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.     

治疗方式对肛管直肠恶性黑色素瘤预后的影响

目的 探讨不同治疗方式对肛管直肠恶性黑色素瘤的预后影响.方法 回顾性分析1965-2007年收治的60例肛管直肠恶性黑色素瘤患者的临床资料,并对预后进行生存分析和COX风险因素分析.结果 60例患者中,肿瘤发生于直肠者50例,发生于肛管者10例.53例行手术切除治疗.对行单纯手术的23例患者和术后辅助化疗、放疗、生物治疗等综合治疗的30例患者的资料进行生存分析,总生存率差异无统计学意义(X2=0.078,P>0.05).53例手术病例中,37例行Miles术,16例行局部扩大切除术,两种术式生存分析差异无统计学意义(X2=1.464,P>0.05).风险因素分析结果提示,肿瘤浸润深度为危险因素,治疗方式为保护因素.结论 手术切除是肛管直肠恶性黑色素瘤的主要治疗手段,对肛管直肠恶性黑色素瘤病变局限者,应首选局部扩大切除术;病变深度和治疗方式是影响预后的风险因素.     

Solitary metastatic gallbladder malignant melanoma originated from the nasal cavity: A case report

INTRODUCTIONSolitary gallbladder metastasis of malignant melanoma is rare and generally originates from skin melanoma. We report a case of gallbladder metastasis from a malignant melanoma of the nasal mucosa that was surgically treated.PRESENTATION OF CASEA 77-year-old Japanese woman diagnosed with malignant melanoma of the left sinonasal cavity three years ago underwent follow-up PET–CT and FDG uptake was detected only at the gallbladder. The nasal melanoma had been stable for the last 1.5 years after chemoradiation and her general condition was good. Cholecystectomy was performed with partial liver resection. Lymphadenectomy of the hepatoduodenal ligament was also performed. The tumor was soft and whitish, and was microscopically diagnosed as a poorly differentiated malignant melanoma that was not similar to the nasal cavity melanoma. No further metastasis is observed for more than 13 months after surgery.DISCUSSIONIn the literature, cutaneous melanoma is described as the origin of most metastatic gallbladder melanomas; however, no skin lesion was evident in this case. We believe that the poorly differentiated compartment of the nasal melanoma had metastasized to the gallbladder.CONCLUSIONFor patients with melanomas and gallbladder tumors, the possibility that metastasis could occur should be considered when selecting optimal treatment. Even when original melanoma is present, surgical treatment for gallbladder metastasis may be useful depending on the patient's conditions.     

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.     

The Diagnostic Value of Endoscopic Ultrasonography and Magnetic Resonance Imaging for Anorectal Malignant Melanoma: Report of a Case

A 74-year-old Japanese woman presented with a 3-month history of anal bleeding. Proctoscopy revealed an unusual polypoid lesion with focal pigmentation at the dentate line, which was histologically diagnosed as a malignant melanoma. Whole-body clinical and radiographic evaluations revealed no alternative primary source. Endoscopic ultrasonography (EUS) showed well-delineated hypoechoic tumors invading the muscularis propria, and magnetic resonance imaging (MRI) revealed regional lymphadenopathy. Following this evaluation, an abdominoperineal resection with regional lymphadenectomy was performed. The excised tumor was histologically confirmed to be malignant melanoma, and its depth and metastatic lymph nodes proved to have been accurately and precisely evaluated by the preoperative examinations. Thus, EUS and MRI are useful preoperative diagnostic tools for the tumor staging of primary anorectal malignant melanomas, as for other rectal tumors. Received: February 1, 2002 / Accepted: September 3, 2002 Reprint requests to: H. Sashiyama     

