Tissus Mous


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Présentation Clinique / Clinical setting

Femme de 31 ans se présentant pour douleur intense quasi-permanente au siège d'une résection tumorale des tissus mous, antrieure au niveau de sa jambe, il y a deux ans, avec le diagnostic d'hémangiopéricytome. Une résection large du siège d'intervention antérieure est réalisé.

Patient 31 yo female with an excruciating pain non subsiding on the anterior aspect of the lower leg, in the location of previous surgery of soft tissue tumor. Previous diagnosis: hemangiopericytoma. Wide excision of the painful lesion is performed.


Radiologie / Radiology




IRM de la lésion antérieure objective la profondeur de la lésion des tissus mous, et son caractère partiellement kystique.

MRI of the previous soft tissue tumor, deep in contact with the periostium, partly cystic.


Pathologie / Pathology


Dans les fragments de tissus mous il est observé des nodules cellulaires de taille variable.Ces nodules sont bien circonscrits.

In the soft tissue sections, cellular nodules are observed well circumscribed.



Ces nodules présentent  par place des amas et des éléments disséquant en files indiennes le tissu conjonctif péri-nodulaire. Ces nodules sont formés de cellules cuboïdes semblant adoptés un arrangement tantôt en nappe ou en amas

Some show in the surrounding connective tissue, clusters and indian file pattern. Cuboid and ovoid cells are arranged in sheets in the central part of the nodules, 


Ils présentent par ailleurs des foyers d'accumulation de substance hyaline interstitielle.

Some interstitial hyalin material is noted.


Le PAS objective une positivit pericellulaire, et il existe un engainement unicellulaire par une trame rticulinique grle dans les nappes cellulaire, dlimitant les amas.

PAS stain discloses pericellular positivity. Silver stain demonstrates a dense meshwork surroundind the cells individually, and the cell clusters.



Dans certains fragments, la prolifération est mal délimitée. Dans le tissu conjonctif adjacent, il existe des structures angiomateuses

The proliferation is ill-circumscribed in some fragments. The surrounding connective tissue harbors angiomatous formations


Ces dernières exhibent des aspects de structures glomiques hyperplasiques.

The latter shows a pattern of hyperplastic glomus structures.


Immunohistochimie / Immunohistochemistry



Les cellules sont positives l'Actine Muscle Lisse, cependant cette positivité est absente dans les foyers compacts. Le CD34 est négatif, de même les pancytokératines, et l'EMA.

The cells are positive to Smooth Muscle Actin. Some central areas of the nodules are negative.CD34 is negative, so are the cytokeratins, and EMA.


 La Proteine S100 révèle la présence d'une positivité au niveau de cellules fusiformes (schwannienne?) au sein de la prolifration.

  PS100 reveals the presence of a small spindle cell component (schwann cells?).


Pathologie Antérieure / Previous Pathology



Les lames des la tumorectomie antérieure soumises pour évaluation dans la présentation actuelle montrent une aspect typique d'hémangiopricytome, tant sur le plan architectural (faible et moyen agrandissement), 

The slides submitted from the previous tumor exision for evaluation in the actual presentation, show a typical hemangiopericytomatous pattern at low, and medium power,



Absence d'atypie cytologique ( faible et moyen agrandissement)

With no cytological atypia (low and high power),

 Engainement monocelulaire  sur l'argentation.

 Abundant reticulin fibers surrounding the cells, and the vascular spaces.





En periphérie de certaines sections il existe des foyers dont les aspects histologiques sont comparables la lésion actuelle. Mais le matériel soumis est insuffisant pour une évaluation immunohistochimique.

The tumor shows, in some peripheral areas, histological patterns identical to the actual lesion. No additional material was available for immunohistochemistry.


Diagnostic Proposé / Proposed diagnosis:

Tumeur du Glomus / Glomus tumor


(Ackerman's Surgical Pathology, 8 th Ed.)

Glomus tumor, also known as glomangioma, originates in the neuromyoarterial glomus, a normal
arteriovenous shunt abundantly supplied with nerve fibers and fulfilling a temperature-regulating function. The classical location of the glomus tumor is the subungual region, but it can occur elsewhere in the skin, soft tissues (particularly in the flexor surface of the arms and about the knee), nerves, stomach, nasal cavity, and trachea. It has also been reported in the sacrococcygeal region, arising from the coccygeal body (glomus coccygeum) and associated with coccydinia, but there is some question as to whether this is a true neoplasm or simply a normal structure of this region.
Subungual lesions are always supplied by numerous nerve fibers and are exquisitely painful, two features often absent in glomus tumors arising elsewhere. The tumor may erode the terminal phalanx or even present as an intraosseous lesion in this location. Superficial lesions are well circumscribed. Glomus tumors in children tend to be multiple and of an infiltrative nature. They may present clinically as varicosities of the lower extremities. 
Microscopically, glomus tumors consist of blood vessels lined by normal endothelial cells and surrounded by a solid proliferation of round or cuboidal "epithelioid" cells with perfectly round nuclei and acidophilic cytoplasm. As seen under an electron microscope, the tumor cells have features of smooth muscle rather than of pericytes. Immunohistochemically, they manifest reactivity for myosin,vimentin, actin, and basal lamina components but not for desmin. Numerous substance Pcontaining nervefibers have been detected among the glomus cells.

Three microscopic types of glomus tumor have been recognized: solid, angiomatous, and myxoid. The solid type can be confused with sweat gland tumor, melanocytic nevus, or metastatic carcinoma. This is particularly the case when the tumor cells are very epithelioid and/or grow in an Indian-file fashion. An oncocytic variant of glomus tumor, in which the cytoplasm of the glomus cells is packed with mitochondria, has also been described. Often the diagnostic relationship between tumor cells and blood vessels can be clearly seen only at the very periphery of the neoplasm. Mast cells are common. 

On rare occasions, glomus tumors behave in an aggressive fashion, with local recurrences and invasion of adjacent structures. In other instances, lesions with the typical cytoarchitectural features of glomus tumor merge with a cytologically malignant tumor; these have been designated glomangiosarcomas.
Theoretically, a de novo glomangiosarcoma should also exist, but its morphologic recognition (particularly its distinction from vascular/epithelioid forms of leiomyosarcoma) would be most difficult. No convincing examples of metastasizing glomus tumors have been reported.