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A case report of unilocular cystic mucinous tubular and spindle cell carcinoma with mural tumor nodule.

ABSTRACT: Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of renal cell carcinoma (RCC). Classic type of MTSCC is characterized by small, elongated tubules lined by clear cuboidal or spindle cells with mucinous stroma. The neoplastic cells are always low-grade histological features. But, unclassified variants of MTSCC have also been reported, e.g., mucin-poor, papillary, high grade, and sarcomatoid variants. We present the first case of simple cyst with mural nodule exhibiting the histological features of mucin-poor MTSCC. We should be aware that MTSCC can arise in a cystic renal lesion.

SUBMITTER: Nagano N

PROVIDER: S-EPMC6742803 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

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A case report of unilocular cystic mucinous tubular and spindle cell carcinoma with mural tumor nodule.

Nagano Nahoko N Ishikawa Noriyoshi N Nagase Mamiko M Fukushima Takafumi T Shiina Hiroaki H Maruyama Riruke R

Urology case reports 20190905

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of renal cell carcinoma (RCC). Classic type of MTSCC is characterized by small, elongated tubules lined by clear cuboidal or spindle cells with mucinous stroma. The neoplastic cells are always low-grade histological features. But, unclassified variants of MTSCC have also been reported, e.g., mucin-poor, papillary, high grade, and sarcomatoid variants. We present the first case of simple cyst with mural nodule exhibiting the histo ...[more]

PMID: 31528542

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Project description:BackgroundMucinous tubular and spindle-cell carcinoma (MTSCC) is a rare kidney cancer subtype with limited cases reported in the literature. We report on outcomes of 25 patients with this variant who were managed at our institution.Materials and methodsThe institution database was queried, and clinical data extracted for patients with MTSCC. Molecular features examined included next-generation sequencing with Memorial Sloan Kettering Integrated Mutation Profiling of Actionable Cancer Targets and allele-specific copy number analysis using the Fraction and Allele-Specific Copy Number Estimates from Tumor Sequencing (FACETS) algorithm in a subset of patients.ResultsAll patients underwent primary tumor-directed therapy (nephrectomy = 23, cryoablation = 2). Metastases were diagnosed in 6 patients (24%), 3 (12%) of whom had de novo metastatic disease. Five of 6 patients with metastatic disease had high-grade histological features compared with 0 of 19 nonmetastatic patients (83% vs. 0%; P < .001, Fisher exact test). Three-year overall survival from diagnosis was 84.8% (95% confidence interval, 59.6-94.9) with a median follow-up time of 3.9 years (range, 1 month to 10.3 years). Three deaths occurred, all from metastatic disease. Four patients received systemic therapy with time to treatment failure ≤6 months across different agents with the exception of 1 patient with prolonged response with sunitinib treatment (30.6 months). The most frequent molecular alterations were neurofibromin 2 mutations (n = 2; 40%), germline alterations (n = 2; 40%) including checkpoint kinase 2 and BRCA2 DNA repair associated mutations, multiple chromosomal copy number losses, and mismatch repair deficiency in 1 patient.ConclusionMTSCC is characterized by localized tumors treated successfully with primary tumor-directed therapy. However, patients with high-grade histological features were more likely to develop metastatic disease with limited responses to standard therapies.

Project description:Mucinous cystic tumors of low malignant potential (MCTLMP) are rare urachal neoplasms. The morphological characteristics and clinical prognosis of MCTLMP is similar to that of mucinous cystic tumors occurring in the ovary and appendix. After complete resection, almost no cases of recurrence or metastasis have been reported. Because MCTLMP is rare, it may be missed in the clinic. MCTLMP can lead to the formation of pseudomyxoma peritonei (PMP), which manifests as the widespread production of mucus in the abdominal cavity and makes the disease complex or difficult to diagnose. At present, only 3 cases of MCTLMP with PMP have been reported in the literature. In the present study a fourth case of urachal MCTLMP in a 74-year-old male that resulted in widespread PMP is presented. Initially, a multilocular cystic lesion was revealed in the urachal duct area at the anterior upper margin of the bladder after a patient, experiencing lower abdominal pain, was imaged. As revealed using light microscopy, the cyst was lined with a mucous columnar epithelium, and part of the epithelium indicated pseudolamellar hyperplasia and papillary structures. The cells indicated mild atypia and low mitotic activity. There was no stromal infiltration of tumor cells, and a large amount of mucous exudate was observed. As preoperative computed tomography examination suggested the presence of a large amount of ascites and there were increased levels of blood tumor markers, carcinoembryonic antigen and carbohydrate antigen 125, clinicians considered that the diagnosis maybe a malignant tumor of the urachal gland with peripheral dissemination. However, the diagnosis of MCTLMP with PMP was confirmed by histopathological examination. The mass was completely removed, along with part of the peritoneum and bladder wall as these were within the tumor margin. The appendix appeared normal during surgery. A one off dose of intraperitoneal infusion chemotherapy with 1,000 mg 5-fluorouracil was performed after surgery. No recurrence was observed during the 8-month follow-up period.

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A case report of unilocular cystic mucinous tubular and spindle cell carcinoma with mural tumor nodule.

Publications

A case report of unilocular cystic mucinous tubular and spindle cell carcinoma with mural tumor nodule.

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