Extra-adrenal Non-functioning Paraganglioma – A Diagnostic and Surgical Challenge

Abstract Paragangliomas are rare neoplasms arising from the neuroendocrine cells. Extra-adrenal paragangliomas arise from the neuroendocrine cells spread in the retroperitoneum from the skull base to the retroperitoneum and sacrum. Non-functioning paragangliomas are a diagnostic challenge as they are clinically silent and attain large size. The clinical implication of these non-functioning paragangliomas is the greater metastatic potential of extra adrenal paraganglioma (20–42%) when compared to adrenal paraganglioma. We report a very rare case of a 60-year lady who presented with abdominal heaviness and retroperitoneal lump. Imaging was suggestive of a retroperitoneal tumor. Serum chromogranin A level was elevated with normal catecholamine levels. The tumor was resected along with metastasectomy of isolated liver metastasis. The patient has no recurrence or metastasis at one year of follow-up. To conclude paraganglioma should be considered as a differential diagnosis in all retroperitoneal tumors. Serum chromogranin A levels and plasma metanephrines should be routinely done for all retroperitoneal cases before planning for the biopsy of the lesion. Resection of the lesions should always be performed as per the oncological principles as pre-operative differentiation of benign and malignant lesions is difficult.

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Infantile Fibrosarcoma Histologically Mimicking a Benign Soft-Tissue Tumor

Journal of Research in Orthopedic Science ◽

10.32598/jrosj.7.4.726.1 ◽

2020 ◽

Vol 7 (4) ◽

pp. 179-184

Author(s):

Sam Hajialiloo Sami ◽

◽

Farshad Zandrahimi ◽

Mohamadreza Heidarikhoo ◽

Mahsa Zahmatkesh ◽

...

Keyword(s):

Soft Tissue ◽

Soft Tissue Tumor ◽

Vascular Lesion ◽

Diagnostic Challenge ◽

Soft Tissue Neoplasm ◽

Infantile Fibrosarcoma ◽

Inappropriate Treatment ◽

Benign Soft Tissue Tumor ◽

One Year

Infantile fibrosarcoma is a rare soft-tissue neoplasm, which may render a diagnostic challenge leading to misdiagnosis and consequently an inappropriate treatment of patients. This study reports a case of infantile fibrosarcoma that mimicked a hemangioma in an 11-month-old girl. As the lesion signal in the MRI was not consistent with the diagnosis of hemangiomas, we performed a core needle biopsy, which its result was consistent with the diagnosis of infantile fibrosarcoma. The lesion was initially treated with surgical resection. However, the lesion recurred one year after the surgery. The recurrence was managed with debulking surgery. The fifth finger was necrotized during the hospitalization after the relapse surgery. Finally, the necrotic finger was amputated. Also, adjuvant chemotherapy was used to prevent further relapses. The 1-year follow-up of the patient was recurrence-free. These findings highlight the importance of considering infantile fibrosarcoma when an infant presents with a lesion that clinically mimics a vascular lesion.

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Sinonasal Polyps: A Diagnostic Challenge

Bengal Journal of Otolaryngology and Head Neck Surgery ◽

10.47210/bjohns.2019.v27i3.16 ◽

2019 ◽

Vol 27 (3) ◽

pp. 243-250

Author(s):

Sucharita Sarkar ◽

Rama Saha ◽

Mou Das ◽

Samares Sardar

Keyword(s):

Treatment Protocol ◽

Case Series ◽

Olfactory Neuroblastoma ◽

Mass Lesion ◽

Diagnostic Challenge ◽

Basal Cell Adenocarcinoma ◽

Adenoid Cystic ◽

Malignant Lesions ◽

One Year ◽

Gross Examination

Introduction Sinonasal polyps, presenting as mass lesion of nose and paranasal sinuses ranges pathologic entity including infective diseases to malignant lesions. 80% are non-neoplastic lesions and less than 1% are malignant. They all present with symptoms of nasal stuffiness or obstruction and mass lesion, producing significant diagnostic challenges as they possess extremely varied clinical behaviour, etiopathogenesis, treatment protocol as well as prognosis. Case Series During period of one year (March 2017 to February 2018), we had six patients presenting with nasal polyp having special features that need attention. After proper investigation each case was operated and gross examination followed by histopathology was done. They revealed six different diagnoses e.g., Olfactory neuroblastoma, Adenoid Cystic Carcinoma, Basal Cell Adenocarcinoma, Sinonasal Mucosal Melanoma, Primitive Neuroectodermal Tumour (PNET) and Aspergilloma. Discussion Clinicians’ attention is drawn to the fact that, similar presentation may have varied differential diagnoses, some of which may be very rare and histopathology is essential for coming to definitive diagnosis.

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A case of eccrine porocarcinoma characterized by a progressive increase in the level of Ki-67 index: case report and review of literature

BMC Surgery ◽

10.1186/s12893-019-0595-4 ◽

2019 ◽

Vol 19 (1) ◽

Author(s):

Jie Shen ◽

Xinfa Pan ◽

Yongfu Lu ◽

Desheng Pan ◽

Yuehui Ma ◽

...

