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 small bowel melanoma. A case report and a review of the literature

Primary malignant melanoma originating from the small intestine is extremely rare. Only a limited number of cases are described in the literature. Most commonly small intestine is affected by metastatic tumors from other primary lesions. We present a case of a 68-years old male diagnosed with primary malignant melanoma as an ulcerated and bleeding mass in the jejunum - located 40 cm away from the Treitz band. In our case the diagnosis was confirmed at laparotomy and enterectomy. Histology revealed a neoplastic infiltration involving the entire intestinal mucosa, with atypia of neoplastic cells and immunoreactivity to HMB45(+), Melan A(+) and S100(+), confirming the diagnosis of melanoma. There was not revealed a primary lesion in the skin, eye, anus, rectum or in other location by the post-operative investigation. An eleven-month close follow-up has not revealed any metastasis. Therefore a definitive diagnosis of primary malignant melanoma was set.     

Primary malignant melanoma of the right colon

The small and large intestines are the most common sites for metastases from cutaneous malignant melanoma. However, primary melanomas in these sites are exceedingly rare. There are several case reports of patients with primary melanoma of the small bowel, but finding of a solitary primary melanoma in the colon is exceedingly rare. We describe a patient that was operated on for bowel obstruction due to colonic intussusception resulting from a right colonic tumor. Histopathological examination confirmed a diagnosis of malignant melanoma. A thorough postoperative investigation did not reveal a primary lesion in any other site. Two years after surgery, there was no evidence for recurrent disease. The treatment and prognosis of metastatic and primary melanoma of the gastrointestinal tract is discussed as well as the embryonic base for development of primary malignant melanoma of the intestine. Primary malignant melanoma of the intestine is an extremely rare lesion that may arise in the large bowel as well. It must be differentiated from other intestinal tumors and mandates a thorough investigation to rule out the possibility of being a metastasis from another more common primary site.     

Primary malignant schwannoma of the small bowel.

Primary malignant schwannoma of the small intestine is an extremely rare disease. We report a primary malignant intestinal schwannoma of the small bowel in a 66-year-old woman, who was admitted to the hospital because of fatigue and anaemia. On clinical examination there was a solid mass at the abdomen. Ultrasound scanning showed an abdominal tumour. Computed tomography showed a mass in the area of the pancreatic tail involving the small intestine. At laparotomy a 10 x 10 cm tumour was found in the proximal jejunum, and 50 cm of the small intestine was resected. The histology of the lesion corresponded to a malignant intestinal schwannoma. The results of previous studies and of our report suggest that the diagnosis of malignant intestinal schwannoma may be difficult, and to exclude malignant conditions laparotomy and surgical excision of the tumour are adequate in the management of this type of lesion.     

Primary Malignant Melanoma of the Oesophagus

Primary malignant melanoma arising in the oesophagus is a rare condition with a dismal prognosis. The diagnosis is often made following surgical resection even though the endoscopic features may be pathognomonic. The classical treatment is oesophagectomy even though the advanced disease stage at the time of presentation and aggressive biological behaviour of the tumour usually results in a fatal outcome. We report the case of a male patient initially diagnosed with squamous oesophageal carcinoma and treated with conventional neo-adjuvant chemo-radiotherapy. Poor clinical and radiological response resulted in a review of the original histology confirming a diagnosis of primary malignant melanoma of the oesophagus. The subsequent alteration in management conferred the patient an improved quality of life. A short review of the literature on primary malignant melanoma of the oesophagus supplements this case report.     

Primary malignant melanoma of the oesophagus

Primary malignant melanoma arising in the oesophagus is a rare condition with a dismal prognosis. The diagnosis is often made following surgical resection even though the endoscopic features may be pathognomonic. The classical treatment is oesophagectomy even though the advanced disease stage at the time of presentation and aggressive biological behaviour of the tumour usually results in a fatal outcome. We report the case of a male patient initially diagnosed with squamous oesophageal carcinoma and treated with conventional neo-adjuvant chemo-radiotherapy. Poor clinical and radiological response resulted in a review of the original histology confirming a diagnosis of primary malignant melanoma of the oesophagus. The subsequent alteration in management conferred the patient an improved quality of life. A short review of the literature on primary malignant melanoma of the oesophagus supplements this case report.     

