Rare cancers:
Gespeichert in:
| Format: | Buch |
|---|---|
| Sprache: | English |
| Veröffentlicht: |
Philadelphia, PA
Elsevier
2012
|
| Schriftenreihe: | Hematology, oncology clinics of North America
26,6 |
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | Includes bibliographical references and index |
| Beschreibung: | XII S., S. 1137 - 1384 Ill., graph. Darst. 24 cm |
| ISBN: | 9781455749423 1455749427 |
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| 245 | 1 | 0 | |a Rare cancers |c ed. Guy Eslick |
| 264 | 1 | |a Philadelphia, PA |b Elsevier |c 2012 | |
| 300 | |a XII S., S. 1137 - 1384 |b Ill., graph. Darst. |c 24 cm | ||
| 336 | |b txt |2 rdacontent | ||
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| 490 | 1 | |a Hematology, oncology clinics of North America |v 26,6 | |
| 490 | 0 | |a Clinics review articles | |
| 500 | |a Includes bibliographical references and index | ||
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Datensatz im Suchindex
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| adam_text | Rare Cancers
Contents
Preface
xi
Guy D. Eslick
What is a Rare Cancer?
1137
Guy D. Eslick
Many rare cancers are essentially an enigma, with little to no information in
the medical literature. Defining a rare cancer is not as easy as it might
seem. As a guide, generally an incidence of fewer than
6/100,000
is con¬
sidered rare. Much of the research regarding rare diseases is in its infancy.
New Insights into the Pathogenesis, Diagnosis, and Management of Mastocytosis
1143
Stephen J. Fuller
This review describes developments in understanding normal mast cell
function and genetic changes that predispose to malignant transformation
to mastocytosis. Most mastocytosis cases are associated with somatically
acquired activating mutations in the KIT receptor. The role these mutations
play in the development of mastocytosis is discussed. Mastocytosis is
classified into cutaneous mastocytosis and systemic mastocytosis. The
classification of mastocytosis and clinical presentation of each variant is
detailed in this report. Progress has been made in developing drugs that
target the wild-type and mutated KIT receptor, and these and other new
therapeutic strategies for the treatment of mastocytosis are reviewed.
Epidemiology and Management of Uveal Melanoma
1169
Yoshihiro Yonekawa and
Ivana
К.
Kim
Uveal melanoma is the most common primary intraocular malignancy in
adults. The annual incidence is approximately
5
per million in the United
States. Risk factors include white race, fair skin, light iris color, ancestry
from northern latitudes, and ocular/oculodermal melanocytosis. Historically,
enucleation was the definitive treatment for uveal melanoma, but brachy-
therapy and proton beam irradiation are now the most commonly employed
treatments. However, there are no effective therapies against metastatic
uveal melanoma. Recent studies suggest that the pathogenesis of uveal
melanoma includes activation of the
МАРК
pathway via GNAQ/1
1,
and
that metastatic potential may be modulated by mechanisms involving BAP1
.
Esthesioneuroblastoma: A Contemporary Review of Diagnosis and Management
1185
Matthew
Bak
and Richard O.
Wein
Esthesioneuroblastoma (ENB) is a rare malignancy, representing only
3%
to
6%
of all sinonasal malignancies. A wide array of treatment options for
ENB have been described in the literature, but prospective clinical trials
are absent given the tumor s rarity and natural history. Delay in diagnosis
leading to an initial advanced stage of presentation is common secondary
viii Contents
to the clinically hidden primary site at the anterior skull base. This article
presents data from the current body of literature and reviews the advo¬
cated roles for surgery, radiation therapy and chemotherapy.
Synovial
Cell Sarcoma of the Larynx
1209
Shruti Jayachandra, Ronald Y. Chin, and Peter Walshe
Synovial
cell sarcoma represents a rare group of cancers, particularly in
the head and neck region, that typically affects young individuals and
has a male preponderance. Prognosis varies with patient age, site and
size of the malignancy, degree of necrosis, high level of mitotic activity,
and neurovascular invasion. Complete surgical resection of the tumor
via partial or total laryngectomy is the first-line treatment in locally invasive
disease. CO2 lasers have been shown to be useful in controlling localized
disease. There is also a role for adjuvant radiotherapy. Ifosfamide-based
chemotherapy is most useful for malignant disease.
Parathyroid Carcinoma: Challenges in Diagnosis and Treatment
1221
Arash Mohebati, Ashok Shaha, and Jafin Shah
Parathyroid carcinoma is a malignant neoplasm affecting
0.5%
to
5.0%
of
all patients with primary hyperparathyroidism. Since it was first described
by
De Quervain
in
1904
to this day, it continues to defy diagnosis and treat¬
ment because of its rarity, overlapping features with benign parathyroid
disease, and lack of distinct characteristics. En bloc surgical extirpation
of the tumor with clear margins remains the best curative treatment. Al¬
though prolonged survival is possible with recurrent or metastatic disease,
cure is rarely achievable. Efficacy of adjuvant therapies, such as radiother¬
apy and chemotherapy, in management of persistent, recurrent, or meta¬
static disease has been disappointing.
