Embryonal tumour with multilayered rosettes: Two case reports of a rare tumour

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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Rare case of angiofibroma presenting as perineal hernia

BMJ Case Reports ◽

10.1136/bcr-2020-236661 ◽

2021 ◽

Vol 14 (1) ◽

pp. e236661

Author(s):

Ruchika Kumari ◽

Cherring Tandup ◽

Ambuj Agarwal ◽

Anish Chowdhury

Keyword(s):

Soft Tissue ◽

Case Reports ◽

En Bloc Resection ◽

Perineal Hernia ◽

Pathological Examination ◽

Soft Tissue Tumour ◽

Open Approach ◽

En Bloc ◽

Rare Tumour ◽

Tissue Tumour

Angiofibroma is a benign soft tissue tumour presenting as a gradually progressive swelling in the vulvovaginal area in women and in the inguinoscrotal region in men. Being a rare tumour, there are only a few case reports in the literature, and among them, presentation as perineal herniation is very rare. En bloc resection of angiofibroma either via laparoscopic or open approach is the choice of treatment to avoid recurrence. Detailed pathological examination and immunohistochemistry workup are imperative to distinguish it from various mesenchymal tumours. Perineal hernia is itself rare and may occur spontaneously or following abdominoperineal resection, sacrectomy or pelvic exenteration. Surgical repair via open transabdominal and transperineal approaches has been described. Here, we report a case of a young woman who presented with spontaneous reducible perineal hernia with a soft tissue tumour as its content, which on histopathological investigation was found to be an angiofibroma.

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Circumstances of discovery of phaeochromocytoma: a retrospective study of 41 consecutive patients

Acta Endocrinologica ◽

10.1530/eje.0.1500681 ◽

2004 ◽

pp. 681-686 ◽

Cited By ~ 111

Author(s):

JP Baguet ◽

L Hammer ◽

TL Mazzuco ◽

O Chabre ◽

JM Mallion ◽

...

Keyword(s):

Blood Pressure ◽

Imaging Techniques ◽

Case Reports ◽

Genetic Disorder ◽

Surgical Excision ◽

University Hospital ◽

Imaging Method ◽

Rare Tumour ◽

Incidental Discovery ◽

Design And Methods

OBJECTIVE:Phaeochromocytoma is a rare tumour of the chromaffin cells, the diagnosis of which is based on an assay of metanephrines and treatment is surgical excision of the tumour. It is usually discovered due to a rich and varied symptomatology or classic paroxysmal hypertension. The main purpose of this study was to specify the exact circumstances of discovery of the phaeochromocytomas operated on in our university hospital between 1990 and 2002. DESIGN AND METHODS: Forty-one consecutive and complete case reports of patients who had surgery for phaeochromocytoma were analysed retrospectively. This series includes 10 patients with a genetic disorder predisposing to phaeochromocytoma. RESULTS: The association of headaches and palpitations with sweating was found in only 24% of cases (10/41). Blood pressure anomalies led to the discovery of phaeochromocytoma in only 51% of cases (21/41) and 59% (24/41) of all the patients suffered from hypertension. In almost half the cases (20/41), the tumour was discovered by an imaging method (ultrasonography, CT scan or MRI) which had been performed for reasons unrelated to a blood pressure abnormality. CONCLUSIONS: Phaeochromocytoma, the symptoms of which are not very specific and during which hypertension is present in only half the patients, is a disease that remains rare. Its incidence could be increasing because of changes in the method of detection. Indeed, in our study, different imaging techniques led to its incidental discovery in half of the cases.

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Bilateral leiomyosarcoma of kidneys with pericardial metastases: a double whammy

BMJ Case Reports ◽

10.1136/bcr-2018-228394 ◽

2019 ◽

Vol 12 (3) ◽

pp. e228394

Author(s):

Santosh Kumar ◽

Tushar Aditya Narain ◽

Gopal Sharma ◽

Shantanu Tyagi

Keyword(s):

Rare Case ◽

Case Reports ◽

Case Series ◽

Disease Status ◽

Rare Tumour ◽

Pericardial Metastasis

Renal sarcoma is a rare but lethal malignancy affecting the kidneys. Literature regarding this rare tumour has been limited to case reports and case series. In this paper, we report a very rare case of bilateral renal leiomyosarcoma with pericardial metastasis. Disease status and dearth of our knowledge in treating this particular type of case prompted us to review our experience of managing such case, which has never been reported previously in literature to our knowledge.

