Clear Cell Myomelanocytic Tumor (PEComa) of the Duodenum in a Child With a History of Neuroblastoma

2005 ◽  
Vol 129 (11) ◽  
pp. 1484-1486 ◽  
Author(s):  
Tony Mhanna ◽  
Dominique Ranchere-Vince ◽  
Valérie Hervieu ◽  
Dominique Tardieu ◽  
Jean-Yves Scoazec ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Surgical Resection ◽  
Biopsy Specimen ◽  
Clear Cell ◽  
Duodenal Wall ◽  
Pathogenetic Mechanism ◽  
Biopsy Material ◽  
Surgical Specimen ◽  
History Of

Abstract We report herein a case of digestive clear cell myomelanocytic tumor (PEComa) that is unique in its location and presentation. The lesion, located in the duodenal wall, was diagnosed in a child with a history of cervical neuroblastoma that was in remission after surgical resection and chemotherapy. The diagnosis was obtained by examination of a biopsy specimen taken during laparoscopy. The decision was made to perform surgical resection. Examination of the surgical specimen confirmed the diagnosis of PEComa. No metastasis was found. After 2 years of follow-up, the patient is alive, without evidence of metastasis or recurrence. This case highlights the distinctive characteristics of the cells in PEComa, recognizable even on limited biopsy material. It also suggests a possible association between PEComa and neuroblastoma, 2 unusual tumors that belong to the spectrum of lesions known to occur in patients with tuberous sclerosis and that may share a possible common pathogenetic mechanism.

scholarly journals A long-term survivor of clear cell sarcoma-like tumor of the gastrointestinal tract with liver metastasis: a case report

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuhisa Okada ◽  
Yasumitsu Hirano ◽  
Shintaro Ishikawa ◽  
Hiroka Kondo ◽  
Toshimasa Ishii ◽  
...  
Keyword(s):  
Small Intestine ◽  
Lymph Node ◽  
Gastrointestinal Tract ◽  
Liver Metastasis ◽  
Surgical Resection ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma

Abstract Background Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection. Case presentation A 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence. Conclusion Repeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.

Hyalinizing clear cell carcinoma of the nasopharynx operated by trans-oral and trans-palatal approach

2015 ◽  
Vol 129 (S2) ◽  
pp. S95-S97 ◽  
Author(s):  
T Nakashima ◽  
R Yasumatsu ◽  
M Yamauchi ◽  
S Toh ◽  
T Nakano ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Cell Carcinoma ◽  
Surgical Resection ◽  
Clear Cell ◽  
Clear Cell Carcinoma ◽  
Minor Salivary Gland ◽  
Good Prognosis ◽  
Salivary Gland Neoplasm ◽  
Hyalinizing Clear Cell Carcinoma

AbstractBackground:Hyalinizing clear cell carcinoma is a rare minor salivary gland neoplasm. The treatment of choice is surgical resection with or without post-operative radiotherapy. This tumour often demonstrates a good prognosis.Case report:We report a case of hyalinizing clear cell carcinoma arising in the nasopharynx. A 27-year-old female presented with progressive hearing disturbance and tinnitus. On examination, an expansile mass was observed in her nasopharynx. Biopsy was performed and the pathology results returned as clear cell carcinoma.Results and conclusion:Surgical resection was performed trans-orally accompanied by trans-palatal approach. She has no recurrence during more than two years of follow up.

scholarly journals Extramedullar Plasmacytoma Arising from the Nasal Septum

2005 ◽  
Vol 84 (11) ◽  
pp. 720-722 ◽  
Author(s):  
Byoung J. Baek ◽  
Seong W. Kim ◽  
Hoon Park ◽  
Jin K. Park ◽  
Kyung Y. Han ◽  
...  
Keyword(s):  
Nasal Septum ◽  
Biopsy Specimen ◽  
Extramedullary Plasmacytoma ◽  
Screening Tests ◽  
Bence Jones Protein ◽  
Light Chain Type ◽  
History Of ◽  
Chain Type ◽  
Rule Out

We report a rare case of extramedullary plasmacytoma of the nasal septum in a 65-year-old woman. She presented with a 2-month history of left-sided nasal obstruction and intermittent blood-tinged nasal crusting. Nasal endoscopy revealed that a dark-red mass had arisen from the nasal septum; no evidence of invasion to adjacent tissues was seen. A biopsy specimen was diagnosed as a plasmacytoma (kappa light chain—type). Serum and urine electrophoresis failed to detect any myeloma component or Bence Jones protein. All other screening tests to rule out multiple myeloma were negative. These findings confirmed the diagnosis of extramedullary plasmacytoma. The mass was completely removed via an endoscopic approach. No recurrence was noted at the 2-year follow-up.

scholarly journals A277 DISCREPANCIES IN EUS-FNA CYTOPATHOLOGY AND SURGICAL SPECIMEN PATHOLOGY FOR HIGH RISK PANCREATIC MUCINOUS CYSTIC LESIONS: A CASE SERIES