Gastrointestinal stromal tumors. A case of small intestine stromal tumor (SIST) with an uncertain biological aspect

Tumors of the small intestine are relatively rare. The diagnosis is difficult to establish because the symptoms are vague and non-specific. Although the small intestine constitutes 75% of the length and over 90% of the mucosal surface area of the gastrointestinal tract, only 1 to 2% of gastrointestinal malignancies occur in this segment. Metastases are usually present at the time of diagnosis. The outcome of these patients can be improved if the possibility of a malignant small bowel tumor is considered in all cases of unexplained abdominal pain or gastrointestinal bleeding, especially in younger age. Malignant tumors occur with increasing frequency in distal small bowel with a preponderance of malignant lesions in the ileum compared with the jejunum and the duodenum. Adenocarcinoma is the most common tumor of the primary malignant small bowel tumors, followed by carcinoid, lymphoma and leiomyosarcoma. Mesenchymal tumors of the gastrointestinal tract, traditionally regarded as smooth muscle tumors, have demonstrated different cellular differentiations based on immunohistochemical and ultrastructural features. Therefore the terms leiomyoma and leiomyosarcoma have been replaced by a more encompassing term, gastrointestinal stromal tumor (GIST). The majority of GISTs occurs in the stomach; stromal tumors involving the small intestine (SISTs) are far less common but seem to have greater malignant potential. The clinical a case of a small intestinal stromal tumor (SIST), localised in the jejunum and characterised by an uncertain histological aspect, is presented and a review of the literature is made.     

Primary malignant melanoma of the bronchus.

A rare case of primary malignant melanoma of the bronchus is described. Before considering this diagnosis, metastasis from an occult primary must be convincingly excluded and the tumour should conform to certain guidelines. This patient presented with a solitary lung tumour for which she underwent left lower lobectomy and continues to be tumour free 54 months after surgery. Two possible mechanisms of aetiology are suggested.     

Malignant lymphoma incidentally diagnosed due to the perforation of the small intestine caused by a fish bone: A case report

IntroductionThe ingestion of a foreign body is relatively common. However, it rarely results in the perforation of gastrointestinal tract. We herein report an unusual case of malignant lymphoma incidentally diagnosed after the perforation of the small intestine by a fish bone.Presentation of caseA 90-year-old woman was admitted to our hospital because of abdominal pain and vomiting. Abdominal computed tomography demonstrated free air and ascites in the abdominal cavity. In the pelvic cavity, a radiopaque linear shadow about 35 mm in diameter was shown in the small intestine, and the stricture was exposed to the abdominal cavity. Therefore, a diagnosis of perforation of the small intestine due to ingestion of a foreign body and panperitonitis was made. Emergent laparotomy was performed. The intraoperative findings revealed perforation of the small intestine with a fish bone in the jejunum. Local inflammation at the perforation site was seen, and circulated wall thickness was observed at the distal side of the jejunum. Partial resection of the jejunum and anastomosis of jejuno-jejunostomy was performed. A pathological examination and immunohistochemical study of the resected specimen resulted in a diagnosis of malignant lymphoma of follicular lymphoma Grade 1.DiscussionIt is very difficult to identify the existence malignancy accompanied with gastrointestinal perforation with ingestion of a foreign body.ConclusionIn cases suspected of involving malignancy, careful observation during surgery is needed in order to avoid missing the accompanying malignancy.     

Peritonitis secondary to spontaneous perforation of a primary gastrointestinal stromal tumour of the small intestine: A case report and a literature review

IntroductionA few cases of acute abdomen caused by perforation of small-intestinal gastrointestinal stromal tumours (GISTs) have been reported in the literature.Presentation of caseTogether with a review of the published cases, here we report a case of an elderly patient with peritonitis due to spontaneous perforation of a GIST of the jejunum. An 82-year-old man was admitted to the emergency unit of our hospital with fever and severe abdominal pain. An abdominal enhanced computed tomography scan detected a 6 cm solid mass in the left upper quadrant adherent to a jejunal loop and surrounded by free fluid and free air. Due to the radiological features of the mass, the diagnosis of a perforation of a GIST arising from the jejunum wall was suspected. The patient underwent emergency laparotomy. Intraoperative findings confirmed diffuse peritonitis secondary to jejunal tumour perforation. A segmental resection of the jejunum containing the mass was performed followed by a mechanical end-to-side anastomosis. The histopathologic examination of the mass confirmed the diagnosis of a perforated GIST of the small intestine (high-risk category). The post-operative course was uneventful and the patient was treated with adjuvant imatinib therapy.DiscussionTwenty-one other cases of spontaneous perforation of small intestine GISTs are reported in the literature and are summarized in the present review.ConclusionThe described case is the tip of the iceberg and spontaneous rupture or perforation of GISTs are a far more frequent first presentation of this rare tumour.     

