Intranodal palisaded myofibroblastoma (IPM) is a rare primary tumour of

lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inc ...

s, that classically presents as an

inguinal In human anatomy, the inguinal region refers to either the groin or the lower lateral regions of the abdomen. It may also refer to: * Conjoint tendon, previously known as the inguinal aponeurotic falx, a structure formed from the transversus abdo ...

mass. It afflicts predominantly males of middle age.

Signs and symptoms

IPMs present as painless

lymphadenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In cl ...

. They usually are found in the inguinal region and grow slowly. The signs and symptoms are non-specific, i.e. it is not possible to diagnose an IPM from the symptoms and manner in which they present.The main (clinical) differential diagnosis of IPM is

metastatic Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, ...

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal bl ...

, e.g.

squamous cell carcinoma Squamous-cell carcinomas (SCCs), also known as epidermoid carcinomas, comprise a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on th ...

malignant melanoma Melanoma, also redundantly known as malignant melanoma, is a type of skin cancer that develops from the pigment-producing cells known as melanocytes. Melanomas typically occur in the skin, but may rarely occur in the mouth, intestines, or eye (u ...

adenocarcinoma Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) (AC) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, o ...

Diagnosis

IPMs are diagnosed by examination of the tissue by a

pathologist Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in t ...

. They have a rim of peripheral

lymphoid tissue The lymphatic system, or lymphoid system, is an organ system in vertebrates that is part of the immune system, and complementary to the circulatory system. It consists of a large network of lymphatic vessels, lymph nodes, lymphatic or lymphoid ...

(remnant of a

) and consist of

spindle cell Von Economo neurons (VENs), also called spindle neurons, are a specific class of mammalian cortical neurons characterized by a large spindle-shaped soma (or body) gradually tapering into a single apical axon (the ramification that ''transmits' ...

s with

nuclear Nuclear may refer to: Physics Relating to the nucleus of the atom: *Nuclear engineering *Nuclear physics *Nuclear power *Nuclear reactor *Nuclear weapon *Nuclear medicine *Radiation therapy *Nuclear warfare Mathematics *Nuclear space * Nuclear ...

palisading.

Red blood cell Red blood cells (RBCs), also referred to as red cells, red blood corpuscles (in humans or other animals not having nucleus in red blood cells), haematids, erythroid cells or erythrocytes (from Greek ''erythros'' for "red" and ''kytos'' for "holl ...

extravasation is common and

blood vessel Blood vessels are the structures of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away from ...

s surrounded by collagen with (fine) peripheral

spoke A spoke is one of some number of rods radiating from the center of a wheel (the hub where the axle connects), connecting the hub with the round traction surface. The term originally referred to portions of a log that had been riven (split l ...

s (amianthoid fibers) are usually seen.

Immunostain In biochemistry, immunostaining is any use of an antibody-based method to detect a specific protein in a sample. The term "immunostaining" was originally used to refer to the immunohistochemical staining of tissue sections, as first described by A ...

s for

smooth muscle Smooth muscle is an involuntary non- striated muscle, so-called because it has no sarcomeres and therefore no striations (''bands'' or ''stripes''). It is divided into two subgroups, single-unit and multiunit smooth muscle. Within single-unit ...

actin Actin is a protein family, family of Globular protein, globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in myofibril, muscle fibrils. It is found in essentially all Eukaryote, eukaryotic cel ...

and

cyclin D1 Cyclin D1 is a protein that in humans is encoded by the ''CCND1'' gene. Gene expression The CCND1 gene encodes the cyclin D1 protein. The human CCND1 gene is located on the long arm of chromosome 11 (band 11q13). It is 13,388 base pairs lon ...

are characteristically positive. The main

histologic Histology, also known as microscopic anatomy or microanatomy, is the branch of biology which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vis ...

differential diagnosis is

schwannoma A schwannoma (or neurilemmoma) is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves. Schwannomas are homogeneous tumors, consisting only of Schwann cells ...

. Image: Intranodal palisaded myofibroblastoma - low mag.jpg , Low mag. Image: Intranodal palisaded myofibroblastoma - high mag.jpg , High mag.

Treatment

Simple surgical excision is considered curative. Rare recurrences have been reported.

References

{{Lymphatic organ disease Diseases of veins, lymphatic vessels and lymph nodes

Signs and symptoms

Diagnosis

Treatment

See also

References