Rare nodular malignant melanoma of the heel in the Caribbean: A case report

IntroductionMalignant melanoma of the heel is a rare melanoma subtype with incidence rates that reflect the complex relationship between sun exposure at certain geographic locations, individual melanin levels and overall melanoma risk. It is oftentimes characterized by poor prognosis because of delays in presentation resulting in longitudinal tumor invasion, lymph node involvement and metastasis.Presentation of caseA 59-year-old woman was admitted to the Eric Williams Medical Sciences Complex, Trinidad and Tobago with a 5 mm pruritic lesion on her left heel. At presentation, the lesion was asymmetric with border irregularities, color heterogeneity, with dynamics in elevation and overall size. She was subsequently diagnosed with malignant melanoma with left inguinal lymphadenopathy. A single stage wide local excision (WLE) of the left heel lesion with a split-thickness skin graft (STSG) and a left inguinal lymphadenectomy were performed. Dacarbazine (Bayer) was administered post operatively.DiscussionGlobally, the incidence of malignant melanoma is rapidly increasing, particularly, in countries like Trinidad and Tobago with a significant population of non-fair skinned individuals. There is need for strategic initiatives to increase patient adherence in these populations.ConclusionThe rarity of malignant heel melanomas heightens the need for increased patient awareness and greater clinical surveillance to ensure early diagnosis and treatment.     

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.

     

A first reported case of metastatic anorectal amelanotic melanoma with a marked response to anti-PD-1 antibody nivolumab: A case report

IntroductionAnorectal amelanotic melanoma (AAMM) is a rare disease with poor prognosis. A standard treatment strategy for AAMM has not been established.Presentation of caseWe report a case of successful treatment of AAMM with nivolumab. A 67-year-old man was referred for colonoscopy which revealed type I tumor in the rectum. AAMM was diagnosed with immunostaining histopathological biopsy findings. Enhanced computed tomography (ECT) revealed the rectal tumor without distant organ metastasis. We performed laparoscopy-assisted abdominoperineal resection. ECT at three months after surgery revealed liver metastases and right ischial bone metastasis. Although we had started dacarbazine monotherapy, black spots that were suspicious of skin metastases had appeared on systemic skin. Therefore, we started nivolumab therapy. ECT at 3 months after initiation of nivolumab showed shrinkage of liver metastasis. We have continued strict follow-up every 2 months and checked no oncologic progression at 17 months after initiation of nivolumab.DiscussionThe anti-PD-1 antibody have improved prognosis of malignant melanoma. However, there are no reports of nivolumab for treatment of AAMM.ConclusionsOur patient is the first reported case of AAMM treated with nivolumab. We consider that nivolumab will be effective for non-cutaneous malignant melanoma.     

Incidence of Sentinel Node Metastasis in Patients With Thin Primary Melanoma (#1 mm) With Vertical Growth Phase

Background: Patients with thin primary melanomas (#1 mm) generally have an excellent prognosis. However, the presence of a vertical growth phase (VGP) adversely impacts the survival rate. We report on the rate of occurrence of nodal metastasis in patients with thin primary melanomas with a VGP who are offered sentinel lymph node (SLN) biopsy.Methods: Among 235 patients with clinically localized cutaneous melanomas who underwent successful SLN biopsy, 71 had lesions 1 mm or smaller with a VGP. The SLN was localized by using blue dye and a radiotracer. If negative for tumor by using hematoxylin and eosin staining, the SLN was further examined by immunohistochemistry.Results: The rate of occurrence of SLN metastasis was 15.2% in patients with melanomas deeper than 1 mm and 5.6% in patients with thin melanomas. Three patients with thin melanomas and a positive SLN had low-risk lesions, based on a highly accurate six-variable multivariate logistic regression model for predicting 8-year survival in stage I/II melanomas. The fourth patient had a low- to intermediate-risk lesion based on this model. At the time of the lymphadenectomy, one patient had two additional nodes with metastasis.Conclusions: VGP in a melanoma 1 mm or smaller seems to be a risk factor for nodal metastasis. The risk of nodal disease may not be accurately predicted by the use of a multivariate logistic regression model that incorporates thickness, mitotic rate, regression, tumor-infiltrating lymphocytes, sex, and anatomical site. Patients with thin lesions having VGP should be evaluated for SLN biopsy and trials of adjuvant therapy when stage III disease is found.Presented at the 52nd Annual Meeting of the Society of Surgical Oncology, Orlando, Florida, March 4–7, 1999.     

