Clinico-Pathological Spectrum of Alveolar Soft Part Sarcoma: Case Series from a Tertiary Care Cancer Referral Centre in India with a Focus on Unusual Clinical and Histological Features
Kanwalpreet KAUR, Amisha GAMI, Ashini SHAH, Jahnvi GANDHI, Priti TRIVEDI
Department of Oncopathology, Gujarat Cancer and Research Institute, AHMEDABAD, INDIA
Keywords: Alveolar soft part sarcoma, IHC, TFE3, Apple bite nuclei
Alveolar soft part sarcoma (ASPS) is characterized by distinctive histomorphology of variably discohesive epithelioid cells arranged
in nests and translocation of t(x;17) (p11.2;q25) resulting in ASPSCR1-TFE3 fusion. The aim of the present study is to review the clinical,
histopathological, and immunohistochemical profile of ASPS with a focus on unusual histological features.
Material and Method: The present study is retrospective and descriptive. All cases with a diagnosis of ASPS were retrieved with clinical and
Results: 22 patients of ASPS were identified. The most common site was the lower extremity and the size range was 3-22 cm. 54.5% of the patients
had metastasis, with the lung as the most common site. Metastasis preceded detection of primary tumour in two cases. All cases showed similar
histopathology of monomorphic epithelioid cells arranged in nests encircled by sinusoidal vasculature. Architecturally, the organoid pattern
(81.8%) was followed by the alveolar pattern. 68.2% of the cases showed apple bite nuclei as the predominant nuclear feature. Rare nuclear
features included binucleation (n=13), multinucleation (n=8), pleomorphism (n=4), nuclear grooves in three cases and intranuclear inclusion
in one case, mitosis (n=5), and focal necrosis (n=6). All cases were positive for TFE3 and negative for AE1/AE3, EMA, HMB45, PAX8, MyoD1,
SMA, synaptophysin, and chromogranin. Only two cases showed focal S100 positivity while one showed focal desmin positivity.
Conclusion: Diffuse strong nuclear TFE3 positivity is sensitive for ASPS in an appropriate clinicoradiological context. Due to the high propensity
for early metastasis, complete metastatic work-up and long term follow up is recommended.
Alveolar soft part sarcomas (ASPS) are rare soft tissue
tumors of uncertain histogenesis having a distinctive
histomorphological appearance of variably discohesive
epithelioid cells arranged in nests and have a specific translocation
of t(x::17)(p11.2;q25) resulting in ASPSCR1-TFE3
Marked histologic overlap with other tumors, and tumor
at unusual site and unusual clinical presentation with mass
at the metastatic site prior to the identification of the primary
make the diagnosis tricky. The differential diagnoses
include a broad range of mesenchymal and non-mesenchymal
neoplasms such as paraganglioma, PEComa, granular
cell tumor, metastatic carcinoma such as metastatic renal
cell carcinoma, hepatocellular carcinoma, and adrenal cortical
The present study analyzes the clinical, histopathological,
and immunohistochemical profile of ASPS and clinical
outcomes in cases, wherever available. Particular emphasis
was given on the unusual histological features. The differential
diagnosis and potential pitfalls in the current era of
the increasing spectrum of TFE3 rearranged tumors have
The present study is retrospective and all cases with a histopathological
diagnosis of ASPS were retrieved from the
archives of the department of Oncopathology from 2012 to
2021 at a tertiary care cancer center. Demographic, clinical,
and radiological data were retrieved from the case records.
Cases with non-availability of either immunohistochemistry
(IHC) or paraffin blocks were excluded. Histomorphological
and immunohistochemical characteristics were analyzed in each case. TFE3 immunohistochemistry was
performed wherever unavailable. The histological parameters
evaluated were growth pattern, presence of crystals
confirmed by periodic acid-Schiff stain with diastase (PASD),
nuclear features, presence of inflammation, fibrous septa,
vascular invasion, necrosis, cystic change and myxoid
A total of 22 patients (0.4%) with ASPS out of 5541 soft
tissue sarcomas were identified from 2012 to 2021. The
patient age range was 2-47 years and the median age was 27
years. The M:F ratio was 0.8:1. The most common site was
the lower extremity in 45% (10/22) of the cases followed by
the upper extremity 27.3% (6/22) of the cases, retroperitoneum
in 18.1% (4/22) cases and one case each in the head and neck, chest wall, and lung. Clinical, radiological, and
outcome details are given in Table I
Tumor size varied from 3 cm to 22 cm with a mean size of
7.8 cm. Lymph node metastasis was seen in 2 cases only.
