Chordoma in Early Childhood: A Clinicopathological Study

Abstract A case of clival chordoma in a 4-year-old girl is presented. The tumor regrew rapidly after it was partially removed, and the patient died after a clinical course of 11 months. An autopsy revealed a massive clival mass and widespread metastases in the dura mater, skull bone, bilateral lungs, liver, sternum, left humerus, and vertebrae. Pathological findings showed that the tumor cells were poorly differentiated, with a rare, but typical, physaliphorous appearance. The presence of epithelial differentiation proteins, mitochondria surrounded by rough endoplasmic reticulum, and desmosomes was demonstrated in the tumor cells both immunohistochemically and ultrastructurally. Thus, the tumor was diagnosed as a chordoma. The literature pertaining to intracranial chordoma in early childhood is reviewed. Rapid growth and distant metastases may occur in chordomas at a young age.

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Ulcerogenic Tumors of the Pancreas

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100100676 ◽

1968 ◽

Vol 26 ◽

pp. 186-187

Author(s):

J. C. Garancis ◽

J. F. Kuzma ◽

S. D. Wilson ◽

E. H. Ellison

Keyword(s):

Endoplasmic Reticulum ◽

Tumor Cells ◽

Islet Cell ◽

Disease Process ◽

Central Core ◽

Islet Cell Tumors ◽

Opaque Zone ◽

Granular Form ◽

Zollinger Ellison Syndrome

It has been proposed that a gastrin-like hormone elaborated by non-beta islet tumors of the pancreas may be responsible for a fulminating ulcer diathesis. Subsequently, a potent gastric secretagogue was isolated from ulcerogenic tumors of the pancreas. This disease process is known now as “Zollinger-Ellison syndrome”.In our studies of two cases of Zollinger-Ellison syndrome, pancreatic lesions were identified as alpha islet cell tumors (Fig. 1). Tumor cells were fairly uniform. The sizes of the alpha granules were not significantly different, but their number and distribution varied greatly from one cell to another. Each granule consisted of a round, highly dense central core, separated from the limiting membrane by an opaque zone. The granular form of the endoplasmic reticulum was particularly prominent. Numerous mitochondria, round or elongated, were dispersed throughout the cytoplasm. Individual or clusters of lysosomes were observed in the majority of cells.

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Temporal and spatial interrelationships of confronting cisternae to nuclear envelope

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100125051 ◽

1992 ◽

Vol 50 (1) ◽

pp. 930-931

Author(s):

John R. Palisano

Keyword(s):

Endoplasmic Reticulum ◽

Nuclear Envelope ◽

Tumor Cells ◽

Hela Cells ◽

Microscopic Investigation ◽

Electron Microscopic Investigation ◽

Serial Sections ◽

Electron Microscopic ◽

Fetal Rat ◽

Temporal And Spatial

Although confronting cistemae (CC) have been observed in a variety of tumor cells and normal fetal rat, mouse, and human epithelial tissues, little is known about their origin or role in mitotic cells. While several investigators have suggested that CC arise from nuclear envelope (NE) folding back on itself during prophase, others have suggested that CC arise when fragments of NE pair with endoplasmic reticulum. An electron microscopic investigation of 0.25 um thick serial sections was undertaken to examine the origin of CC in HeLa cells.

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Palpebral Amelanotic Melanomas in F344 Rats

Veterinary Pathology ◽

10.1177/030098589303000309 ◽

1993 ◽

Vol 30 (3) ◽

pp. 280-286 ◽

Cited By ~ 10

Author(s):

K. Yoshitomi ◽

G. A. Boorman

Keyword(s):

Tumor Cells ◽

Electron Microscopic Examination ◽

Ultrastructural Feature ◽

Amelanotic Melanoma ◽

Electron Microscopic ◽

Cell Processes ◽

Poorly Differentiated ◽

The Right ◽

F344 Rats ◽

Left Eyelid

Spontaneous amelanotic melanomas in the eyelids of F344 rats were found in one of 1/926 (0.11 %) male and 5/925 (0.54%) female F344 rats that were used as control and treated animals in five different carcinogenicity studies conducted by the National Toxicology Program (Research Triangle Park, NC). These melanomas were grossly recognized as single, tan or white, well-circumscribed masses of the right or left eyelid. These melanomas primarily occurred in the dermis of the skin of the eyelids and consisted of poorly differentiated spindle cells characteristically arranged in interlacing fascicles. Rarely, epithelioid tumor cells were also observed, and these tumor cells showed a negative histochemical reaction for melanin. The epidermis and dermal–epidermal junction were usually uninvolved. The diagnosis of amelanotic melanoma could only be established by electron microscopic examination. The most striking ultrastructural feature of the tumor cells was a large number of intracytoplasmic premelanosomes (stage II melanosomes without melanin), which nearly filled the cytoplasm of most tumor cells. Giant premelanosomes and melanophagosomes were also seen. The tumor cells did not possess the ultrastructural features characteristic of Schwann cells (thin, long cell processes and pericytoplasmic basal laminae). The histologic and ultrastructural features of these palpebral tumors were similar to those of cutaneous amelanotic melanomas of the pinna in F344 rats.