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Rapidly recurring ovarian fibrosarcoma after optimal debulking surgery in a 46-year-old woman

BMJ Case Reports ◽

10.1136/bcr-2020-234757 ◽

2020 ◽

Vol 13 (7) ◽

pp. e234757

Author(s):

Hariyono Winarto ◽

Tantri Hellyanti ◽

Sigit Purbadi ◽

Sutrisno Sutrisno

Keyword(s):

Cell Morphology ◽

Spindle Cell ◽

Frozen Section ◽

Case Reports ◽

Tumour Recurrence ◽

Nulliparous Woman ◽

Debulking Surgery ◽

Rare Tumour ◽

Immunohistochemistry Staining ◽

Optimal Debulking

Ovarian fibrosarcoma is an extremely rare tumour with no universally accepted guidelines for treatment. We present a 46-year-old nulliparous woman with ovarian fibrosarcoma who mainly presented with a painful abdominal enlargement. Optimal debulking surgery was performed, and a specimen of the tumour was examined. A frozen section examined during surgery revealed spindle cell morphology, raising the suspicion of ovarian fibrosarcoma, which was later confirmed by immunohistochemistry staining. Our patient refused to undergo chemotherapy and died 3 months after surgery due to tumour recurrence. Although no clear consensus exists for administering chemotherapy for fibrosarcoma, some published case reports have shown a lower chance of recurrence and better prognosis in patients who undergo chemotherapy compared with our patient.

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A rare case of endometriosis to clear cell ovarian carcinoma: a case report

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20194900 ◽

2019 ◽

Vol 8 (11) ◽

pp. 4585

Author(s):

Anaswara T. ◽

Prasanna Venugopalan ◽

Vidhu V. Nair ◽

Alfy Ann George ◽

I. Praseeda

Keyword(s):

Clear Cell ◽

Clear Cell Carcinoma ◽

Case Reports ◽

Treatment Options ◽

Rare Tumour ◽

Clear Cell Ovarian Carcinoma ◽

Advanced Stages ◽

Low Incidence ◽

Worse Prognosis ◽

In Pregnancy

Clear cell carcinoma of ovary is a rare tumour with a very low incidence in pregnancy. It is attributed to develop from an existing background of endometriosis. There are very few case reports of the above combination tumours in pregnancy. It is a very aggressive tumour with a worse prognosis and low survival rate because of its peculiar chemo resistant nature. Early detection and effective treatment are the best approach. The treatment options for advanced stages are still under research.

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A Rare Tumour in Childhood Desmoplastic Infantile Astrocytoma 2 Case Reports in the Context of the Literature

SiSli Etfal Hastanesi Tip Bulteni / The Medical Bulletin of Sisli Hospital ◽

10.14744/semb.2018.29292 ◽

2018 ◽

Author(s):

Canan Tanık

Keyword(s):

Case Reports ◽

Rare Tumour ◽

Desmoplastic Infantile Astrocytoma

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Primary Leiomyoma of the Liver: A Review of a Rare Tumour

HPB Surgery ◽

10.1155/2014/959202 ◽

2014 ◽

Vol 2014 ◽

pp. 1-8 ◽

Cited By ~ 9

Author(s):

Ayodeji Oluwarotimi Omiyale

Keyword(s):

Clinical Course ◽

Incidental Finding ◽

Case Reports ◽

Extensive Literature ◽

Liver Tumours ◽

Rare Tumour ◽

Extensive Literature Search ◽

The Mean ◽

Average Size

Context. Primary leiomyoma of the liver is a rare tumour with uncertain pathogenesis with similar presentation with other tumours of the liver. Little is known about its clinical course. Objectives. To review the literature for case reports of primary leiomyoma of the liver. Methods. Extensive literature search was carried out for case reports of primary leiomyoma of the liver. Results. A total of 36 cases of primary leiomyoma of the liver were reviewed. The mean age of presentation is 43 years with slight female sex affectation; females accounted for 55.6% of the cases reported in the literature. The average size of the tumour is 8.7 cm. 34.4% of the cases reviewed were incidental finding with the mean follow-up time of 33 months with most cases reporting no evidence of disease. Conclusions. Primary leiomyoma of the liver is very rare tumour with complex pathogenesis which remains largely unknown. Imaging of the tumour does not allow for a tissue specific diagnosis; hence histological review of the tissue specimen and immunohistochemical stains are imperative for diagnosis. Surgical resection is both diagnostic and curative. The diagnosis of primary leiomyoma of the liver should be considered as a differential in the management of liver tumours.

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