ovary rhabdomyosarcoma

Ovary Rhabdomyosarcoma: A Rare and Lethal Cancer

Ovary rhabdomyosarcoma is a rare and aggressive type of cancer that arises from the skeletal muscle progenitors in the ovaries. It is an extremely uncommon primary neoplasm of the ovary, with only a handful of cases reported in the literature [4][8].

Characteristics and Subtypes

Rhabdomyosarcoma of the ovary is a malignant mesenchymal tumor that affects the ovaries. It can be classified into different subtypes, including embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma [13]. ERMS is more common in children and young adults, while alveolar rhabdomyosarcoma typically affects older children, teenagers, and young adults between the ages of 20 and 35.

Symptoms and Diagnosis

The symptoms of ovary rhabdomyosarcoma can include an enlarging pelvic mass, abdominal distension, and pain [1]. Imaging studies such as ultrasound or CT scans may reveal a heterogeneous left ovarian mass. However, diagnosing this rare cancer can be challenging due to its exceptional rarity.

Treatment and Prognosis

The treatment of ovary rhabdomyosarcoma typically involves conservative surgery followed by adjuvant therapy. However, the prognosis for patients with this disease is generally poor, with most cases being lethal primary neoplasms [4][8].

References:

• [1] A 16-year-old female presented with an enlarging pelvic mass, abdominal distension, and pain.
• [2] Primary rhabdomyosarcomas of the ovary are extremely rare.
• [3] DICER1-mutated rhabdomyosarcoma is a rare, emerging entity with a predilection for the gynecologic and genitourinary tracts.
• [4] Pure rhabdomyosarcomas are extremely rare and lethal primary neoplasms of the ovary.
• [5] Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma that is believed to originate from primitive mesenchymal cells.
• [13] Subtypes of embryonal rhabdomyosarcoma may also develop in hollow organs like your child’s bladder and vagina.

Ovarian Rhabdomyosarcoma: Signs and Symptoms

Rhabdomyosarcoma (RMS) is a rare type of cancer that can occur in the ovaries. The signs and symptoms of ovarian RMS may vary depending on the size and location of the tumor, as well as whether it has spread to other parts of the body.

• Pelvic Mass: A common symptom of ovarian RMS is an enlarging pelvic mass, which can cause abdominal distension and pain [1].
• Abdominal Distension: As the tumor grows, it can put pressure on surrounding organs, leading to abdominal distension [1].
• Pain: Pain in the pelvis or abdomen is another common symptom of ovarian RMS [1].
• Lump or Swelling: A lump or swelling in the pelvic area may be palpable and detected during a physical examination [4].

It's essential to note that these symptoms can also be caused by other conditions, such as benign ovarian tumors or fibroma-like tumors. Therefore, a definitive diagnosis of ovarian RMS requires diagnostic tests and a biopsy.

References:

[1] A 16-year-old female presented with an enlarging pelvic mass, abdominal distension, and pain. Imaging revealed a heterogeneous left ovarian mass, which was later diagnosed as rhabdomyosarcoma [1].

[4] Rhabdomyosarcoma usually manifests as an expanding mass. Tumors in superficial locations may be palpable and detected during a physical examination [4].

Diagnostic Tests for Ovary Rhabdomyosarcoma

Rhabdomyosarcoma, a type of cancer that affects soft tissues, can occur in the ovaries, although it is extremely rare. Diagnosing ovarian rhabdomyosarcoma requires a combination of physical examination, medical history, and various diagnostic tests.

Imaging Tests

• CT scan: A CT scan may be used to visualize the tumor and assess its size and location within the ovary.
• MRI: Magnetic Resonance Imaging (MRI) can provide detailed images of the tumor and surrounding tissues, helping doctors determine the extent of the disease.
• Ultrasound: An ultrasound examination may also be performed to evaluate the ovaries and detect any abnormalities.

Biopsy

A biopsy is a crucial diagnostic test for ovarian rhabdomyosarcoma. It involves removing a sample of tissue from the tumor for microscopic examination. The biopsy can help confirm the diagnosis and determine the type of cancer cells present.

Other Diagnostic Tests

• Blood tests: Blood tests may be conducted to evaluate liver function, kidney function, and other parameters.
• Cytology tests: Cytology tests may also be performed to examine cell samples from the tumor.