Giant leiomyosarcoma of the rectum with lymph node metastasis: A case report and review of the literature

IntroductionLeiomyosarcoma of the gastrointestinal tract is very rare, with a reported frequency of less than 0.1% of all malignancies of the colorectum. It is important to diagnose leiomyosarcoma definitively by immunohistochemical profiling of smooth muscle actin, desmin, and CD34. True leiomyosarcoma of the colorectum diagnosed by immunnohistochemical profiling is extremely rare that only 13 reports have been published in reviews of resected gastrointestinal mesenchymal tumors after 1998. In addition, lymph node involvement is rare in patients with leiomyosarcoma. Herein we report an aggressive case of LMS in a rectosigmoid lesion with lymph node metastasis.Case presentationA 76-year-old woman visited our hospital complaining of intermittent anal bleeding that had lasted 5 months. Image studies aiming at examining the cause of her anal bleeding revealed a tumor located between the right ovary, uterus, and the rectosigmoid. Histopathology of biopsied materials from the colonoscopy suggested a malignant tumor of mesenchymal origin. Surgical resection was performed with curative intent. The tumor was diagnosed as leiomyosarcoma by pathological examination. Moreover, one of the 31 regional lymph nodes retrieved was metastasized by leiomyosarcoma. Eight months later, follow-up CT scans revealed multiple recurrent lesions in the liver and peritoneum. Despite systematic chemotherapy, she deceased 12 months after the surgery.ConclusionIt is crucial to diagnose leioyosarcoma precisely based on immunohistochemistry, and thereby distinguish it from GIST. Although lymph node metastasis is rare, lymphadenectomy appears to be important for high-risk LMSs to perform R0 resection. Further investigation on leiomyosarcoma cases so far is required to establish standard treatment strategies.     

Adenocarcinoma arising from an anal gland—Report of a case

INTRODUCTIONAdenocarcinoma arising from an anal gland is extremely rare. Most anal canal cancers are squamous cell carcinoma, and adenocarcinoma is infrequently diagnosed. Diagnostic criteria and the standard treatment for adenocarcinoma of the anal canal have not been clearly defined, in part because of the rarity of this lesion.PRESENTATION OF CASEAn 84-year-old man who presented with a piece of tissue prolapsing from the anus. An incisional biopsy showed adenocarcinoma, and an abdomino-perineal resection was then performed. Cytokeratin 7 (CK7), cytokeratin 19 (CK19) stained positive in the specimen, suggesting that the tumor developed from an anal gland. The patient was discharged after surgery without any complications.DISCUSSIONExact diagnostic criteria for adenocarcinoma of the anal canal have not been previously described. In the present case, CK7 and CK19 were stained, and the tumor showed positivity for both of these markers, which is compatible with the staining patterns of anal gland origin cancer. Radical resection is recommended rather than local resection, because of the tumor's high recurrence rate. Some authors recommend combined modality treatment with preoperative or postoperative chemoradiotherapy because of the high rate of distant recurrence.CONCLUSIONThe preoperative diagnosis of adenocarcinoma arising from an anal gland is not easily established. However, it may be possible to suspect an anal glandular adenocarcinoma based on a meticulous physical examination, appropriate diagnostic studies and pathological findings on biopsy.     