2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 155-156
Author(s):  
S Li ◽  
M Monachese ◽  
P James
Keyword(s):  
High Risk ◽  
Surgical Resection ◽  
Case Series ◽  
Surgical Pathology ◽  
Low Grade ◽  
Cystic Lesions ◽  
Cystic Neoplasms ◽  
Cross Sectional ◽  
Surgical Specimen

Abstract Background Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) is the primary method of sampling pancreatic cystic lesions with reported specificity near 100% for diagnosing malignancy. Discrepant positive malignant cytopathology with final surgical pathology of pancreatic cystic lesions has not previously been described. Aims To present a case series and review the literature regarding the implications of positive malignant cytology with discrepant surgical pathology for high risk pancreatic mucinous cystic lesions. Methods Patient demographics, clinical history, procedure details, pathology evaluations and follow-up were collected. A thorough literature review was performed. Results Three patients with high-risk pancreatic cystic lesions on cross-sectional imaging underwent EUS-FNA evaluation. None of these patients had a history of pancreatitis. Cytology was reported as positive for adenocarcinoma in all patients by separate gastrointestinal cytopathologists. All patients underwent surgical resection. The pathology for all resected specimens were reported as intraductal papillary mucinous neoplasm. The resected cysts for two patients demonstrated foci of high-grade dysplasia and the third noted low grade dysplasia. All surgical pathology underwent consensus review by three separate gastrointestinal pathologists. None of the patients were treated with adjuvant chemotherapy. All patients have been followed post-operatively with surveillance magnetic resonance imaging with no evidence of recurrence to date (median follow-up time 239 days, range 133 – 447 days). Conclusions This phenomenon sheds light on the potential for variable interpretations of EUS-FNA cytopathology and surgical resection pathology for high risk pancreatic cystic neoplasms. EUS-FNA may identify foci of adenocarcinoma that is not seen on surgical pathology specimens. Further research is required to examine the long-term outcomes of these patients. Funding Agencies None

scholarly journals Primary Extra-Osseous Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Amanda H. Seipel ◽  
Hiba Mechahougui ◽  
Nicolas Mach ◽  
Frédéric Triponez ◽  
William C. Faquin ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Ultrasound Examination ◽  
Fusion Transcript ◽  
Differentiated Thyroid Carcinoma ◽  
Surgical Specimen ◽  
Pet Ct ◽  
History Of ◽  
Poorly Differentiated ◽  
Pet Ct Scan

AbstractExtra-osseous Ewing sarcoma (ES) is a rare and aggressive malignant tumor found in a variety of organs. Primary ES of the thyroid is exceedingly rare and few cases have been documented to date. We describe the case of a 54-year old woman with a history of breast carcinoma in whom a unique hypermetabolic left thyroid nodule was identified during a follow-up PET-CT scan. An ultrasound examination showed a hypoechogenic nodule of 3.7 cm. A cytological diagnosis of poorly differentiated thyroid carcinoma was made, and a total thyroidectomy was performed. The surgical specimen revealed a poorly differentiated neoplasm composed of medium-sized cells with scant cytoplasm, expressing pancytokeratin, CD99 and NKX2.2 but lacking p63 and p40 expression. Molecular analysis revealed a EWSR1-FLI1 fusion transcript supporting the diagnosis of a primary extra-osseous ES of the thyroid. The patient received adjuvant chemotherapy and has no evidence of recurrent disease.

Diethylstilbestrol exposure during pregnancy with primary clear cell carcinoma of the cervix in an 8-year-old granddaughter: a multigenerational effect of endocrine disruptors?

2020 ◽  
Author(s):  
Laura Gaspari ◽  
Françoise Paris ◽  
Nathalie Cassel-Knipping ◽  
Julia Villeret ◽  
Arnauld Verschuur ◽  
...  
Keyword(s):  
Clear Cell ◽  
Causal Link ◽  
Clear Cell Adenocarcinoma ◽  
Fertility Sparing Surgery ◽  
Fertility Sparing ◽  
History Of ◽  
Exposure During Pregnancy ◽  
Multigenerational Effects ◽  
First Time

ABSTRACT To date, vaginal/cervical clear cell adenocarcinoma (CCAC) has not been reported in the granddaughters of women treated with diethylstilbestrol (DES) during pregnancy. We present an 8-year-old girl with a history of severe vaginal bleeding who was diagnosed with cervical CCAC. She underwent fertility-sparing surgery and radiotherapy. No sign of recurrence was detected throughout a 10-year follow-up. Her grandmother had received DES therapy during pregnancy with the patient’s mother. Although no direct causal link is demonstrated, this case raises for the first time, the hypothesis of multigenerational effects of DES in girls and strongly suggests the need to follow the granddaughters of DES-treated women.

scholarly journals Soft tissue Rosai-Dorfman disease of the posterior mediastinum

