Tractus Génital Feminin

Cas 0809349

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  Femme de 67 ans se plaignant de pertes sanguinolantes. Antécédents de carcinome du sein, traité il y 6 ans. A l'examen clinique, présence d'une tumeur bourgeonnante an niveau col utérin, et à l'echographie comblant la cavité utérine.   A 67 yo woman complaining of uterine bleeding. History of breast carcinoma treated 6 years ago. Clinical examination reveals a mass protruding from the cervix. Ultrasound discloses an enlarged uterus, filled by the mass.  
  Hysterectomie totale.   Total hysterectomy.  
  Macroscopie: pièce ouverte longitudinalement.   Gross: Specimen opened transversally.  
     
     
     
     
  Desmine   Myogenin (MYF4)  
     
  Invasion vasculaire   Cytokeratin (AE1-AE3)  
 

Diagnostic proposé:

 

 

 

 

 

 

 

 

 

 

Tumeur mixte mullerienne maligne avec composantes hétérologues.

 

Proposed diagnosis:

 

 

 

 

 

 

 

 

 

 

Malignant mixed müllerian tumor with heterologous components

 
 

Arguments

Dans le texte de la réference ci-dessous (en gras)

 

 

In the highlighted reference text below

 

Reference: Ackerman's Surgical Pathology (8 Ed.)

Malignant mixed müllerian tumor (mixed mesodermal tumor)

Malignant mixed müllerian tumors are rare uterine neoplasms that are seen practically always in postmenopausal patients, although exceptions occur. They present with uterine bleeding and enlargement. The usual location is the uterine body, particularly the posterior wall in the region of the fundus. Grossly, they present as large, soft, polypoid growths involving the endometrium and myometrium, sometimes protruding from the cervix. Foci of necrosis and hemorrhage are common.

Microscopically, the characteristic feature is the admixture of carcinomatous and sarcoma-like elements, resulting in a characteristic biphasic appearance. The carcinomatous component is usually of glandular type, whether endometrioid, clear cell, or papillary serous. As a rule, it is of poorly differentiated appearance and high-grade nature; therefore a careful search for stromal elements should be carried out whenever such patterns are found in an endometrial D&C, particularly if accompanied by extensive necrosis and hemorrhage. Squamous cell, undifferentiated, and primitive neurectodermal patterns may also be seen. The appearance of the sarcomatous component is the basis for the time-honored division of these neoplasms into a homologous and a heterologous variety. In the former, the malignant stroma is formed by either round cells resembling those of the endometrial stroma or by spindle cells resembling leiomyosarcoma or fibrosarcoma. In the latter, specific heterologous mesenchymal elements (such as skeletal muscle, cartilage, bone, or fat) also are present. Identification of cross striations, or of skeletal muscle markers by immunocytochemistry, is required to document the presence of a rhabdomyosarcomatous component. The distinction between these two varieties may require the study of numerous sections and therefore may not be possible on material obtained by curettage. Actually, the malignant stroma may be so inconspicuous in such a specimen as to be missed altogether, the lesion being misdiagnosed as an ordinary adenocarcinoma. This is particularly the case for the peritoneal metastases, in which the stromal component is often scanty or altogether absent. Since the epithelial elements can form papillae and be accompanied by psammoma bodies, a confusion with metastatic papillary serous cystadenocarcinoma from the ovary may occur. Parenthetically, cases have been reported of coexistent uterine malignant mixed müllerian tumor and ovarian serous adenocarcinoma. Exceptionally, a rhabdoid component is present.

The very fact that it is the epithelial component of the tumor that shows the most capability for invasion and metastases suggests that these tumors should be primarily regarded as carcinomas rather than sarcomas, employing a reasoning analogous to that currently accepted for carcinomas with sarcoma-like stroma of the upper aerodigestive tract and other sites. Immunohistochemical and ultrastructural studies support this view: Keratin is always detectable in the epithelial areas, but is also present in the sarcomatous component in over half of the cases; by electron microscopy, hybrid epithelial/stromal cells coexist with those having purely epithelial or stromal features. An additional supportive finding is the concordant pattern of p53 staining in the carcinomatous and sarcomatous areas, which would be difficult to explain if these tumors were biclonal.

  Malignant mixed müllerian tumors are easily distinguished from teratomas by their occurrence in an older age group and by the absence of skin appendages, glia, thyroid, and other tissue; it should be noted, however, that they may exceptionally contain neuroectodermal elements. They should also be clearly separated from botryoid rhabdomyosarcoma (sarcoma botryoides). The latter term should be reserved for the tumor of childhood or adolescence arising from the cervix or vagina and lacking a carcinomatous component.  
 

Mixed müllerian tumors are highly aggressive neoplasms, more so than even the higher grades and the more unfavorable variants of endometrial carcinoma. Extension into the pelvis, lymphatic and vascular permeation, and distant lymph-borne and blood-borne metastases are common. If the tumor has extended to the serosa of the uterus or beyond at the time of surgery, the prognosis is hopeless. The only patients with some chance of cure are those in whom the tumor is restricted to the inner half of the myometrium at the time of surgery. This determination implies a thorough sampling of the hysterectomy specimen by the pathologist.

 
 

In many series, tumors having only homologous stromal elements (called "carcinosarcomas" by Norris and Taylor) have been found to have a slightly better prognosis than those with heterologous elements, a fact that may justify their separation. It should be pointed out, however, that the difference between the two is small, in some series absent altogether, and far outweighed by the stage of the disease. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy is the treatment of choice. The response to radiation therapy and chemotherapy has been generally poor, although some encouraging reports have appeared. The most common sites of recurrent disease are lung and abdominal cavity.

 
  Norris and Taylor made the disturbing observation that 30% of the patients with heterologous malignant mixed müllerian tumors and 13% of those with homologous tumors that they studied had a history of previous irradiation to the pelvic area, usually given for some benign disorder. The median interval between irradiation and the time of diagnosis of the tumor was 16.4 years both in their series and in that of Doss et al. Postirradiation uterine sarcomas tend to occur in a younger age group and spread earlier to the pelvis than comparable tumors not related to irradiation.  

Mixed müllerian tumors and other sarcomas of the endometrial stroma have also been seen in association with chronic estrogenic stimulation (ovarian thecoma, polycystic ovarian disease, and prolonged estrogen therapy).

These tumors can arise in extrauterine locations, the most common sites being ovary and pelvic structures. Their morphologic and immunohistochemical features are similar to those of their uterine counterparts.