Keyword(s):

Metastatic Potential ◽

Progressive Increase ◽

Intracranial Metastasis ◽

Ki 67 ◽

Late Event ◽

Long Term Follow Up ◽

Eccrine Carcinoma ◽

One Year ◽

Eccrine Porocarcinoma

Abstract Background Eccrine porocarcinoma is an extremely rare skin adnexal malignant neoplasia with highly invasive and metastatic potential. We report an additional case of eccrine porocarcinoma with intracranial metastases. This case is characterized by a complete record of the progress of eccrine porocarcinoma, its immunohistochemistry after three operations showed a progressive increase in the level of Ki-67 index. Case presentation We herein report a case of a 37-year-old-male with eccrine carcinoma occurring on the left posterior occipital scalp which invaded the skull and dura, presenting with progressive headache. This patient has performed three surgeries in total. During the last hospitalization, he underwent an extended surgical resection, lymphadenectomy, myocutaneous flap transplantation and vascular anastomosis in our institution. After surgery, he was treating with radiotherapy at 200 Gray in 12 fractions. But one year after the operation, he developed chest tightness, imaging examination and biopsy puncture revealed pulmonary metastasis. Conclusion Intracranial metastasis of eccrine porocarcinoma is a late event with poor prognosis. This case emphases on that progressively increased level of Ki-67 index may predict more chance to occur the intracranial metastasis of scalp eccrine porocarcinoma, long-term follow-up and appropriately dense follow-up interval is necessary.

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Benign myoepithelioma of the hard palate: a clinical and histological diagnostic challenge. Case report and literature review

Journal of Oral Medicine and Oral Surgery ◽

10.1051/mbcb/2017040 ◽

2018 ◽

Vol 24 (2) ◽

pp. 81-88

Author(s):

Louis Maffi-Berthier ◽

François Le pelletier ◽

Anne-laure Ejeil

Keyword(s):

Clinical Data ◽

Salivary Gland Tumor ◽

Oral Pathology ◽

Metastatic Potential ◽

Low Grade ◽

Diagnostic Challenge ◽

Histological Data ◽

Proper Diagnosis ◽

Few Data

Introduction: Myoepithelioma (ME) is a rare salivary gland tumor. Constructed aroung a clinical case, this article aims to gather up up-to-date epidemiological, clinical and histological data about myoeptihelioma with emphasis on the diagnostic approach and differential diagnoses, paraclinical exams and the main histological features reported for its characterization. Observation: A 41-year-old female, presenting a 1-year slowly enlarging palatine nodule was referred to the Oral Pathology Consultation. Clinical data and paraclinic examination were non-specific. A thorough histological examination, comparing clinical data with cyto-architectural and immunostaining profile of the tumor allowed a positive diagnosis of ME. Discussion: The clinical aspect of ME is close from other more frequent tumors within the same areas. Accordingly, its discovery is often incidental and its diagnosis histological. ME display variable architecture and composition, requiring full tumor examination for proper diagnosis. When benign, ME act as mixed tumor regarding local extension, prognosis and recurrence. Malignant ME behaves as a low-grade malignant tumor with metastatic potential. Conclusion: Despite its rarity, ME should be hypothesized in front of a palatine nodule. Clinician and pathologist should be particularly cautious regarding nature, malignancy and follow-up of this tumor, since few data are up-to-now available.

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Fibrous Hamartoma of Infancy in the Scrotum

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0039-1697924 ◽

2019 ◽

Vol 07 (01) ◽

pp. e100-e103

Author(s):

Hrvoje Stepančec ◽

Zoran Kokot ◽

Draženko Keretić ◽

Sandra Radiković ◽

Donat Grgurović

Keyword(s):

Case Report ◽

Clinical Picture ◽

Benign Tumor ◽

Male Infant ◽

Histopathological Analysis ◽

Rare Tumor ◽

Diagnostic Challenge ◽

Fibrous Hamartoma Of Infancy ◽

One Year

AbstractFibrous hamartoma of infancy is a solid benign tumor of the subcutis, which usually occurs within the first 2 years of life. It predominantly occurs in males, and is clinically presented as a solid, painless, well-limited subcutaneous formation, tending to grow, and in most cases without any symptoms. It occurs in various locations. The aim of this case report was to present a case of a rare tumor of infancy in the scrotal region, in an 8-month-old male infant, with a nonspecific clinical picture, suggestive of a malignant formation, thus presenting a diagnostic challenge for a doctor. The tumor was completely removed. The diagnosis was confirmed by histopathological analysis. One year after the surgical procedure, a follow-up ultrasonography examination showed no relapse.

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Chromogranin A as circulating marker for diagnosis and management of neuroendocrine neoplasms: more flaws than fame

Endocrine Related Cancer ◽

10.1530/erc-17-0269 ◽

2018 ◽

Vol 25 (1) ◽

pp. R11-R29 ◽

Cited By ~ 38

Author(s):

Vincenzo Marotta ◽

Maria Chiara Zatelli ◽

Concetta Sciammarella ◽

Maria Rosaria Ambrosio ◽

Marta Bondanelli ◽

...