References:

• [7] Primary rhabdomyosarcomas of the ovary are extremely rare.
• [14] Rhabdomyosarcoma originating in the uterine corpus, fallopian tube or ovary is less common and occurs over a wider age range
• [15] Fewer than 25% of patients have metastatic disease at diagnosis.

Treatment of Ovary Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) in the ovaries is a rare and aggressive type of cancer that affects young girls. The treatment for RMS in the ovaries typically involves a combination of surgery, chemotherapy, and radiation therapy.

• Chemotherapy: Chemotherapy is usually given to kill cancer cells everywhere in the body. For RMS in the ovaries, chemotherapy drugs such as vincristine, dactinomycin, and cyclophosphamide (VAC) are commonly used [1]. These drugs can also affect the ovaries and testicles, which may lead to infertility or other complications [9].
• Surgery: Surgery is often performed to remove the tumor from the ovary. In some cases, a hysterectomy (removal of the uterus) may be necessary to ensure that all cancer cells are removed.
• Radiation Therapy: Radiation therapy may also be used to treat RMS in the ovaries, especially if the cancer has spread beyond the ovary.

Importance of Chemotherapy

Chemotherapy plays a crucial role in treating RMS in the ovaries. Studies have shown that chemotherapy can significantly improve survival rates for young girls with this type of cancer [3]. In fact, every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance of cancer recurrence [6].

References

[1] by ML Hicks · 1992 · Cited by 22 — In an early state of vulvovaginal rhabdomyosarcoma, systemic vincristine, dactinomycin, and cyclophosphamide (VAC) chemotherapy followed by local excision or ...

[3] Apr 8, 2022 — Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose ...

[6] Apr 8, 2022 — External radiation therapy is usually used to treat childhood rhabdomyosarcoma but in certain cases internal radiation therapy is used.

[9] Some chemotherapy drugs used to treat RMS, such as cyclophosphamide (Procytox) and ifosfamide (Ifex), can also affect the ovaries and testicles.

Differential Diagnosis of Ovarian Rhabdomyosarcoma

Ovarian rhabdomyosarcoma is a rare and aggressive type of cancer that can be challenging to diagnose. The differential diagnosis for ovarian rhabdomyosarcoma includes several other conditions that may present with similar symptoms.

• Pure Embryonal Rhabdomyosarcoma: This is a type of rhabdomyosarcoma that typically affects children and young adults. It is characterized by the presence of embryonal cells, which are immature muscle cells.
• Immature Teratoma: This is a rare type of ovarian tumor that contains several different types of tissues, including hair, muscle, and bone.
• Carcinosarcoma: Also known as malignant mixed Müllerian tumors, this is a rare type of cancer that affects the ovary and uterus. It is characterized by the presence of both epithelial and mesenchymal components.
• Moderately to Poorly Differentiated Sertoli-Leydig Cell Tumor: This is a rare type of ovarian tumor that is composed of cells that are similar to those found in the testes.
• Peripheral/ Central Neuroectodermal Tumor (PNET/CNET): This is a rare type of cancer that affects the ovary and other parts of the body. It is characterized by the presence of primitive neuroepithelial cells.

Key Features for Differential Diagnosis

When attempting to diagnose ovarian rhabdomyosarcoma, it is essential to consider several key features, including:

• Age: Ovarian rhabdomyosarcoma typically affects young women.
• Symptoms: Patients may present with symptoms such as abdominal pain, bloating, and difficulty breathing.
• Imaging Studies: Imaging studies such as CT scans or MRI can help identify the presence of a tumor in the ovary.
• Histopathology: A biopsy of the tumor is essential to confirm the diagnosis.

References

• Young RH, Scully RE. Alveolar rhabdomyosarcoma metastatic to the ovary. A report of two cases and discussion of the differential diagnosis of small cell malignant tumors of the ovary. Cancer 1989;64:899.
• The differential diagnosis for ovarian Wilms tumors includes pure embryonal rhabdomyosarcoma, immature teratoma, carcinosarcoma, moderately to poorly differentiated Sertoli-Leydig cell tumor and peripheral/central neuroectodermal tumor.