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You can’t judge a book by its cover or a tumor by its expression profile

Clinics and Practice ◽

10.4081/cp.2012.e21 ◽

2012 ◽

Vol 2 (1) ◽

pp. 21

Author(s):

Steven M. Sorscher ◽

Theodore Thomas

Keyword(s):

Expression Profile ◽

Expression Profiling ◽

Radiographic Progression ◽

Clinical Course ◽

Unknown Primary ◽

Great Promise ◽

Primary Tumors ◽

Remarkable Case ◽

Rate Of Progression ◽

Poorly Differentiated

Expression profiling has shown great promise in matching cancers of unknown primary to likely primary tumors of origin based on patterns of mRNA expression. However, it remains uncertain as to whether even well matched tumors will demonstrate the clinical features, such as rate of progression, of their matched counterparts. In this case report, we note that based on histology, immunohistochemistry and expression profile this patient’s poorly differentiated neuroendocrine tumor would have been expected to grow very rapidly on no therapy. Instead, this cancer was very indolent, with only very little radiographic progression over several years. We believe this report represents a remarkable case of a tumor where features, including expression profile, would not at all have accurately predicted the clinical course seen. While some series have suggested that matching by expression profiling predicts outcome, this case shows a dramatically different result.

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MO961KIDNEY TRANSPLANTATION AS A TREATMENT OF CHOICE FOR AA AMYLOIDOSIS DUE TO PERIODIC FEVER SYNDROME

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab110.0040 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Ivana Dedinska ◽

Karol Graňák ◽

Matej Vnučák

Keyword(s):

Early Childhood ◽

Kidney Transplantation ◽

Clinical Course ◽

Periodic Fever ◽

Periodic Fever Syndrome ◽

Aa Amyloidosis ◽

Periodic Fever Syndromes ◽

Post Transplant ◽

Fever Syndromes ◽

End Stage

Abstract Background and Aims Renal AA amyloidosis is the most serious complication of periodic fever syndrome, which, inadequate suppression, due to persistent inflammation, leads to nephrotic syndrome and renal failure over several years. In most cases, periodic fever syndromes begin to manifest clinically in early childhood. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment for end-stage chronic kidney disease (CKD) based on AA amyloidosis. Method We present cases of two patients after deceased donor kidney transplantation, who have been diagnosed with adult periodic fever syndrome. Conclusion Periodic fever syndromes are diseases beginning to manifest clinically in early childhood in 60–90% of cases, most often in the case of familial Mediterranean fever around 4 years of age, in the case of CAPS usually only a few months after birth. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment of end-stage CKD based on AA amyloidosis associated with periodic fever syndrome. Adequate targeted treatment against IL-1 or TNF is important and appears to be safe during the post-transplant period, with regular monitoring of renal function, acute phase inflammatory reactants, and histological findings by protocol graft biopsies. It will be important and necessary to assess the development of diseases in the post-transplant period in the long term.

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Clinicopathological Study of Poorly Differentiated Colorectal Adenocarcinoma.

Nippon Daicho Komonbyo Gakkai Zasshi ◽

10.3862/jcoloproctology.45.244 ◽

1992 ◽

Vol 45 (2) ◽

pp. 244-247 ◽

Cited By ~ 2

Author(s):

M. Kanno ◽

H. Sakamoto ◽

Y. Tsukioka ◽

Y. Takano ◽

Y. Kurosaka ◽

...

Keyword(s):

Colorectal Adenocarcinoma ◽

Clinicopathological Study ◽

Poorly Differentiated

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Clinicopathological Study for Poorly Differentiated Colorectal Adenocarcinoma

Nihon Gekakei Rengo Gakkaishi (Journal of Japanese College of Surgeons) ◽

10.4030/jjcs1979.29.4_694 ◽

2004 ◽

Vol 29 (4) ◽

pp. 694-698

Author(s):

Yuzo UMEDA ◽

Kouji TANAKAYA ◽

Hitoshi TAKEUCHI

Keyword(s):

Colorectal Adenocarcinoma ◽

Clinicopathological Study ◽

Poorly Differentiated

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Well differentiated endocrine carcinomas of the pancreas

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh1110625c ◽

2011 ◽

Vol 139 (9-10) ◽

pp. 625-630

Author(s):

Radoje Colovic ◽

Nikica Grubor ◽

Marjan Micev ◽

Vladimir Dugalic ◽

Dejan Stojakov ◽

...

Keyword(s):

Distal Pancreatectomy ◽

Pancreatic Head ◽

Distant Metastases ◽

Massive Bleeding ◽

R0 Resection ◽

Gastric Bleeding ◽

Poorly Differentiated ◽

The Difference ◽

Well Differentiated ◽

Endocrine Carcinomas

Introduction. For the difference from poorly differentiated, well differentiated endocrine carcinomas of the pancreas are the tumours in whom with aggressive surgery and chemotherapy fair results can be achieved. Objective. The aim of the study was to point out the importance of such treatment. Methods. Over a 6-year period eight patients (seven female and one male) of average age 51 years (ranging from 23 to 71 years) were operated on for well differentiated endocrine carcinoma: six of the head and two of the tail of the pancreas. There were two functional and six nonfunctional tumours. Pain in the upper part of the abdomen in seven, mild loss in weight in two, strong heartburn in two, obstructive jaundice in three, diarrhoea in one, sudden massive bleeding from gastric varicosities due to prehepatic portal hypertension caused by pancreatic head tumour in one, and bruise in one patient were registered preoperatively. US and CT in all, angiography in one, octreoscan in two and PET scan in one patient were performed. Whipple?s procedure was performed in six and distal pancreatectomy in two patients, as well as systemic lymphadenectomy in all and excision of liver secondary tumours in two patients. In the patient with massive gastric bleeding a total gastrectomy was performed first, followed by Whipple?s procedure a month later. Results. R0 resection was achieved in all patients. Lymph nodes metastases were found in six patients. Six patients were given chemotherapy. One patient died 3 years after surgery, seven are still alive, on average 2.5 years. A local recurrence after distal pancreatectomy that occurred 5 years after surgery was successfully reresected and the patient is on peptide-receptor radiotherapy. In other six patients there were no local recurence or distant metastases. Conclusion. With aggressive surgery and chemotherapy fair results can be achieved in well differentiated endocrine carcinomas of the pancreas.

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