Keyword(s):

Clinical Application ◽

Treatment Strategy ◽

Chromogranin A ◽

Current Knowledge ◽

Morphological Evolution ◽

Neuroendocrine Cells ◽

Neuroendocrine Neoplasms ◽

Large Dense Core Vesicles ◽

Definition Of

Owing to the heterogeneity of neuroendocrine neoplasms (NENs), the availability of reliable circulating markers is critical for improving diagnostics, prognostic stratification, follow-up and definition of treatment strategy. This review is focused on chromogranin A (CgA), a hydrophilic glycoprotein present in large dense core vesicles of neuroendocrine cells. Despite being long identified as the most useful NEN-related circulating marker, clinical application of CgA is controversial. CgA assays still lack standardization, thus hampering not only clinical management but also the comparison between different analyses. In the diagnostic setting, clinical utility of CgA is limited as hampered by (a) the variety of oncological and non-oncological conditions affecting marker levels, which impairs specificity; (b) the fact that 30–50% of NENs show normal CgA, which impairs sensitivity. Regarding the prognostic phase, there is prospective evidence which demonstrates that advanced NENs secreting CgA have poorer outcome, as compared with those showing non-elevated marker levels. Although the identification of cut-offs allowing a proper risk stratification of CgA-secreting patients has not been performed, this represents the most important clinical application of the marker. By contrast, based on prospective studies, the trend of elevated circulating CgA does not represent a valid indicator of morphological evolution and has therefore no utility for the follow-up phase. Ultimately, current knowledge about the role of the marker for the definition of treatment strategy is poor and is limited by the small number of available studies, their prevalent retrospective nature and the absence of control groups of untreated subjects.

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Infantile haemangiopericytoma: a rare congenital cervical tumour

The Journal of Laryngology & Otology ◽

10.1017/s0022215113003630 ◽

2014 ◽

Vol 128 (2) ◽

pp. 212-215 ◽

Cited By ~ 4

Author(s):

E M Farrar ◽

J E H Bates ◽

S Bitetti ◽

I A Bruce

Keyword(s):

Histological Analysis ◽

Clinical Course ◽

Surgical Excision ◽

Neck Mass ◽

Diagnostic Challenge ◽

Adults And Children ◽

Posterior Neck ◽

Borderline Malignancy ◽

One Year

AbstractObjective:Infantile haemangiopericytoma is a rare childhood vascular tumour of borderline malignancy and unpredictable clinical course. It can present a diagnostic challenge due to indeterminate clinical, radiological and pathological features. This report presents the case of a large congenital haemangiopericytoma of the neck in a neonate, and discusses diagnosis, imaging, pathology and surgical management.Clinical presentation:A full-term neonate presented with a large posterior neck mass at birth. Pre-operative radiological appearances were suggestive of teratoma, but following surgical excision the diagnosis of infantile haemangiopericytoma was confirmed on histological analysis. There were no signs of recurrence at 12-month follow up.Conclusion:Haemangiopericytoma can follow an aggressive course in adults, including local recurrence and metastasis. The infantile variant is rare but typically follows a distinct clinical course, and is associated with more benign behaviour compared with similar tumours in adults and children over one year. Congenital haemangiopericytoma can be effectively treated with surgery, without requiring adjuvant therapy.

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The Role of Chromogranin A in Adrenal Tumors

Revista de Chimie ◽

10.37358/rc.18.3.6174 ◽

2018 ◽

Vol 69 (3) ◽

pp. 678-681

Author(s):

Alexandra Mirica ◽

Ioana Anca Badarau ◽

Ana Maria Stefanescu ◽

Radu Mirica ◽

Sorin Paun ◽

...

Keyword(s):

Chromogranin A ◽

Current Knowledge ◽

Diagnostic Value ◽

Neuroendocrine Cells ◽

Adrenal Tumors ◽

Adrenocortical Adenomas ◽

Diagnostic Usefulness ◽

Malignant Lesions ◽

Dense Core Granules

Chromogranin A (CgA) is part of the family of granins, which are acidic glycoproteins that represent an important part of secretory dense core granules. They are specific to various neuroendocrine and endocrine tissues, as components of diffuse neuroendocrine system and endocrine glands. CgA is co-secreted and co-released in the circulation along with hormones, bioamines and peptides secreted from the neuroendocrine cells . In the last decade, studies have emphasized the major importance of serum CgA in the diagnosis and follow-up of neuroendocrine tumors such as gastroenteropancreatic tumors, pheochromocytoma, medullary thyroid carcinoma. But its diagnostic value for adrenocortical adenomas or for adrenal malignancy, is still controversial. The current study aims to provide a comprehensive review , for synthesizing current knowledge regarding corelations between plasma CgA concentration and various adrenal tumors. Furthermore, there will be also analyzed and synthesized the clinical applicability and the diagnostic usefulness of dosing CgA in adrenal pathology, both medullary and cortical benign and malignant lesions.

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