colostomy. This was an extremely rare case of adenocarcinoma complicated by malignant melanoma after resection.     

Colorectal melanoma: review of two distinct forms of presentation

Malignant melanoma of the colon and rectum is an infrequent disease. Primary anorectal melanoma accounts for 0.1-4.6% of all malignant neoplasms of the anal canal. Melanoma metastatic to the colon is symptomatic only in 4.4% of patients with a primary melanoma at another site and most of these tumors are diagnosed postmortem. We report two cases of colorrectal malignant melanoma. The first case concerned a patient with rectal bleeding who was diagnosed with a rectal lesion compatible with melanoma. Abdominoperineal resection was performed due to positivity of the sentinel lymph node. We discuss the utility of sentinel lymph node detection in this kind of tumor. In the second case, we discovered a polyp compatible with metastatic melanoma in the transverse colon in a patient with a previous diagnosis of melanoma. In both surgical specimens, the diagnosis of melanoma was confirmed by positivity for protein S-100, Melan-A and HMB-45.     

Endoscopic mucosal resection for early esophageal cancer. A case report

With the rapid progress of the diagnostic techniques, especially endoscopy, the discovery rate of early superficial cancer of the esophagus has increased markedly. Relatively recently introduced, endoscopic mucosal resection (EMR) is now widely used in the treatment of early stage cancer of the esophagus. We used EMR in the case of a patient who was incidentally diagnosed with a type IIa early esophageal cancer using a band-ligating device. We had no serious complications. At the histopathological examination we noticed that the carcinoma involved the upper third of the submucosal layer. For this reason the patient was given additional therapy (radiotherapy), too. A month after the EMR, at the histological examination we found no local signs of recurrent disease.     

A case of verrucous squamous cell carcinoma of the esophagus

We report a rare case of verrucous squamous cell carcinoma (VSC) of the esophagus. A 73-year-old woman presented to our hospital with dysphagia. On gastrointestinal endoscopy, a white mass with a cauliflower-like appearance (3.5 cm in diameter) was detected in the upper esophagus at 23 cm from the incisors. Histological examination of a biopsy specimen revealed squamous epithelial hyperplasia and no malignancy. During 17 months of follow-up by repeated endoscopy, the lesion showed no marked changes in appearance. However, reexamination of the specimen obtained by partial endoscopic mucosal resection (EMR) using a jumbo biopsy forceps revealed very well differentiated squamous cell carcinoma corresponding to VSC. The clinical and histopathological characteristics of esophageal VSC are discussed on the basis of the published literature.     

Mucosa‐associated lymphoid tissue lymphoma occurring in the transverse colon

A case of primary mucosa‐associated lymphoid tissue lymphoma (MALToma) occurring in the transverse colon is reported. Endoscopic examination revealed a flat and oval‐shaped submucosal tumor‐like lesion, approximately 2 cm in diameter in the transverse colon. The tumor extracted by laparotomy showed proliferation of centrocyte‐like cells positive for L26 immunostaining. The patient was diagnosed as having MALToma in the transverse colon. In this case, although the tumor invasion was limited to the submucosal layer, lymph node involvement was seen. Awareness of the clinical presentation and familiarization of colorectal MALToma are important. We emphasize that even if either antibiotic therapy or surgery is chosen for the therapy of colorectal MALToma, lymph node involvement should generally be taken into consideration, although the tumor invasion was limited to the submucosal layer.     

Primary malignant melanoma of the esophagus successfully treated by an esophagectomy followed by systemic chemotherapy

Primary malignant melanoma of the esophagus is a rare disease that tends to demonstrate an extremely poor prognosis. We herein describe a case of primary malignant melanoma of the esophagus that was successfully treated. The tumor was incidentally detected by a barium swallow examination performed during a routine medical checkup. The tumor was resected by a transhiatal radical esophagectomy. Histologically, the tumor metastasized to one of the perigastric lymph nodes, although tumor invasion was confined to the submucosa at the primary site. Immunohistochemically, the tumor cells were strongly positive for S100 protein and HMB-45. Postoperatively, systemic chemotherapy consisting of DTIC, ACNU, and VCR was administered. The patient has survived without recurrence for 12 months after these treatments. A transhiatal esophagectomy followed by systemic chemotherapy may therefore be an effective treatment for potentially curable primary melanoma of the esophagus.     

