Vulvar lipoma: A rare tumour

Parathyroid carcinoma is a rare tumour, which is often difficult to diagnose. This is especially true in patients with pre-existing tertiary hyperparathyroidism of end-stage renal disease. A case is presented of parathyroid carcinoma in a haemodialysis patient withunusual thymic involvement. After demonstrating the difficulty in pre-operative diagnosis and risk of recurrence, the importance of non-surgical treatment options is discussed and the investigation of individual tumour chemosensitivity is introduced, which is new to this type of cancer.

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Glomangiopericytoma of the sphenoethmoid complex

BMJ Case Reports ◽

10.1136/bcr-2020-236048 ◽

2020 ◽

Vol 13 (12) ◽

pp. e236048

Author(s):

Larry Shemen ◽

Wayne Yan ◽

Adnan Hasanovic ◽

Jiankun Tong

Keyword(s):

Surgical Resection ◽

World Literature ◽

Vascular Lesion ◽

Common Type ◽

Optimal Treatment ◽

Rare Tumour ◽

Complete Surgical Resection ◽

The World

Sinonasal glomangiopericytoma is a rare sinonasal tumour accounting for less than. 5% of all sinonasal tumours. This tumour often presents as another, more common type of vascular lesion and is similarly prone to haemorrhage. The optimal treatment includes complete surgical resection. We, herein, present two such cases adding to the world literature of this rare tumour.

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Hepatic angiosarcoma in south glamorgan: a rare tumour still appearing 40 years after occupational exposure

Gut ◽

10.1136/gut.2011.239301.525 ◽

2011 ◽

Vol 60 (Suppl 1) ◽

pp. A247-A248

Author(s):

R. Stacey ◽

M. Hassell ◽

J. T. Green

Keyword(s):

Occupational Exposure ◽

Rare Tumour ◽

Hepatic Angiosarcoma

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Composite phaeochromocytomas—a systematic review of published literature

Langenbeck s Archives of Surgery ◽

10.1007/s00423-021-02129-5 ◽

2021 ◽

Author(s):

K. Dhanasekar ◽

V. Visakan ◽

F. Tahir ◽

S. P. Balasubramanian

Keyword(s):

Case Reports ◽

Descriptive Analysis ◽

Management Strategies ◽

Gastrointestinal Symptoms ◽

Rare Condition ◽

Epidemiological Data ◽

Human Case ◽

Rare Tumour ◽

Palpable Mass

Abstract Introduction Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre’s records and presents a systematic literature review of composite phaeochromocytomas. Methods In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist. Results There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4–86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5–168) months. Conclusion Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.

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Definitive carbon ion radiotherapy for tracheobronchial adenoid cystic carcinoma: a preliminary report

BMC Cancer ◽

10.1186/s12885-021-08493-1 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Jian Chen ◽

Jingfang Mao ◽

Ningyi Ma ◽

Kai-Liang Wu ◽

Jiade Lu ◽

...

Keyword(s):

Adenoid Cystic Carcinoma ◽

Group Performance ◽

Primary Tumour ◽

Performance Status ◽

Eastern Cooperative Oncology Group ◽

Tumour Response ◽

Carbon Ion Radiotherapy ◽

Rare Tumour ◽

Carbon Ion ◽

Adenoid Cystic

Abstract Background Tracheobronchial adenoid cystic carcinoma (TACC) is a rare tumour. About one-third of patients miss their chance of surgery or complete resection as it is mostly detected in the advanced stage; hence, photon radiotherapy (RT) is used. However, the outcomes of photon RT remain unsatisfactory. Carbon ion radiotherapy (CIRT) is thought to improve the therapeutic gain ratio; however, the outcomes of CIRT in TACC are unclear. Therefore, we aimed to assess the effects and toxicities of CIRT in patients with TACC. Methods The inclusion criteria were as follows: 1) age 18–80 years; 2) Eastern Cooperative Oncology Group Performance Status 0–2; 3) histologically confirmed TACC; 4) stage III–IV disease; 5) visible primary tumour; and 6) no previous RT history. The planned prescription doses of CIRT were 66–72.6 GyE/22–23 fractions. The rates of overall survival (OS), local control (LC), and progression-free survival (PFS) were calculated using the Kaplan-Meier method. Treatment-induced toxicities and tumour response were scored according to the Common Terminology Criteria for Adverse Events and Response Evaluation Criteria in Solid Tumors, respectively. Results Eighteen patients with a median age of 48 (range 30–73) years were enrolled. The median follow-up time was 20.7 (range 5.8–44.1) months. The overall response rate was 88.2%. Five patients developed lung metastasis after 12.2–41.0 months and one of them experienced local recurrence at 31.9 months after CIRT. The rates of 2-year OS, LC, and PFS were 100, 100, and 61.4%, respectively. Except for one patient who experienced grade 4 tracheal stenosis, which was relieved after stent implantation, no other ≥3 grade toxicities were observed. Conclusions CIRT might be safe and effective in the management of TACC based on a short observation period. Further studies with more cases and longer observation are warranted.

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Rare tumour of neck

Transactions of the Royal Academy of Medicine in Ireland ◽

10.1007/bf03170628 ◽

1891 ◽

Vol 9 (1) ◽

pp. 428-429

Author(s):

W. R. Graves

Keyword(s):

Rare Tumour

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Development of a genomic predictive model for cholangiocarcinoma using copy number alteration data

Journal of Clinical Pathology ◽

10.1136/jclinpath-2020-207346 ◽

2021 ◽

pp. jclinpath-2020-207346

Author(s):

Inês Tavares ◽

Ricardo Martins ◽

Ilda Patrícia Ribeiro ◽

Luísa Esteves ◽

Francisco Caramelo ◽

...

Keyword(s):

Survival Analysis ◽

Copy Number ◽

Patient Management ◽

Copy Number Alteration ◽

Comparative Genomic Hybridisation ◽

Support Vector ◽

Comparative Genomic ◽

Support Vector Machine Algorithm ◽

Rare Tumour ◽

Genomic Model

AimsCholangiocarcinoma (CC) is a rare tumour arising from the biliary tract epithelium. The aim of this study was to perform a genomic characterisation of CC tumours and to implement a model to differentiate extrahepatic (ECC) and intrahepatic (ICC) cholangiocarcinoma.MethodsDNA extracted from tumour samples of 23 patients with CC, namely 10 patients with ECC and 13 patients with ICC, was analysed by array comparative genomic hybridisation. A support vector machine algorithm for classification was applied to the genomic data to distinguish between ICC and ECC. A survival analysis comparing both groups of patients was also performed.ResultsWith these whole genome results, we observed several common alterations between tumour samples of the same CC anatomical type, namely gain of Xp and loss of 3p, 11q11, 14q, 16q, Yp and Yq in ICC tumours, and gain of 16p25.3 and loss of 3q26.1, 6p25.3–22.3, 12p13.31, 17p, 18q and Yp in ECC tumours. Gain of 2q37.3 was observed in the samples of both tumour subtypes, ICC and ECC. The developed genomic model comprised four chromosomal regions that seem to enable the distinction between ICC and ECC, with an accuracy of 71.43% (95% CI 43% to 100%). Survival analysis revealed that in our cohort, patients with ECC survived on average 8 months less than patients with ICC.ConclusionsThis genomic characterisation and the introduction of genomic models to clinical practice could be important for patient management and for the development of targeted therapies. The power of this genomic model should be evaluated in other CC populations.

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