Cure Alveolar Soft Part Sarcoma International (iCureASPS)

This write up was release a few months after our son Josh , was diagnosed .. it was humbling and frightening at the same time .
The emotions we all feel from time to time .
Be deligent with your scans. They are and will always be a valuable tool in fighting metastic disease .

Lung Metastasis of Primary Alveolar Soft-Part Sarcoma Occurring 20 Years after Initial Treatment

Case Report | Open Access
Volume 2013 |Article ID 690520 | https://doi.org/10.1155/2013/690520


Abstract

A 30-year old woman was referred to our center because of suspicion of a primary lung tumor of the right upper lobe. Histological examination of the lung lesion revealed lung metastasis of a previously treated alveolar soft part sarcoma of the musculus vastus medialis of the right femur, which was resected 20 years ago. Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. It is a slow-growing malignant soft tissue tumor arising in muscle tissue, usually in young adults. Due to pleural and extensive mediastinal infiltration with bilateral lung metastases, a systemic treatment with chemotherapy doxorubicin and ifosfamide was initiated. Late metastases from previously treated alveolar part sarcoma should be considered in patients with suspicious lung lesions even if surgical treatment was performed a long time ago.



https://www.hindawi.com/journals/crionm ... troduction

Written in 2013 but has a wealth of stats , on ASPS. 👍🏼


1. Introduction
Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. Alveolar soft-part sarcoma is a very uncommon soft tissue tumor known for late metastases to lung, bone, and brain. Lillehei and coworkers reported an interval of 21 years between primary presentation and development of lung metastasis and 33 years between primary presentation and development of brain and renal masses. Our case represents the second longest interval of 20 years between primary tumor presentation and development of a pulmonary recurrence.

2. Background
A 30-year old female patient was referred to our institution because of a large lung mass of the right upper lobe. The patient complained about increasing dyspnea for 2 months. Her initial physical examination was unremarkable and laboratory tests were all within normal limits. Spirometry revealed a mild restrictive pattern (TLC 88% soll) and reduced diffusion capacity (DLCO-SB 52% soll). CT thorax scan (Figure 1) revealed a huge tumor mass 13.2 × 13.7 × 11 cm in diameter of the right upper lobe of the lung with extensive pleural infiltration and infiltration of the mediastinum.


Figure 1

Contrast-enhanced computed tomography scan of a large tumor mass in the right upper lobe with extensive mediastinal infiltration.
Contrast-enhanced computed tomography scan (Figure 2) also revealed ipsilateral right-sided pleural tumor infiltration.


Figure 2

Contrast-enhanced computed tomography revealed pleural infiltration on the right side.
The patient, interestingly, had a history of alveolar soft-part sarcoma of the musculus vastus medialis of the right femur successfully resected 20 years ago. The primary alveolar soft-part sarcoma had been initially staged as IA (T1 N0 M0) 20 years ago. All regular follow-up evaluations were unremarkable. However, the last chest X-ray evaluation was performed 8 years ago. Clinical workup revealed bilateral pulmonary metastases with ipsilateral pleural infiltration.

Histological workup of material obtained during bronchoscopy showed a solid tumor composed of medium-sized to large cells arranged in compartmentalizing cords and nests, which were separated by delicate septa with capillaries. The cytoplasm of the tumor cells was broad and clear or eosinophilic. The nuclei were round and uniform, frequently with a single nucleolus (Figure 3).


Figure 3

The cytoplasm of the tumor cells was broad and clear or eosinophilic. The nuclei were round and uniform, frequently with a single nucleolus.
After immunohistochemistry demonstrating reactivity of the tumor cells for protein S100, but not for melanocytic markers or keratin markers, and comparison with the primary tumor, a diagnosis of metastatic alveolar soft-part sarcoma was rendered.