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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

High grade spindle cell neoplasm (leiomyosarcoma) with marked cellularity and pleomorphism with increased mitotic activity and areas of necrosis present elsewhere in the tumor (Hematoxylin & Eosin, ×20).
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fig003: High grade spindle cell neoplasm (leiomyosarcoma) with marked cellularity and pleomorphism with increased mitotic activity and areas of necrosis present elsewhere in the tumor (Hematoxylin & Eosin, ×20).

Mentions: The presumptive radiological diagnosis was recurrent angiomyxoma. She underwent embolization of the feeding vessels prior to the surgical resection, which involved a complete excision of the mass without entry into the capsule of the tumor. There was no local invasion of any surrounding structures. This was followed by a gracilis muscle flap closure of the resultant perineal defect. The resected tumor measured 11.5×10.0×9.5 cm and weighed 402 grams. Histopathology revealed a high grade spindle cell neoplasm characterized by marked cellularity and pleomorphism with increased mitotic activity (>5 per 10 high power fields) and areas of necrosis (Figure 3). Immunohistochemistry showed positive immunoreactivity of the tumor cells for actin and desmin while S-100, CD117 and keratin AE1/AE3 were negative, confirming the diagnosis of a high grade leiomyosarcoma. The anal margin was involved by tumor. The previously excised specimen from 14 months ago was compared to the current tumor with the former demonstrating a multilobulated hypocellular tumor with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation consistent with a diagnosis of superficial angiomyxoma (Figure 1). No atypia, increased mitotic activity or necrosis was present. She underwent adjuvant radiation therapy receiving 60 Gray in 30 fractions to the post-operative bed involving the left perineum and pelvis followed by a boost of 6 Gray in 3 fractions to the area of positive margin along the anal canal (total dose: 66 Gray in 33 fractions.) She was also offered adjuvant chemotherapy with adriamycin and ifosfamide, which she declined. She has had close follow-up clinical exams and imaging for the last 36 months, without any recurrence of her disease.


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)

High grade spindle cell neoplasm (leiomyosarcoma) with marked cellularity and pleomorphism with increased mitotic activity and areas of necrosis present elsewhere in the tumor (Hematoxylin & Eosin, ×20).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig003: High grade spindle cell neoplasm (leiomyosarcoma) with marked cellularity and pleomorphism with increased mitotic activity and areas of necrosis present elsewhere in the tumor (Hematoxylin & Eosin, ×20).
Mentions: The presumptive radiological diagnosis was recurrent angiomyxoma. She underwent embolization of the feeding vessels prior to the surgical resection, which involved a complete excision of the mass without entry into the capsule of the tumor. There was no local invasion of any surrounding structures. This was followed by a gracilis muscle flap closure of the resultant perineal defect. The resected tumor measured 11.5×10.0×9.5 cm and weighed 402 grams. Histopathology revealed a high grade spindle cell neoplasm characterized by marked cellularity and pleomorphism with increased mitotic activity (>5 per 10 high power fields) and areas of necrosis (Figure 3). Immunohistochemistry showed positive immunoreactivity of the tumor cells for actin and desmin while S-100, CD117 and keratin AE1/AE3 were negative, confirming the diagnosis of a high grade leiomyosarcoma. The anal margin was involved by tumor. The previously excised specimen from 14 months ago was compared to the current tumor with the former demonstrating a multilobulated hypocellular tumor with circumscribed borders composed of bland stellate and spindled cells in a loose stroma with scattered thin walled vessels and sparse inflammation consistent with a diagnosis of superficial angiomyxoma (Figure 1). No atypia, increased mitotic activity or necrosis was present. She underwent adjuvant radiation therapy receiving 60 Gray in 30 fractions to the post-operative bed involving the left perineum and pelvis followed by a boost of 6 Gray in 3 fractions to the area of positive margin along the anal canal (total dose: 66 Gray in 33 fractions.) She was also offered adjuvant chemotherapy with adriamycin and ifosfamide, which she declined. She has had close follow-up clinical exams and imaging for the last 36 months, without any recurrence of her disease.

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