Distant metastasis (54.5%;12/22) was more frequent than
lymph node metastasis. Out of these 12 cases with metastasis,
91.7% of the patients had synchronous metastasis while
three showed metachronous metastasis. The lung was the
most common site (90.9%) followed by the brain, bone,
and liver (Figure 1). In one case of ASPS of the forearm, an
unusual site of metastasis was bilateral nasal cavities, with
biopsy showing a submucosal tumor. Five patients amongst
these had multiple site metastasis. The metastasis preceded
detection of the primary tumor in two cases. One case presented
with a posterior fossa mass and the second case with a pathological fracture of the right femur, and both were
diagnosed as ASPS on biopsy. Subsequently, PET revealed
a primary mass in the left iliac region and the right thigh
respectively. Thus, most cases presented with AJCC stage
IV at the time of diagnosis (54.5%;12/22), followed by stage
IIIa (22.7%; 5/22), stage I (13.6%; 3/22), and stage II (0.9%;
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|Figure 1: A) MRI pelvis showing heterogeneously enhancing 13x10x11 cm3 soft tissue lesion with lytic lesion in left iliac bone and left
acetabulum which is hypointense in T1W and heterogenous hyperintense T2W, B) Gross of ASPS showing multilobular fleshy tumour
with lymphovascular invasion, C) Axial CT thorax revealed presence of multiple metastatic lesions of variable sizes, diffusely scattered in
both lung fields, D) CT skull show dural based metastasis.
One patient with T-ALL post remission showed ASPS in
the left paravertebral location, post remission. The sibling
of the patient also had T-ALL and developed glioblastoma
4 years post remission. The patient was further evaluated
and diagnosed with constitutional mismatch repair deficiency
syndrome (CMMRD) with a homozygous deletion
(chr7:6026910; delC) detected in exon 11 of the PMS2 gene.
Microscopically, all cases showed a multilobular architecture
separated by fibrotic bands. The most predominant
architecture pattern of the tumor cells within the lobules
was the organoid pattern (81.8%;18/22) followed by the
alveolar pattern (n=4) encircled by sinusoidal capillary vasculature
(Figure 2A,B). The size of the nests was variable
with the number of cells in one nest varying from 10 cells
to as many as 200 cells. Focal solid areas without any intervening
vasculature were seen in 3 cases (Figure 2C). Thick
fibrotic bands were seen in 50% (n=11) of the cases (Figure 2D). The rare architectural features noted were infiltration
of single cells in septa and focal spindling of tumor cells in
3 cases each (Figure 2E).
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|Figure 2: Histomorphological features of ASPS, A) Alveolar pattern (H&E, x100), B) Organoid pattern with spindling (H&E, x100),
C) Solid pattern (H&E, x100), D) Thick fibrous septae (H&E, x100), E) Single cell infiltration in septae (H&E, x100), F) Classical
vesicular nucleus with prominent eosinophilic nucleoli (H&E, x400).
Cytologically, tumor cells were epithelioid or polygonal
with abundant eosinophilic granular cytoplasm in 91% of
the cases and predominantly clear cytoplasm in 2 cases.
It was also noted that the cytoplasm was more condensed
near the nucleus and clearing towards the edge of the cell.
The classically described round to oval nuclei with vesicular
chromatin and prominent eosinophilic nucleoli with anisonucleosis
was a major feature (>50% of the tumor nuclei) in
only 31.8 % (7/22) of the cases (Figure 2F) while in 68.2%
(15/22) of the cases the majority of the nuclei showed
wrinkling and a concave nuclear contour without nucleoli,
described as apple bite nuclei (Figure 3A). Rare nuclear
features included binucleation (n=13), multinucleation
(n=8), pleomorphism (n=4), and nuclear grooves in three
cases and intranuclear inclusion in one case (Figure 3B-F).
Mitotic activity in general in ASPS is rare with only 5 cases
showing occasional mitoses. Necrosis was infrequent and
focally seen only in 6 cases. A lymphovascular embolus was
a common phenomenon seen in 50% of the cases. None of
our cases showed perineural invasion. Intratumoral hemorrhage
in the center of the nests was seen in 2 cases.
Click Here to Zoom
|Figure 3: Unusual Histomorphological features of ASPS, A) crenated, crescent shaped apple bite nuclei (H&E, x400), B) nuclear grooves
(H&E, x400), C) intranuclear inclusion (H&E, x400), D) mitosis (H&E, x400), E) multinucleation (H&E, x400), F) nuclear pleomorphism
(H&E, x400), G) PAS D positive needle shaped crystals (PAS-D, x400), H) Diffuse nuclear TFE positive (IHC, x400), I) Focal desmin
positive (IHC, x400).
Many cells with PAS-D positive rod-like crystalline structures
in a sheaf-like or stacked configuration in the cytoplasm
were seen in 5 cases while this was seen in occasional
cells in 3 cases (Figure 3G). There was no significant inflammatory
host response in any case. There were focal intratumoral
lymphocytes in 2 cases but peritumoral lymphocytes
were seen in only one case. Other inflammatory cells such
as plasma cells, granulocytes and histiocytes were absent.