2009 ◽  
Vol 35 (7) ◽  
pp. 717-720 ◽  
Author(s):  
Antônio Luiz Penna Costa ◽  
Natália Oliveira e Silva ◽  
Marina Pamponet Motta ◽  
Rodrigo Abensur Athanazio ◽  
Daniel Abensur Athanazio ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Surgical Excision ◽  
Posterior Mediastinum ◽  
Extranodal Involvement ◽  
Surgical Specimen ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
Paravertebral Mass

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

Rectal ganglioneuroma in a dog

1999 ◽  
Vol 35 (2) ◽  
pp. 107-110 ◽  
Author(s):  
ME Reimer ◽  
MS Leib ◽  
MS Reimer ◽  
GK Saunders ◽  
SA Johnston
Keyword(s):  
Gastrointestinal Tract ◽  
Surgical Resection ◽  
Large Bowel ◽  
Clinical Signs ◽  
Successful Outcome ◽  
First Report ◽  
History Of ◽  
The Masses

An 18-month-old, spayed female Australian terrier cross was presented with a 10-month history of chronic large bowel diarrhea. Ulceration and two proliferative masses in the rectum were seen on colonoscopy. Surgical resection was performed to remove the masses, and the dog recovered without complications related to surgery. Histopathology was consistent with the diagnosis of ganglioneuroma. The dog had no clinical signs of disease within three months of surgery and was completely normal 2.5 years after diagnosis. This is the first report providing follow-up and successful outcome of a ganglioneuroma in the gastrointestinal tract of a dog.

La valutazione RM della sclerosi tuberosa

2003 ◽  
Vol 16 (3) ◽  
pp. 547-550
Author(s):  
C. Uggetti
Keyword(s):  
Tuberous Sclerosis ◽  
Neoplastic Transformation ◽  
Pathogenetic Mechanism ◽  
Clinical Correlations ◽  
Asymptomatic Subjects ◽  
Changes Over Time ◽  
Over Time ◽  
Subependymal Nodules ◽  
Rule Out

The myriad brain abnormalities characterising tuberous sclerosis are all the result of the same pathogenetic mechanism, a genetic impairment of the germinative matrix. MR scanning plays a major role in the diagnosis and follow-up of patients who must be monitored up to the age of twenty to rule out the possible neoplastic transformation of subependymal nodules. MR scans should also be offered to the parents of children with tuberous sclerosis to disclose asymptomatic subjects. Starting from a total of 36 scans in a personal series of 24 patients, we describe the best sequences for brain investigation, the different MR findings and their changes over time with possible clinical correlations.

The correlation between molecular characteristics and treatment outcomes in patients (pts) with grade 2-3 (G2-3) gliomas.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e14551-e14551
Author(s):  
Jaleh Fallah ◽  
Vineeth Tatineni ◽  
Martin C. Tom ◽  
Wei Wei ◽  
Deborah Park ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Surgical Resection ◽  
Tumor Grade ◽  
Treatment Modalities ◽  
Rank Test ◽  
Molecular Characteristics ◽  
Idh Mutation ◽  
Kaplan Meier ◽  
History Of

e14551 Background: In a retrospective study, we investigated the correlation between the molecular characteristics and treatment outcomes in pts with G2-3 glioma. Methods: Pts with G2-3 glioma and known IDH mutation status who were diagnosed between 1994 and 2017 were analyzed. In most of the pts, IDH mutation was determined by immunohistochemistry only. Overall survival (OS) was defined as the date of biopsy/surgical resection to the date of last follow up or death. OS was estimated by Kaplan-Meier method and compared by log rank test. Results: 606 pts with G2 (81%) or G3 (19%) glioma were included. The median age at diagnosis was 38 years (Interquartile range 27-52), 55% of the pts were male, 83% were white, 47% had IDH-mt tumor and 67% underwent surgical resection. The median follow-up was 55.6 months (mo). The median OS (mOS) in pts with IDH mutated (mt) and IDH wild type (wt) tumor were 201 and 128 mo, respectively. The predictors of worse OS in pts with IDH-mt tumor included G3, receipt of chemotherapy or radiation therapy (RT), bilateral disease and lack of 1p/19q codeletion. The determinants of worse OS in pts with IDH-wt tumor included male gender, receipt of chemotherapy or RT, history of prior malignancy, smoking, G3, astrocytoma histology, no surgical resection, EGFR amplification, and lack of 1p/19q codeletion. The mOS by IDH, 1p/19q, and MGMT status is summarized in the table. RT and chemotherapy were more commonly used among pts who had G3 glioma and those who underwent biopsy only. Conclusions: Tumor grade continues to be a determinant of pt outcomes in the setting of molecularly defined gliomas. Presence of 1p/19q codeletion is a predictor of favorable OS in pts with G2-3 glioma. Surgical resection is a determinant of OS in pts with IDH-wt G2-3 gliomas, but not in pts with IDH-mt tumor. The worse OS in pts who were treated with RT or chemotherapy is likely due to the use of these treatment modalities in more aggressive tumors and in those who only had biopsy. [Table: see text]

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