Endoscopic ultrasound‐guided fine needle aspiration biopsy in esophageal and mediastinal diseases: Clinical indications and results

Background: Endoscopic ultrasound‐guided fine needle aspiration biopsy (EUS‐FNAB) was developed to attain endosonographical images in real time in endoscopic biopsy, just like in percutaneous biopsy with ultrasonic or computer‐tomographical images. The results of EUS‐FNAB in esophageal and mediastinal diseases were evaluated and clinical indications of this technique were investigated. Methods: The study was performed in 58 patients, consisting of 30 with esophageal or mediastinal tumors and 28 requiring mediastinal lymph node examination. The instruments were linear array EUS transducer PEF‐703FA (Toshiba‐Fujinon, Tokyo, Japan) and 21G Endosonopsy (Hakko Shoji, Tokyo, Japan). The aspirated material was recovered on a filter paper and was formalin‐fixed to be examined histopathologically. Results: The tumors measured 6–60 mm (mean 29 mm). Collection of tissue was successful in 95% of the patients, and diagnostic accuracy was 95%. The biopsy specimen was satisfactory to establish histological diagnosis in every case of 27 patients with malignant diseases. No complication was experienced. Conclusion: The EUS‐FNAB is indicated in cases where the technique of EUS‐guided puncture is required, or is considered optimum in view of the safety and in cases where histological diagnosis is critical for the decision of treatment program. In many cases of mediastinal diseases, not even a detection of lesion is feasible without this technique, let alone a collection of tissue. The EUS‐FNAB is thus performed as a first choice of examination to obtain biopsy specimen in such cases. Endoscsopic ultrasound‐guided lymph node puncture is applied in order to assess the appropriateness of endoscopic mucosal resection (EMR) in patients with esophageal cancer, to follow up the patients after EMR and chemoradiotherapy (CRT), and to evaluate the efficacy of neoadjuvant CRT.     

CHARACTERISTICS AND ROLE OF ENDOSCOPIC MUCOSAL RESECTION USING CAP‐FITTED PANENDOSCOPE FOR EARLY GASTRIC CANCER

Background: It is accepted in Japan and in the gastric cancer treatment guidelines that small gastric mucosal cancers without lymph node metastasis can be curatively treated by endoscopic mucosal resection (EMR). Various techniques of EMR for early gastric cancer have been reported, and in the present study, the results of EMR using cap‐fitted panendoscope (EMRC) for early gastric neoplastic lesions are examined, and the characteristics and the role of EMRC procedure are discussed. Methods: From August 1992 to March 2003, 181 gastric neoplastic lesions in 155 patients were treated by EMRC at the Department of Surgery, Esophagogastric Division, Tokyo Medical and Dental University. The frequency of residue and the cause of residue following EMRC for 181 gastric neoplastic lesions were examined. Five‐year survival rates were calculated and compared according to the 49 patients who underwent en bloc resection and the 49 patients who underwent planned fractionated resection by EMRC for early gastric cancer. Results: There was residue in nine (5%) of 181 lesions treated by EMRC. Residues from four elevated lesions resulted from muscular resection and insufficient additional resection; residues from five depressed lesions resulted from incorrect diagnosis of the spread of the lesions prior to resection and insufficient additional resection. In the EMRC patients, the survival rates for 49 patients treated by en bloc resection (93%) and 49 patients by planned fractionated resection (91%) were not significantly different. Conclusion: The EMRC technique, that is en bloc and planned fractionated resection by EMRC procedure, has an important role as an easy and curative EMR method for almost all of the indicated early gastric cancers by the gastric cancer treatment guidelines with no limitations from the lesion sites.