IHC was performed in all cases to rule out paraganglioma,
PEComa, granular cell tumor, metastatic carcinoma such as
renal cell carcinoma, hepatocellular carcinoma, or adrenocortical carcinoma that can mimic ASPS as per the clinical
context and morphological features. All cases showed diffuse
nuclear positivity for TFE3 (Figure 3H) and consistent
negativity for AE1/AE3, EMA, vimentin, HMB45, PAX8,
MyoD1, SMA, synaptophysin, and chromogranin. Only
two cases showed focal S100 positivity while one showed
focal desmin positivity (Figure 3I). Histomorphological
and IHC details are given in Table II.
All except one patient with localized disease of stage I-III
were treated with surgical resection with clear margins,
with no evidence of disease on follow-up.
Response was noted in 5 cases with tumor size <5 cm while
only 3 cases out of 16 cases with a diameter >5 cm showed no
evidence of disease on follow up. It was not affected by site
in our study. A single patient of ASPS of the lung with stage
I was treated with chemotherapy and radiotherapy with no
response, rather progression of disease with metachronous
metastasis to the liver and bone. All patients with disseminated
disease i.e. stage IV were treated with anthracycline
based chemotherapy and radiotherapy of 10 cycles of 30
gray. On regular follow-up, radiologically, no response but
rather progression of disease was seen with increase in the
size of the tumor at the primary site as well as an increase in
the size of the metastasis. Out of 4 paediatric patients (age
<17 years), a response was only noted in one case each of
stage I and stage II cancers while another two of stage IV
disease showed no response. No hospital death was reported
in any patient. All were alive with disease in the limited
period of follow-up ranging from 4 to 108 months.
ASPSs are rare soft tissue tumors that constitute < 1% of all
soft tissue sarcomas 1-3
. The present study mirrors similar
findings with only 0.4% the cases of ASPS out of all soft
tissue sarcomas diagnosed over a period of 10 years. Studies
have established that ASPS affects more commonly young
adults; concordantly the age range in the present cohort
was 2-47 years with four pediatric patients 1-16
. The literature
has a well documented female to male predominance
before the age of 30 years, with a reversed ratio for
older ages 1-7
. Our study also corroborates these findings
with the M:F ratio being 0.6:1 in patients less than 30 years
while all three patients of age >30 years were male. However,
Rekhi et. al. reported a male preponderance in their
. The prominent predilection for the extremities in
our series is also well reflected in earlier studies 1-3,7,10
A rare site seen in the present series was primary pulmonary
ASPS in a 25-year male without evidence of soft tissue
tumor elsewhere at the time of initial diagnosis confirmed
by the PET scan. To the best of our knowledge, only three
cases of primary pulmonary ASPS have been reported in
the English literature till date 8
The clinical course in our series illustrates the high incidence
of metastatic disease at the time of diagnosis with
50% of the cases. Many studies have reported metastatic
disease at diagnosis in 55% to 65% of the patients 4,7.
The most common metastatic site was the lung while brain
metastasis was always a part of disseminated metastasis and
never occurred in isolation, a phenomenon also observed
by Portera et al. and Keyton et al 7,10. In our study, in
two cases, metastases was detected prior to the finding of a primary, a phenomenon also encountered by other authors
2,12. One of our cases with the primary in the forearm
also presented with metastasis in the nasal cavity which
is not reported as the site of metastasis in any of the large
series, though rare cases of primary sinonasal ASPS have
been reported 1,3,7,9-12. Metastases to the lymph nodes
are uncommon and were seen in only 2 cases in the present
cohort. Portera et al. reported lymph node metastasis in a
single patient only out of 70 cases 7. Our study had the
first reported case of ASPS in patients with CMMRD 13.
CMMRD is a childhood cancer predisposition syndrome
caused by biallelic pathogenic variants in one of four mismatch
repair (MMR) genes, i.e., MLH1, MSH2, MSH6 and
PMS2. It is classically associated with hematological, brain,
and intestinal malignancies but rare in sarcoma. Only 30
MMR deficient bone and soft tissue sarcomas including
3 ASPS were encountered in the literature 13,14. ASPS
metastasis to the breast is considered extremely rare and is
reported only in a handful of cases but was seen in one of
our cases 15.