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.

     

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 amelanotic anorectal melanoma--a case report

BACKGROUND: The amelanotic melanoma of the rectal mucosa is very rare with an unfavourable prognosis. The surgical approach is still discussed controversially. Therefore transrectal ultrasound is of major importance in the preoperative staging and postoperative follow-up especially in diagnosis of local recurrence by using the ultrasound-guided, transrectal aspiration. METHODS: In literature 5 cases of amelanotic malignant melanoma were reported. The overall survival time is 10 months after diagnosis. All patients were female. RESULTS: We report about a 55-year-old female patient with an amelanotic melanoma of rectal mucosa. 7 months after a wide local excision of the tumour and interferon therapy in case of the absence of pararectal, inguinal metastases and other metastases the patient developed pararectal metastasis. An abdominoperineal resection and resection of inguinal lymph nodes was performed. Two months later paraaortal lymph nodes were detected. We started chemotherapy with Dacarbazin and with regard of the tumour progress the chemotherapy was changed to Vindesin 25 months after first operation supported by a radiotherapy with 40 Gray. The patient died 36 months after diagnosis. CONCLUSION: The prognosis of primary malignant anorectal melanoma is poor, irrespective of surgical treatment. Wide local resection is the first choice for primary anorectal melanoma. Abdominoperineal resection should be reserved for cases were complete tumour resection is impossible. Chemotherapy, radiotherapy and immunotherapy should be considered in the treatment of anorectal melanoma to influence the overall survival.     

Rare tumour mimicking meninigioma: A case of primary intracranial amelanotic malignant melanoma with negative staining of S100 and HMB‐45

Primary intracranial malignant melanoma is a rare disease, and the imaging findings usually mimic meningioma. Diagnosis is based on histology. Here, we report a case of primary intracranial malignant melanoma in a Chinese patient. A 27‐year‐old man presented with a 1‐month history of headache. Magnetic resonance imaging findings resembled features of a meningioma. Craniotomy with tumour excision was done and confirmed to be S100 and human melanoma black 45‐negative primary intracranial malignant melanoma. There was no extracranial involvement. The 9‐month follow up was reported. To our knowledge, this is the first reported S100 and human melanoma black 45‐negative primary intracranial amelanotic malignant melanoma.     

Primary malignant melanoma of the bladder

Primary malignant melanomma of bladder is extremely rare: 18 cases are reported to date. An 82 year-old man underwent trans-urethral resection of bladder for a bleeding tumor of the posterior wall. Histological diagnosis was melanoma of the bladder. There was no history of previous or regressed cutaneous malignant melanoma. Margins of the bladder lesion contained atypical melanocytes similar to those commonly seen in the periphery of primary mucous membrane lesions. Clinical studies and radiological examinations were negative for other primary site of melanoma. The patient had a bladder recurrence that was consistent with primary tumor and died of widespread disease 9 months after diagnosis.     

Acute abdomen caused by small bowel perforation due to metastatic malignant melanoma

Background-Aim

Malignant melanoma is one of the most common metastatic tumours of the small bowel. Nevertheless, perforation of the small bowel due to these metastatic lesions is an extremely rare condition; only eight cases have been published to date. Reporting such a rare case may provide further data for the management of similar cases.

Case report

A 61-year-old male patient with a known metastatic malignant melanoma (MM) to the liver, lung and adrenal gland who was under treatment with cortisone and monoclonal antibodies (ipilimumab) presented to the emergency department with acute abdomen. Two years earlier, the patient was operated on for a forehead malignant melanoma; he underwent cervical lymph node dissection and was under systemic treatment for distal disease. On admission, the patient was in distress and tachycardic. Clinical examination revealed a painful, rigid abdomen. During the workup, the CT scan showed, apart from the known secondary lesions, free peritoneal air and fluid and at least two further lesions in the jejunum. The patient was taken to theatre and underwent a laparotomy where two lesions were found in the jejunum (one 40 cm and one 60 cm from the Treitz ligament).The distal lesion had caused perforation of the jejunum. There were also suspicious lesions in the segment’s mesentery. We proceeded with resection of the involved part of the small bowel and side-to-side anastomoses. Histology confirmed the lesions to be metastatic MM. The patient had an uneventful postoperative course, and was discharged eight days later in order to be seen by his oncologist.

