Pediatric Oncology Rare Tumors and Sarcoma (PORTS) Program

The Pediatric Oncology Rare Tumors and Sarcoma (PORTS) Program, part of the Division of Pediatric Hematology Oncology and Stem Cell Transplantation at Cohen Children’s, focuses on rare tumors, sarcomas and histiocytic disorders in children and adolescents. Our program coordinates the expertise of pediatric physicians from the medical, surgical and orthopaedic specialties as well as radiation oncology, diagnostic radiology and surgical pathology.

Our multidisciplinary team of physicians is able to network and consult with other experts around the world to provide personalized treatment plans tailored to each patient’s unique situation and clinical condition. The PORTS program allows children and their families who are affected by rare tumors, sarcomas and histiocytic disorders to receive expert care within close proximity to their communities.

In general, all childhood cancers are regarded as rare. Within childhood cancers, rare tumors account for 15 percent of all cancers in patients younger than age 20 and for 30 percent of all tumors in patients aged 15 to 19. Rare tumors in children range from certain cancers that occur only during childhood to adult cancers that are also found in children and young adults.

Due to rarity of these tumors, it is vital that young patients are referred to specialized pediatric centers that are familiar with these diseases for their treatment. Often, no standard diagnostic or treatment protocols exist for rare tumors. We have developed treatment regimens for multiple childhood rare tumors and, when applicable, we use advanced molecular diagnostic techniques to facilitate a timely and accurate diagnosis. Treatment options may include surgery, radiation therapy and chemotherapy.

Sarcomas are cancers of the musculoskeletal system that arise in either bone or soft tissue in various parts of the body. Sarcomas represent 10 to 12 percent of all pediatric cancers.

Bone sarcomas arise in the skeleton and the most common forms are osteosarcoma and Ewing sarcoma. Approximately 700 children and adolescents younger than 20 years old are diagnosed annually  in the U.S. with these primary bone tumors.

Soft tissue sarcomas (STS) are cancerous tumors of the muscle, tendons, fat, blood vessels and nerves. These tumors are most often found in the arms, legs, chest and abdomen in children but can develop anywhere in the body. As a group, they are highly invasive and have a high risk for local recurrence. They usually metastasize via the bloodstream and, less commonly, via the lymphatics.

STS can be divided into two groups: rhabdomyosarcoma (RMS) and nonrhabdomyosarcoma soft-tissue sarcomas (NRSTS). Although the cause is unknown, these tumors are treated with a combination of chemotherapy, surgery and, in some cases, radiation therapy. These tumors are best managed by a strong multidisciplinary team, including a pediatric oncologist, an orthopaedic oncologist, pediatric surgeon, pathologist and radiation oncologist. 

Langerhan cell histiocyotsis (LCH) is the most common histiocytic disorder in children. With this condition, the body overproduces abnormal Langerhan cells, which can form tumors affecting single or multiple bones and organs throughout the body.

LCH is a low-risk disease when it is seen in single or multiple low-risk organs. When there are multiple systems involved as well as high risk organs - including the spleen, liver and/or bone marrow - LCH is considered high risk and may be more difficult to treat. Some patients may develop long-term side effects such as diabetes insipidus, growth issues or neurologic problems, while other patients remain without side effects. In a minority of cases, the disease can be life-threatening.

Hemophagocytic lymphohistiocytosis (HLH) is another histiocytic disorder that can be divided into two categories. Some are primary (inherited/familial) while others are secondary to specific types of infections, malignancies, medications or rheumatologic disorders. It is important that HLH is diagnosed early so that treatment can be started before irreversible organ damage occurs.

Treatment of histiocytic disorders can include chemotherapy and immune therapy. With some isolated forms of LCH, only surgery or local steroid injection may be sufficient. 

To diagnose tumors and histiocytic disorders, our pediatric oncologists use a number of different tests to determine the type and location of the tumor. After a complete medical history and physical examination, your child’s physician will determine which tests are necessary. The diagnostic tests can include blood tests, an MRI, CT scan and PET/CT scan, a bone marrow test, and specialized tests based on the type of cancer suspected. A biopsy of the tumor will be necessary to determine the type of the tumor.

Treatment of histiocytic disorders will depend on the type, location and size of the tumors, the child’s age, medical history and the extent of the disease. Treatment options include chemotherapy, surgery and radiation therapy. Your child may undergo any one of these treatments or a combination of them.

We treat the following rare tumors, sarcomas and histiocytic disorders:

Rare tumors

• Carcinomas in childhood
  • Adrenocortical carcinoma and tumors
  • Colon cancer
  • Hepatocellular carcinoma
  • Mucoepidermoid carcinoma
  • Nasopharyngeal carcinoma
  • Pancreatic carcinoma
  • Renal cell carcinoma
  • Thymic carcinoma and thymomas

•   Neuroendocrine tumors            
• Pheochromocytoma
  • Paragangliomas
    • Carcinoid tumors
    • Pleuropulmonary blastoma (PPB)
    • Recurrent respiratory papillomatosis
    • Germ cell tumors
      • Germinomas
      • Yolk sac
      • Embryonal carcinoma
      • Choriocarcinoma

Sarcomas

• Bone sarcomas
  • Osteosarcoma
  • Ewing sarcoma 
  • Soft tissue sarcomas
    • Rhabdomyosarcoma (RMS)
    • Non-Rhabdomyosarcoma soft tissue sarcoma (NR-STS)
      • Alveolar soft part sarcoma
      • Dermatofibrosarcoma protuberans
      • Desmoid tumors
      • Desmoplastic small round cell tumors
      • Fibrosarcoma
      • Malignant peripheral nerve sheath tumor
      • Rhabdoid tumor
      • Synovial sarcoma
      • Undifferentiated sarcoma

Histiocytic disorders

• Langerhan cell histiocytosis (LCH)
• Hemophagocytic lymphohistiocytosis (HLH)
  • Primary HLH
  • Secondary HLH
  • Non-Langerhan cell histiocytosis
    • Erdheim Chester disease
    • Juvenile xanthogranuloma
    • Rosai dorfman

Our research is focused on understanding the genetic basis of rare tumors, sarcomas and histiocytic disorders and identifying novel treatment targets. We collaborate with other pediatric treatment centers to improve the diagnosis and treatment of rare tumors. We also actively participate in national registries for individual tumors.

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Donations to support the Pediatric Oncology Rare Tumors and Sarcoma (PORTS) Program may be made online. Please make sure to specify under “additional information” that the donation is for the Rare Tumors and Sarcoma Fund or for the Histiocyosis Research Fund.