Gastrointestinal Stromal Tumor
—
Background, Pathology, Treatment
1239
Burton L.
Eisenberg
and J. Marc
Pipas
Gastrointestinal stromal tumor (GIST) represents the most common mese-
chymal tumor of the gastrointestinal tract. Discovery of the relationship
between unregulated KIT kinase and GIST transformation has led to diag¬
nostic and. therapeutic targeting. Imatinib is the recommended first-line
treatment of metastatic GIST. In addition, the combination of surgery and
imatinib for primary GIST is indicated in the adjuvant setting of high-risk
patients and there may be benefit for this combination in the neoadjuvant
setting. The success of molecular targeted therapy in GIST represents an
important and exciting advance in solid tumor oncology.
Neoplasms of the Appendix: Current Treatment Guidelines
1261
Suven Shankar, Panayotis Ledakis, Hatem El Halabi,
Vadim Gushchin,
and
Armando
Sardi
The purpose of this article is to update the medical community on the cur¬
rent management of patients with appendiceal neoplasms. The authors
discuss clinical evidence of medical and surgical treatment with emphasis
on presentation, diagnosis, pathology, and surgical technique. Current
Contents
available clinical evidence on the use of systemic chemotherapy is in¬
cluded. The authors describe in detail management of peritoneal carcino-
matosis arising from tumors of the appendix with cytoreductive surgery
and hyperthermic intraperitoneal therapy as standard of care.
Urethral Cancer
1291
Petros D. Grivas, Matthew Davenport, James E.
Montié,
L
Priya
Kunju,
Felix Feng, and
Alón
Z.
Weizer
Urethral carcinoma is a rare tumor with predominantly poor survival. Both
the disease and its treatment can affect both sexual and urinary function.
The natural history of urethral carcinoma varies, therefore the appropriate
application of surgery, radiation, and chemotherapy remain unknown.
Management of this disease remains driven by individual clinician experi¬
ence and data derived from small case series. This article discusses the
histology and anatomy of the male and female urethra, as well as their nat¬
ural history. In addition, the epidemiology, clinical presentation, diagnosis,
staging, treatment, and future directions of management of cancer arising
in the urethra are addressed.
Current Treatment of Anal Squamous Cell Carcinoma
1315
Rob Glynne-Jones and Andrew Renehan
The primary aim of anal cancer treatment is loco-regional control with
preservation of anal function. Phase III trials consistently demonstrate ra¬
diotherapy with concurrent 5FU and mitomycin
(MMC) chemoradiation
is
the standard of care for anal cancer. Salvage surgery is associated with
considerable morbidity and requires specialised input. With current so¬
phisticated radiological staging and the ability to spare critical normal tis¬
sues with intensity-modulated radiotherapy, a one-size-fits-all approach
is probably inappropriate. Radiotherapy dose-escalation and intensifica¬
tion of the concurrent chemotherapy might improve local control, but
may also adversely affect colostomy-free survival. Integration of biologic
therapy with conventional chemotherapies looks hopeful in the future.
Merkel
Cell Carcinoma
1351
Sandra Y. Han, Jeffrey P. North, Theresa Canavan, Nancy Kim, and Siegrid S. Yu
Merkel
cell carcinoma
(MCC)
is a rare but aggressive carcinoma of the skin,
arising most commonly in sun-exposed sites of elderly patients. The diag¬
nosis is based on characteristic histopathologic features. In
2008,
the dis¬
covery of the
Merkel
cell polyomavirus led to intensified research into the
viral pathogenesisis of
MCC. MCC
staging guidelines were established in
2010,
and it demonstrated the importance of distinguishing clinical vs. path¬
ologic evaluation of lymph nodes in
MCC.
Surgery and/or radiation is of the
mainstay of therapy for early disease, while chemotherapy is reserved for
more advanced disease. Treatments based on
immunologie
mechanisms
are currently in development.
Index
1375
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| isbn | 9781455749423 1455749427 |
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| spelling | Rare cancers ed. Guy Eslick Philadelphia, PA Elsevier 2012 XII S., S. 1137 - 1384 Ill., graph. Darst. 24 cm txt rdacontent n rdamedia nc rdacarrier Hematology, oncology clinics of North America 26,6 Clinics review articles Includes bibliographical references and index Cancer Onkologie (DE-588)4075658-0 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Onkologie (DE-588)4075658-0 s DE-604 Eslick, Guy D. Sonstige oth Hematology, oncology clinics of North America 26,6 (DE-604)BV000625446 26,6 Digitalisierung UB Regensburg application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=025495367&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
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| title | Rare cancers |
| title_auth | Rare cancers |
| title_exact_search | Rare cancers |
| title_full | Rare cancers ed. Guy Eslick |
| title_fullStr | Rare cancers ed. Guy Eslick |
| title_full_unstemmed | Rare cancers ed. Guy Eslick |
| title_short | Rare cancers |
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| topic | Cancer Onkologie (DE-588)4075658-0 gnd |
| topic_facet | Cancer Onkologie Aufsatzsammlung |
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