Wide local excision for anal GIST: A case report and review of literature

IntroductionGastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin commonly detected in stomach and small bowel. GIST arising primarily from the anal canal is extremely rare. Due to the malignant potential, these tumors are treated with radical surgery like abdominoperineal resection. But with the advent of imatinib therapy and a better understanding of the tumor biology, some cases have been successfully treated with wide local excision.Presentation of caseWe describe a case of a 70-year-old lady presenting with a 2 cm mass in the anal canal. Endoanal ultrasound revealed a well-circumscribed solid nodule in the intersphincteric space. The patient was successfully treated by wide local excision and adjuvant therapy with imatinib mesylate.DiscussionOnly 14 confirmed cases of primary anal GIST have been reported in the literature. It appears as a well circumscribed hypoechoic mass arising from the intersphincteric space encroaching into the lumen on endorectal ultrasound. Lymphadenopathy is absent. Anal sphincters get involved as the lesion increases in size. Treatment is often planned based on the extent of the disease, the mitotic rate, patient’s general condition and willingness for a permanent colostomy.ConclusionSmall lesions (<2 cm) with low mitotic rate may be successfully managed by local excision. Radical surgery should be reserved for large, aggressive tumors.     

直肠肛管恶性黑色素瘤的临床特征分析

目的了解原发性直肠肛管恶性黑色素瘤的临床特点。方法回顾性分析9例原发性直肠肛管恶性黑色素瘤的临床资料,并复习文献。结果直肠肛管恶性黑色素瘤以女性多见,平均发病年龄56岁,病程5.8个月;首发症状以血便为最常见,其次为肛门肿物突出。94.7%的直肠肛管恶性黑色素瘤在距离肛缘5.0cm范围内;肿瘤最大径(3.3±2.1)cm;其中54.5%可活动;有19.1%的肿瘤表面光滑;6.6%的肿瘤质地软;14.0%同期发现转移,肝转移最常见,腹股沟淋巴结转移其次;的病例出现误诊,超过者被误诊为良性疾病;手术治疗中以Miles术为主,经肛门局部切除术其次。结论直肠肛管恶性黑色素瘤极易误诊。手术治疗为主。     

Multiple primary melanomas: Implications for screening and follow-up programs for melanoma

Background: Once individuals are diagnosed with malignant melanoma, they are at an increased risk of developing another melanoma when compared with the normal population. Methods: To determine the impact of an intensive follow-up protocol on the stage of disease at diagnosis of subsequent primary melanomas, a retrospective query was performed of an electronic medical record database of 2,600 consecutively registered melanoma patients. Results: Sixty-seven patients (2.6%) had another melanoma diagnosed at the time of presentation to the clinic or within 2 months (synchronous) and another 44 patients (1.7%) developed a second primary melanoma during the follow-up period (metachronous). For the 44 patients diagnosed with metachronous lesions, the Breslow mean tumor thickness for the first invasive melanoma was 2.27 mm compared with 0.90 mm for the second melanoma. The first melanomas diagnosed are thicker by an average of 3.8 mm (p=0.008). The mean Clark level for the initial melanoma was greater than the mean level for subsequently diagnosed melanomas (p=0.002). Twenty-three percent of the initial melanomas were ulcerated, whereas only one of the second primary lesions showed this adverse prognostic factor (p=0.002). Conclusions: Once individuals are diagnosed with melanoma, they are in a high-risk population for having other primary site melanomas diagnosed and should be placed in an intensive follow-up protocol consisting of a complete skin examination. Presented at the 48th Annual Cancer Symposium of The Society of Surgical Oncology, Boston, Massachusetts, March 23–26, 1995.     

Laparoscopic Surgery for Anorectal Malignancies Other than Carcinoma

Objective:

Numerous concerns have been raised relative to the appropriateness of laparoscopic surgery for cure of rectal adenocarcinomas. However, because of their rarity, little information exists about the role of laparoscopy for other anorectal malignancies. We report the outcome of five patients who underwent laparoscopic surgery for other anorectal malignancies.

Methods:

All patients who underwent laparoscopic surgery for treatment of non-carcinomatous anorectal malignancy were assessed by means of endoscopic, radiological and histopathologic diagnostic tests.

