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High Grade Leiomyosarcoma Mimicking a Recurrent Angiomyxoma in the Perineum.

Sood N, Swaika A, Hanooshi B, Waldorf J, Peterson J, Wu K, Attia S, Dinh TA - Rare Tumors (2015)

Bottom Line: Initially, she was treated for a presumptive recurrence of angiomyxoma.She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy.Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical and Surgical Gynecology, Mayo Clinic Florida , Jacksonville, FL, USA.

ABSTRACT
Perineal leiomyosarcoma is an extremely rare and aggressive cancer with a high metastatic potential and no defined standard treatment. There are only a few (six) reported cases in the literature. We report the case of a 67-year-old woman with a perineal leiomyosarcoma arising at the same site of a previously resected superficial angiomyxoma. Initially, she was treated for a presumptive recurrence of angiomyxoma. As she did not respond to medical therapy, she underwent repeat surgical excision. Pathology revealed a high grade leiomyosarcoma, histologically strikingly distinct from the initial diagnosis. She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy. This is the first reported case of a high grade perineal leiomyosarcoma originating at the same site as a resected benign superficial angiomyxoma. Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma.

No MeSH data available.


Related in: MedlinePlus

Multilobulated hypocellular tumor (angiomyxoma) with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation (Hematoxylin & Eosin, ×2). Inset: Hematoxylin & Eosin, ×20.
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fig001: Multilobulated hypocellular tumor (angiomyxoma) with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation (Hematoxylin & Eosin, ×2). Inset: Hematoxylin & Eosin, ×20.

Mentions: A 67-year-old Caucasian female presented to our clinic for the management of a recurrent perineal mass. Fourteen months prior, she had similar complaints and was diagnosed with superficial angiomyxoma of the perineum after surgical resection of the mass from the left paravaginal and pararectal space. The margins were negative. Immunohistochemistry of this 5×3×2.5 cm benign myxoid lesion was positive for smooth muscle actin and estrogen receptor, and negative for desmin and caldesmon. A diagnosis of superficial angiomyxoma was made (Figure 1). Two months after the procedure, she noted swelling with pain at the surgical site. A magnetic resonance imaging (MRI) of the pelvis confirmed a recurrent mass (6.4×6.2×8.5 cm) at the same site and was presumed to be a recurrent angiomyxoma. Biopsy was not obtained. The patient was started on anti-estrogen therapy with tamoxifen. She was continued on tamoxifen for a total of 8 months with very minimal resolution of her clinical symptoms. A repeated MRI revealed increasing size of the mass, for which she was switched to an aromatase inhibitor, letrozole. The patient was referred to our facility after 6 weeks of letrozole did not seem to provide any clinical or radiological benefit. The patient had a history of uterine leiomyoma for which she underwent a total abdominal hysterectomy and bilateral salpin-go-oophorectomy twenty-two years prior to this present presentation. She also had a history of vaginal dysplasia and was treated with excision sixteen years prior to presentation. At our first clinical visit, the patient complained of persistent pain in her perineal region, with noticeable discomfort in a seated position. She denied complaints with bladder or bowel habits. She denied any weight loss, fever or vaginal bleeding. A recto-vaginal examination revealed a large mass within the perineum, extending into the buttocks. MRI demonstrated a hypervascular, well circumscribed 9.8×8.1×8.8 cm ischioanal fossa tumor with considerable mass effect on adjacent structures but without invasion (Figure 2). Pelvic lymph nodes were not involved radiologically and the metastatic workup was negative.


High Grade Leiomyosarcoma Mimicking a Recurrent Angiomyxoma in the Perineum.

Sood N, Swaika A, Hanooshi B, Waldorf J, Peterson J, Wu K, Attia S, Dinh TA - Rare Tumors (2015)

Multilobulated hypocellular tumor (angiomyxoma) with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation (Hematoxylin & Eosin, ×2). Inset: Hematoxylin & Eosin, ×20.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508648&req=5

fig001: Multilobulated hypocellular tumor (angiomyxoma) with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation (Hematoxylin & Eosin, ×2). Inset: Hematoxylin & Eosin, ×20.
Mentions: A 67-year-old Caucasian female presented to our clinic for the management of a recurrent perineal mass. Fourteen months prior, she had similar complaints and was diagnosed with superficial angiomyxoma of the perineum after surgical resection of the mass from the left paravaginal and pararectal space. The margins were negative. Immunohistochemistry of this 5×3×2.5 cm benign myxoid lesion was positive for smooth muscle actin and estrogen receptor, and negative for desmin and caldesmon. A diagnosis of superficial angiomyxoma was made (Figure 1). Two months after the procedure, she noted swelling with pain at the surgical site. A magnetic resonance imaging (MRI) of the pelvis confirmed a recurrent mass (6.4×6.2×8.5 cm) at the same site and was presumed to be a recurrent angiomyxoma. Biopsy was not obtained. The patient was started on anti-estrogen therapy with tamoxifen. She was continued on tamoxifen for a total of 8 months with very minimal resolution of her clinical symptoms. A repeated MRI revealed increasing size of the mass, for which she was switched to an aromatase inhibitor, letrozole. The patient was referred to our facility after 6 weeks of letrozole did not seem to provide any clinical or radiological benefit. The patient had a history of uterine leiomyoma for which she underwent a total abdominal hysterectomy and bilateral salpin-go-oophorectomy twenty-two years prior to this present presentation. She also had a history of vaginal dysplasia and was treated with excision sixteen years prior to presentation. At our first clinical visit, the patient complained of persistent pain in her perineal region, with noticeable discomfort in a seated position. She denied complaints with bladder or bowel habits. She denied any weight loss, fever or vaginal bleeding. A recto-vaginal examination revealed a large mass within the perineum, extending into the buttocks. MRI demonstrated a hypervascular, well circumscribed 9.8×8.1×8.8 cm ischioanal fossa tumor with considerable mass effect on adjacent structures but without invasion (Figure 2). Pelvic lymph nodes were not involved radiologically and the metastatic workup was negative.

Bottom Line: Initially, she was treated for a presumptive recurrence of angiomyxoma.She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy.Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical and Surgical Gynecology, Mayo Clinic Florida , Jacksonville, FL, USA.

ABSTRACT
Perineal leiomyosarcoma is an extremely rare and aggressive cancer with a high metastatic potential and no defined standard treatment. There are only a few (six) reported cases in the literature. We report the case of a 67-year-old woman with a perineal leiomyosarcoma arising at the same site of a previously resected superficial angiomyxoma. Initially, she was treated for a presumptive recurrence of angiomyxoma. As she did not respond to medical therapy, she underwent repeat surgical excision. Pathology revealed a high grade leiomyosarcoma, histologically strikingly distinct from the initial diagnosis. She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy. This is the first reported case of a high grade perineal leiomyosarcoma originating at the same site as a resected benign superficial angiomyxoma. Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma.

No MeSH data available.


Related in: MedlinePlus