malignant primary tumors of chest wall

in adults the most common primary malignant Legends are chondrosarcoma, plasmacytoma and fibrosarcoma.

• Chondrosarcoma frequently appears as a
  large lobulated
  excrescent mass arising from a rib, with scattered calcification. As with other cartilaginous tumors, chondrosarcomas com-
  monly develop from the costo-chondral junction, and radiographically they may be indistinguishable from an
  osteochondroma or chondroma.
• Plasmacytoma is less fre-
  quent than chondrosarcoma, but the systemic disease, multi-
  ple myeloma, is frequently seen to involve several ribs as well
  as the sternum. The lesions of plasmacytoma or multiple
  myeloma typically appear as well-defined lytic lesions associ-
  ated with extrapleural soft masses, similar to most metastatic
  lesions. In advanced plasmacytoma, marked erosion, expansion, and destruction of the bony cortex is often present,
  sometimes with a thick ridging around the periphery, causing
  the „soap bubble“ appearance.
• Fibrosarcoma is the most
  common malignant tumor of the chest wall arising from the
  soft tissue in adults. However, as reported in most earlier
  series, the term fibrosarcoma probably includes many cases
  that now would be classified as malignant fibrous histiocy-
  toma, as well as spindle cell tumors such as malignant schwannoma or synovial sarcoma. Fibrosarcoma often presents as a mass of soft tissue density associated with necrotic low density
  areas; foci of calcification may be present. Approximately 15%
  of malignant schwannomas develop in the trunk, and about
  one-third are on the anterior chest wall. Like their benign
  counterpart, they appear as rounded or elliptical masses adjacent to the rib.

** Any radiographic evidence of bony destruction
is indicative of a malignant process. **

Other less common pri-
mary chest wall tumors seen in the adult population are
osteosarcoma, liposarcoma, and angiosarcoma.

In children and adolescents, the most common primary
tumor of the chest wall is Ewing’s sarcoma that generally
presents as a lytic and sometimes expansive lesion of a rib or clavicle with associated new bone formation and a soft tissue mass. Frequently, pleural effusion, fever and general symptoms are present. Ewing’s sarcoma is also seen as the common
metastatic tumor of the bony thorax.

Other less common primary malignant tumors in the pedi-
atric age group are osteosarcoma, rhabdomyosarcoma, and
mesenchymoma.

METASTATIC DISEASE

Metastatic lesions are the most common tumors of the chest wall and are seen more frequently than either primary malig-
nant or benign tumors. In the adult population, the most com-
mon metastatic diseases are lung, breast, kidney, and prostate
carcinomas. With the exceptions of prostate and breast can-
cer, the large majority of metastatic tumors to the chest wall
are lytic. In children, neuroblastoma, leukemia, and Ewing’s
sarcoma are the most common metastatic lesions.

CUP

[^1]:![[Does Cancer of Unknown Primary (CUP) Truly Exist as a Distinct Cancer Entity.pdf]]

cup, #onkologie

CUP kann auch als eine ==distinkte Tumorentität gesehen werden==.

[^1]

[^2]: ![[Site-specific survival rates for cancer of unknown primary according to location of metastases.pdf]]

Fall Frau Sener

![[Pasted image 20210322170846.png]]
![[Pasted image 20210322170932.png]]

Beim Tumorstaging nur isolierter PET-pos Herd am OL-Bronchus-Abgang identifiziert. Biopsie ergab großzelliges Karzinom. Mit der Diagnose „großzelligem Lungenkarzinom“ wurde die Patientin operiert, zwar OL-Manschettenlobektomie rechts. die postop. patholog. Untersuchung ergab allerdings LK-Metastase eins großzelligen Karzinoms. Im Restlungenlappen war kein Tumor zu sehen. Auch in der PET ist kein Primarius zu identifizieren.
Man kann diesen Fall als CUP mit mutmaßlichen Primarius in der Lunge definieren. Oder man muss nicht unbedingt so genau definieren. Stattdessen nur den Befund beschreiben „LK-Metatase eines großzelligen neuroendokrinen Karzinoms im Oberlappen rechts“

Mutmaßen den Primarius

Die Lokalisation der CUP-Metatsasen kann Hinweis auf nicht detektierbaren Primarius geben! [^2]
CUP-Metatasen in Atemweg, Nervensystem, Knochen und Haut: sehr wahrscheinlich Lungenkarzinom
CUP-Metatasen in peritorealem und retroperitonealem, pelvical Raum: sehr wahrscheinlich Ovarial-Ca, oder Pankreas- oder Kolorektaler Karzinom.

Definition CUP

CUP kann diagnostisiert werden, wenn der mutmaßliche Primarius nicht detektierbar ist trotz immmunhistochemie, gene expression profiling und bildgebung, selbst Immunhistochemie, Gene expression profiling Hinweise auf Primarius geben können.

Nevertheless, the diagnosis of CUP remains valid as long as no primary tumor in the respective organ is detectable.
[^2]

Autopsie ergab,dass Lunge, Leber, Pankreas und GI-Trakt häufig der Primarius von CUP sind. [^1] page1
Histologisch sind CUP meist Adenokarzinom (70%) und undifferenziertes Karzinom (20%), neuroendokrine Tumoren.

Eigenschaft und Prognose

sehr schlecht. weniger als 25% überleben 1 J.
aggresive clinical course, atypical metastasis pattern, rapid progression of metastases, generally poor response to chemotherapy [^1]

Thorough Diagnosis, Suche nach primarius

zuätzlich zu üblichen Staging noch Gastro-Kolo, Dermatologische Untersuchung (melanom), Broncho, Gynäkolog. Untersuchung einschließlich Mamographie.

ESMO hat nur die basis Untersuchung empfohlen.

Auf jeden Fall muss man ein Rezidiv eines früheren Tumors (antecedent cancer) ausschließen! [^1]

putative primaries (mutmaßliche)

warum findet man Primarius nicht?

Theorie: entweder Primarius verwindet oder sehr klein, noch nicht detektierbar, kann wahrscheinlich im Verlauf gefunden werden. [^1] page 2

Therapie

Standard Chemo für CUP (Carboplatin/Paclitaxel) vs. site-specific Chemo according to the putative (mutmaßlichen) Primary

[Bochtler2013](file:///d:/Thoracic_Disease/14_Rare Malign Disease/CUP- Cancer of Unknown Primary Site\Does Cancer of Unknown Primary (CUP) Truly Exist as a Distinct Cancer Entity.pdf)