Results:

Two patients with anorectal melanoma and one with anal leiomyosarcoma underwent laparoscopic abdominoperineal resection. A laparoscopic loop ileostomy was performed for an HIV-positive patient with rectal Kaposi''s sarcoma. Another patient with anorectal melanoma had intraoperative identification of distant liver metastasis and therefore underwent diagnostic laparoscopy instead of an intended abdominoperineal resection. There were no intraoperative laparoscopic complications. During the follow-up period three patients who underwent abdominoperineal resection were alive, one of whom had rectal melanoma and developed liver metastasis without local recurrence. The two patients with distant liver metastasis and rectal Kaposi''s sarcoma died 46 days and five months after surgery, respectively. There were no port-site or local recurrences.

Conclusion:

Laparoscopic abdominoperineal resection for non-carcinomatous anorectal malignancies is technically feasible and avoids many of the concerns associated with attempted curative laparoscopic resection of carcinoma.     

A case of malignant melanoma of the penis

We experienced a 53-year-old male with malignant melanoma of the penis. A lentigo was noted in the dorsal penis in 1980. On February 4, 1982, he was referred to our Hospital with the complaint of enlargement of the lentigo of the penis and swelling of the bilateral inguinal lymph nodes. Lymph node biopsy disclosed metastasis of the malignant melanoma. He was hospitalized for treatment of the lesion. On physical examination at admission, a black tumor, 3 cm in diameter, with necrosis at the center and unclear demarcation was observed in the dorsal part, very close to the coronal sulcus, slightly to the left. Many black spots were scattered around the tumor. Curative operation could not be expected because of metastasis to the lymph nodes and the lung. Combination chemotherapy with DTIC, ACNU and vincristine, and immunotherapy were carried out. CT scan, lymphangiography and intravenous pyelography confirmed right hydronephrosis due to compression of enlarged retroperitoneal lymph nodes. Agenesis of the left kidney was confirmed through these evaluations. Right ureterostomy was performed and a metastatic lesion was identified in the resected ureter. After the operative treatment, chemotherapy and immunotherapy were performed again, but their effects were limited, and he died on May 6, 1982. Metastatic lesions in the lungs, liver, brain, skin and agenesis of the left kidney were confirmed by autopsy. Eleven malignant melanomas of the penis including our case have been reported in the Japanese literature. This case is considered to be secondary to an ureter tumor.     

The curious incident of 3 melanomas and their possible origins—A case report and review of literature

BackgroundWe describe an unusual case of 2 intra-parenchymal breast melanomas with a concomitant subcutaneous melanoma in the ipsilateral upper limb and no definite primary lesion.Case reportOur patient is a 40-year-old Chinese female who presented with a breast lump in her left breast for which excision biopsy showed melanoma. A PET-CT revealed a second lesion in her breast. A left upper arm nodule with no overlying skin changes was also noted. She underwent a mastectomy and excision biopsy of the upper arm nodule. Histology showed that the second breast lesion was also a melanoma, while the arm nodule contained melanoma cells within a fibrous capsule.ConclusionThe presence of a melanoma in the breast should prompt a close and meticulous search for a primary lesion and potential signs of metastasis. Encapsulated subcutaneous nodules can be attributed to replaced lymph nodes or subcutaneous melanoma which can be either primary dermal melanoma or metastasis from an unknown primary.     

Primary anorectal malignant melanoma

A case of primary anorectal malignant melanoma seen in a 46 year old woman is presented herein. Her most marked symptoms were bloody stools and anal pain. Endoscopic examination indicated a tumor with ulceration but without pigmentation in the anorectal region. Histologic examination of the biopsied specimens showed spindle-shaped cells with atypia proliferating in a bandlike arrangement, as in leiomyosarcoma. An abdominoperineal resection was done and detailed histological examination of the tumor comfirmed the nature of the tumor to be malignant melanoma. The postoperative immunochemotherapy consisted of Dimethyl-Triazeno-Imidasole-Carboxamide (DTIC), Amino-Methyl-Pyrimidinyl-Methyl-Chlorethyl-Nitrosourea-Hydrochloride (ACNU), Vincristine (VCR) and OK-432. The patient has been well without recurrence for fifteen months following her operation through the continuous administration of these agents.     