Conclusion

Despite the fact that perforation of the small bowel due to metastatic melanoma is an extremely rare condition, we should be aware of this probability and it should be considered as one of the working diagnoses in patients with MM who present with an acute abdomen. Prompt surgical resection with intention to cure should be the primary aim of the operating surgeon since long survival can be anticipated.     

Primary malignant melanoma of the female urethra

We report here on a rare case of primary malignant melanoma of the female urethra. A 69-year-old female presented at our hospital with a several month history of dysuria, poor stream, gross hematuria, intermittent blood spots, and a painful mass at the external urethral meatus. The physical examination revealed a soft, small, chestnut-sized lesion through the urethral orifice. The mass was tan colored, ulcerated, covered with necrotic tissue, and protruded from the external urethral meatus. The mass was removed by wide local excision under spinal anesthesia. The pathological diagnosis was malignant melanoma of the urethra. Computed tomography of the abdomen as well as a whole-body bone scan showed no evidence of metastasis. The patient has been free of disease for 6 months postoperatively. We discuss the clinicopathologic features and treatment of this tumor.     

Primary malignant melanoma of the female urethra. Report of a case

Primary malignant melanoma of the female urethra is a rare tumour with great agressivity and poor prognosis. An early diagnosis may benefit of radical surgery and adjuvant immunotherapy with curative effect. We present a sixty-five years old female with a malignant melanoma of urethra and a secondary additional melanoma in vulva, that was treated with both radical ureterectomy and vulvectomy. A bilateral inguinal lymphadenectomy and immunotherapy with alpha-interferon were added. The patient is free of disease one year postoperatively.     

Ileal Malignant Melanoma Causing Intussusception: Report of a Case

Cutaneous malignant melanoma (MM) often metastasizes to the gastrointestinal (GI) tract; however, primary MM of the small intestine is a controversial diagnosis. We report the case of a 76-year-old woman found to have a primary MM in the ileum. After clinical evaluation, the radiological workup, which included magnetic resonance enteroclysis (MRE), revealed a large polypoid intraluminal tumor. She underwent laparotomy and the lesion was excised. Histological examination of the resected specimen revealed morphological and immunohistochemical characteristics of MM and a detailed postoperative examination failed to identify a primary lesion on the skin, anus, oculus, or any other site. The patient died of brain metastasis 6 months after surgery. According to our review of the literature, this is the first case of primary MM of the small intestine diagnosed with the help of MRE.     

人原发性小肠恶性黑色素瘤裸小鼠原位移植肝转移模型的建立

目的为探讨小肠恶性黑色素瘤的发病机制和实验治疗提供理想的动物模型。方法将原发性小肠恶性黑色素瘤患者术中原发灶和肝转移灶新鲜瘤组织块分别植入裸小鼠的小肠黏膜层，观察原位移植成瘤率，移植瘤的侵袭和肝转移率。进行形态学[光镜、电镜和免疫组织化学（免疫组化）]染色体核型和流式细胞分析。结果人小肠恶性黑色素瘤原发灶和肝转移灶新鲜瘤组织均获移植成功。建成一株人原发性小肠（原发灶）恶性黑色素瘤裸鼠原位移植模型（HSIM-0501）和一株人原发性小肠（肝转移灶）恶性黑色素瘤裸鼠原位移植肝转移模型（HSIM-0502）。移植瘤组织病理学为高度恶性黑色素瘤；免疫组化显示S-100蛋白；黑色素瘤单克隆抗体45阳性；电镜下瘤细胞质内可见大量黑色素颗粒及黑色素复合体。染色体众数55～59条；流式细胞DNA指数值1．49-1．61；均为异倍体。HSIM-0501和HSIM-0502分别传至25代和27代；共移植裸鼠317只；肿瘤移植成瘤率和液氮冻存复苏成活率均为100％。HSIM-0501肝转移率为46．2％，淋巴结转移率为36．7％；HSIM-0502肝转移率和淋巴结转移率均为100％。移植瘤在裸鼠小肠内自主侵袭生长，发生血液转移、淋巴结转移和腹腔内种植性转移。结论HSIM-0501和HSIM-0502是首次成功建立的人原发性小肠恶性黑色素裸鼠原位移植肝转移模型，可用于小肠恶性黑色素瘤的发病机制、侵袭和转移及抗转移实验治疗的研究。