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Introduction: Cystic lymphangioma is a benign malformation tumor of the lymphatic system. Its location is variable, and mesenteric localization remains extremely rare. Case presentation: We describe a rare case of cystic lymphangioma of the mesentery in a 26 years old woman. The diagnosis was suspected following an abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI), showing a large polylobulated cyst in contact with the stomach, the tail of the pancreas, the spleen, and the antero-external cortex of the left kidney. The patient underwent laparoscopic surgery with a pericystectomy. Pathological examination confirmed the diagnosis of cystic lymphangioma of the mesentery. The patient's postoperative recovery was uneventful. After a Follow up of one year after surgery, there was no evidence of recurrence. Clinical discussion: Cystic lymphangioma of the mesentery is a benign malformation tumor of the lymphatic system. Its clinical aspects are very polymorphic; the diagnosis is evoked by radiological imaging but requires pathological confirmation. Surgery is the gold standard in the management of this pathology. Conclusion: We highlight the importance of radical surgical resection to avoid Cystic lymphangioma complications and minimize the recurrence risk.
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INTRODUCTION: GIST's are the most common mesenchymal tumor of the gastrointestinal tract, clinically and radiologically heterogeneous, only a histological study can confirm the diagnosis. The link between NF1 and GISTs have been established but not fully elucidated. CASE PRESENTATION: we report 02 cases of NF1 associated GIST, a 60 years old woman with multiples GISTs in the duodenum, proximal jejunum and in the colon presenting an iron deficiency anemia due to chronic bleeding, operated with R0 resection and a low risk of recurrences and a 41 year old male patient with acute abdominal pain with a giant abdominal mass mimicking a hydatid cyst with no relevant medical history, diagnosed at the same time for typical clinical NF1, CT scan showed the cystic mass but did not confirmed its origin, a complete resection of the mass with no capsule fraction was tricky but successful and the histopathology found a high risk of recurrences. The 2 patients received adjuvant imatinib therapy with recurrence free survival at 12 months follow up. Our cases represents a rare entity (multiples GISTs and cystic GIST) within a rarest population (NF1 associated GIST). DISCUSSION: the diagnosis of NF1 is based on typical clinical criterias but the GISTs are known to be the variable, symptomatic or silent, small size or giant. Imaging is based on CT scan with intravenous contrast studying the vascular pattern, the extra intestinal and metastasis localizations. MRI is no superior, but useful in the study of pelvic GISTs and liver metastasis. Histopathology is the only way to confirm the diagnosis with marker staining with CD117 and DOG-1. The emerging imatinib, sunitinib and regorafinib are used as neoadjuvant or adjuvant therapies in GISTs with high or moderate risk of recurrences. No consensual guidelines are yet established for the follow up as the recurrences are more frequent. CONCLUSION: GIST's association to NF1 is established, but the different aspects of the physiopathological, clinical and the treatment haven't been established yet with no larger population to study. We believe that the understanding of the development of this type of tumors within the NF type 1 group would allow a better treatment and follow up and may be can lead to screening.
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BACKGROUND: Sarcomatoid transformation in chromophobe renal cell carcinoma is extremely rare.Gastrointestinal symptoms in renal cell carcinoma are rare and are often secondary to the tumor local growth. While these symptoms are essentially represented by gastrointestinal bleeding, symptoms related to colonic perforation are extremely rare. Only four cases have been described having such a direct invasion which was revealed by gastrointestinal bleeding and that all of them were clear cell- RCC type. No case of chromophobe renal cell carcinoma with direct colonic invasion witch was revealed by colic occlusion, perforation and secondary peritonitis has been found in the literature. Our case report provides more evidence that chromophobe renal cell carcinoma has a propensity to progress to a high-grade spindle cell malignancy with sarcomatoid features gaining an ability to invade other organs such as colon in our case. OBSERVATION: We report a case of a pT4 stage renal cell carcinoma, chromophobe type of solid growth with high nuclear grade (Fuhrman grade 4), extensive sarcomatoid differentiation (60 %), and multifocal tumor necrosis. The sarcomatoid proliferation was invading the colon and reaching its submucosa. Anti-CD10, anti-EMA and anti-cytokeratin 7 immunostains have shown a diffuse and intense staining of the tumor cells. CONCLUSION: The sarcomatoid transformation of chromophoberenal cell carcinoma is extremely rare. Its occurrence worsens the prognosis especially by locoregional aggressiveness.