Thoracic metastasis of malignant melanoma of unknown primary: A case report and literature review

IntroductionMetastatic melanoma of unknown primary (MUP) is an unusual entity found in distant sites without evident skin lesion. We report a case of 45-year-old woman who underwent monobloc resection of a metastatic thoracic malignant melanoma of unknown primary, and who is currently under immunotherapy without local or distant recurrence during a follow-up of 18 months. We demonstrate through this case that R0 resection of an MUP associated with immunotherapy improves the prognosis and survival in these patients.Case reportThis is a 45-year-old woman who underwent monobloc resection of a mass carrying the anterior arch of the second left rib associated with a wedge resection of a nodule at the left upper lobe. Histology confirmed that it was a malignant melanoma. Her history was negative for melanocytic lesions, physical examination and imaging had failed to identify a primary lesion. The patient is currently under nivolumab for Stage IV melanoma and does not present any complications or recurrence during the long term follow up.DiscussionMetastatic melanoma of unknown primary (MUP) is a melanocytic lesion in distant sites in the absence of apparent skin involvement and is rare, accounting for 3, 2% of all incident melanomas as well as being yet poorly understood in terms of pathogenesis (Bae et al., 2015) [1]. MUP is clinically understudied, investigators to date have reported largely on the use of localized treatment for MUP (surgery or radiotherapy), while the efficacy of systemic therapy in MUP patients remains unexplored. Clinical trials of immunotherapy and targeted therapy in patients with advanced cutaneous melanoma have not explicitly reported response rates specific to MUP patient subgroups due to its low incidence and lack of annotation. MUP's response to these now FDA-approved therapies could add to the discussion of MUP's elusive biological characteristics, as well as aid in making clinical recommendations (Utter et al., 2017).ConclusionMetastatic MUP is an extremely rare entity which is still poorly understood, few cases are described in the literature, its treatment remains controversial and there are no specific treatment recommendations for patients with MUP. Several authors recommend local treatment when possible and tend to apply similar strategies for patients with paired stage primary known melanoma (PKM).     

Free disease long-term survival in primary thoracic spine leiomyosarcoma after total en bloc spondylectomy: A case report

IntroductionTo describe an unusual primary vertebral leiomyosarcoma in thoracic spine.Presentation of caseAn isolated lesion of the T11 vertebra in a 62-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade malignant lesion. TC-guided biopsy failed thus open incisional biopsy was needed. A diagnosis of low-intermediate mesenchymal sarcoma was made. A total en bloc spondylectomy of T11 was performed with three-column reconstruction. The histology and immunostaining showed the appearance of leiomyosarcoma. After diagnosis, post-operative radiation therapy was performed. Metastatic lesion was ruled out by CT scans of the chest, abdomen and pelvis, in addition to total body radionuclide scanning and 18-F-FDG-PET. After five years of follow-up, no signs of local recurrence, metastasis or distant lesions suggesting a primary lesion were observed.DiscussionVertebral primary leiomyosarcoma is exceedingly rare. Primary vertebral leiomyosarcoma diagnosis must be performed when the metastatic origin is excluded. For the treatment of primary tumors, total en bloc spondylectomy (TES) is the technique of choice to achieve marginal or wide tumor resection, decrease the risk of local recurrence and remote lesions and increase survival.ConclusionsA well-planned pre-operative study and a wide surgical excision can result in local tumor control and long-term survival. This case presents the longest disease-free survival period of a primary leiomyosarcoma in spinal location after total en bloc spondylectomy.