Desmoplastic Small Round Cell Tumor of the Lung

Muramatsu, Takashi; Shimamura, Mie; Furuichi, Motohiko; Nishii, Teppei; Takeshita, Shinji; Shiono, Motomi

doi:10.1016/j.athoracsur.2010.08.024

Cited by 9 publications

(5 citation statements)

References 7 publications

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“…Like PNET (and SCLC) it forms part of the spectrum of small blue round cell tumours and typically is composed of cellular solid nests of monomorphic small cells set in sclerotic collagenous stroma. Primary pulmonary desmoplastic small round cell tumor (DSCRT) is very rare with only single case reports published, while pleural examples have been more frequently reported 64 65. The tumour shows a mixed epithelial/mesenchymal immunoprofile, staining for cytokeratin, epithelial membrane antigen (EMA), desmin and vimentin.…”

Section: Large Cell Neuroendocrine Carcinomamentioning

confidence: 99%

Neuroendocrine tumours—challenges in the diagnosis and classification of pulmonary neuroendocrine tumours

Bakker

Thunnissen

2013

J Clin Pathol

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Pulmonary neuroendocrine (NE) proliferations are a diverse group of disorders which share distinct cytological, architectural and biosynthetic features. Tumours composed of NE cells are dispersed among different tumour categories in the WHO classification of tumours and as such do not conform to a singular group with regards to treatment and prognosis. This is reflected by the highly variable behaviour of NE proliferations, ranging from asymptomatic, for instance in diffuse idiopathic pulmonary NE cell hyperplasia and tumourlets, to highly malignant cancers such as small cell lung cancer and large cell NE carcinoma. In this review NE proliferations are described as distinct entities ranging from low grade lesions to high grade cancers. The differential diagnoses are considered with each of the entries. Finally, mention is made of tumours which may show some NE features.

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Section: Large Cell Neuroendocrine Carcinomamentioning

confidence: 99%

Neuroendocrine tumours—challenges in the diagnosis and classification of pulmonary neuroendocrine tumours

Bakker

Thunnissen

2013

J Clin Pathol

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“…4 Very rarely, DSRCT can arise from the head and neck, base of skull, paratesticular region, ovary, brain, or thoracic viscera. 10 Extraperitoneal organ involvement is inconstant and secondary; including lung, 11 liver, 12 kidney, 13 stomach, 14 and pancreas. 15 Liver and lungs are the 2 most common sites of distant metastasis.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor in Transverse Colon: Report of a Rare Case

Huang

Sha

Zhang

et al. 2015

International Surgery

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Desmoplastic small round cell tumor (DSRCT) is an extremely rare, highly aggressive, malignant tumor of undetermined histogenesis. Adolescent males are primarily affected with a typically abdominal or pelvic mass. Diagnosis is based on histologic analysis of biopsy and cytogenetic studies. Owing to the rarity of the tumor and the unusually aggressive presentation, treatment is challenging and has not been standardized. DSRCT has a very poor prognosis, with a median survival range of 17 to 25 months. In this work, we report a case of DSRCT in the transverse colon, which has never before been reported in the literature. Our case study is of a 30-year-old Chinese female who presented with a history of 6 months abdominal discomfort and fatigue. A palpable, hardly mobile mass was detected in the right lower quadrant abdomen by physical examination. Abdominal and pelvic magnetic resonance imaging revealed an 8.0 3 10.5 3 11.1-cm mass with no pulmonary and hepatic metastasis. The patient underwent exploratory laparotomy and transverse colectomy, which revealed a mass in the transverse colon and no metastasis in the peritoneum, greater omentum, or mesentery. Immunohistochemistry findings revealed positive staining for epithelial, mesenchymal, and neural markers, which confirmed the presentation of DSRCT. A 6-month postoperative follow-up failed to find any recurrence. DSRCT is a highly aggressive, malignant, mesenchymal tumor with a very poor prognosis. No consensus has been reached for the treatment protocol of DSRCT. However, debulking surgery with postoperative chemotherapy or radiotherapy might promise more optimistic results on survival.

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“…The disease most commonly involves the omentum and peritoneum, followed by the retroperitoneum [1]. Pleural DSRCT is a kind of extra-abdominal DSRCT, which is rare in the lung [2], paranasal sinuses [3], central nervous system [4], soft scalp tissue [5], salivary glands, and pleura [6]. The clinical manifestations are nonspeci c and usually related to the size and location of the tumor.…”

Section: Introductionmentioning

confidence: 99%

One Case Report of Desmoplastic Small Round Cell Tumor of Pleura

Zhang¹,

Li²,

Su³

et al. 2020

Preprint

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BACGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive malignancy tumor that often affects the abdomen, especially in males. Extra-abdominal DSRCT is extremely rare. CASE REPORT: Here we report a 33-year-old man diagnosed with DSRCT from pleura without any clinical symptoms. Routine blood examination was normal, and an X-ray examination revealed a chest mass during the routine physical examination. The patient was diagnosed by immunohistochemistry combined with clinical features and imaging examination and finally confirmed by pathology. After the diagnosis, we immediately underwent surgery and postoperative radiotherapy. In the latest follow-up, there was no sign of recurrence. CONCLUSION: Because the disease is asymptomatic at the beginning, it is easy to deteriorate, and there is no specific targeted treatment. Therefore, early diagnosis and treatment are particularly important.

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Desmoplastic Small Round Cell Tumor of the Lung

Cited by 9 publications

References 7 publications

Neuroendocrine tumours—challenges in the diagnosis and classification of pulmonary neuroendocrine tumours

Neuroendocrine tumours—challenges in the diagnosis and classification of pulmonary neuroendocrine tumours

Desmoplastic Small Round Cell Tumor in Transverse Colon: Report of a Rare Case

One Case Report of Desmoplastic Small Round Cell Tumor of Pleura

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