ASPS is known to have a very classical histomorphology
showing very little variation from case to case and site to
site. However, the diagnosis is challenging because of morphological
overlap with other tumors, particularly on small
biopsies and uncommon sites of occurrence or evaluation
of metastatic site prior to identification of primary such as
in biopsies from the posterior fossa, bone, or nasal cavity
in the present series. Difficulties are further confounded by
the occurrence of rare morphologic features particularly in
biopsies such as solid pattern, clear cytoplasm, and unusual
With regard to the pattern, the tumor always had a lobular
architecture with variably thick fibrous septae separating
the lobules. We noted a significant preponderance of
a ‘non-alveolar’ organoid growth pattern over the alveolar
pattern, despite the name of the entity. This needs to be kept
in mind, particularly when looking at a small biopsy.
Focal clear cytoplasm seen in two of our cases as a dominant
feature raises the possibility for these cases to be confused
with other clear cell tumors. The cells were also found
to have a feathery kind of cytoplasm with condensation of
the cytoplasm around the nucleus with pale cytoplasm at
the periphery giving a lacy skirt kind of appearance.
Most of the studies in the literature including WHO 2013
and the latest WHO 2020 classification of tumors of soft
tissue have emphasized vesicular nuclei with prominent
eosinophilic nucleoli as a characteristic feature of ASPS but
it was not the most prominent finding in the present series 1-12. The dominant nuclear feature (>50% of tumor
nuclei) were bland nuclei with marked nuclear folding
leading to concave, apple bite, and crenated nuclei without
any nucleoli in nearly 68.2% of the cases and these nuclei
were focal in the rest of the patients. These features were
first observed by Fanburg-Smith et al. and Chatura et al. in
lingual ASPS but it was an universal finding in the present
series, independent of site 12,16. We also observed focal
nuclear grooves in 3 cases which are not documented in
the literature. Intranuclear inclusion was seen in one case
and also observed in two cases by Rekhi et al 17. Awareness
of these nuclear features is important and should not
deviate one from the diagnosis of ASPS due to the absence
of classical vesicular nuclei, particularly in small biopsies.
The exact molecular pathogenetic relation between specific
cellular-level structural features and cancer genes is
not known. Nucleolar enlargement classically is associated
with increased ribosome production, and production of
new ribosomes appears essential for cell-cycle progression.
Nuclear envelope irregularity may be the effect of downstream
signaling pathway of the aberrant transcription factor
ASPSCR1-TFE3 altering the structure of the nuclear
membrane 18,19. Other rare features such as multinucleation
and pleomorphism have been observed in other studies
also but with no prognostic significance 2,3,12. Focal
mucinous and cystic change reported in the literature was
not seen in any of our cases.
Based on morphology, the differential diagnoses considered
in the present study were paraganglioma, granular
cell tumor, metastatic renal cell carcinoma, adrenocortical
carcinoma, hepatocellular carcinoma, rhabdomyosarcoma,
PEComa, and melanoma. Previously there was no specific
marker for diagnosis of ASPS but the discovery of an
unbalanced t(X::17) resulting in a fusion of the ASPL gene
on chromosome 17 to the TFE3 gene on chromosome X
changed this scenario 1,5. Recently, novel HNRNPH3-
TFE3, DVL2-TFE3, and PRCC-TFE3 fusions have also been
identified 6. Thus, immunodetection of the C terminus
of the TFE3 protein in ASPS was considered a diagnostic
landmark, but it should be interpreted carefully since the
list of tumors with TFE3 immunopositivity is increasing.
Cathepsin K is a cysteine protease abundantly expressed by
osteoclasts and its expression is driven by microphthalmia
transcription factor (MITF). TFE3 also belongs to the same
transcription factor subfamily as MITF. It is hypothesized
that the TFE3 fusion proteins function like MITF in the
neoplasms, and thus activate cathepsin K expression which
can be detected by IHC 20.
TFE3 rearrangements are not specific to ASPS but have also
been identified in a subset of PEComa and a Mit Translocation
renal cell carcinoma, both of which are morphological
mimickers of ASPS. TFE3 immunoreactivity is not
specific for TFE3 rearranged tumors, - Williams et al. have
documented TFE3 positivity in four cases of granular cell
tumors while Rekhi et al. observed TFE3 positivity in 28.5%
of granular cell tumor 2,3. Cathepsin K immunoexpression
is non-specific and has been reported in renal cell
tumors, granular cell tumors, as well as numerous additional
sarcomas including Kaposi sarcoma, liposarcoma, chondrosarcoma,
undifferentiated pleomorphic sarcoma, and
leiomyosarcoma 21. Granular cell tumors are diffusely
immunopositive for S100, SOX10 and inhibin, which are
negative in ASPS. There was focal weak S100 positivity in
one of our tumors. Cytoplasmic granules can be also seen in
granular cell tumor but PAS-positive diastase-resistant rodlike/
rhomboid crystalline inclusions seen in 36.4% of the
cases in the present series are specific for ASPS, and can be
highlighted with MCT1 and CD147 immunostains while
cytoplasmic granules in granular cell tumor are CD68 positive
1,3. Though TFE3 positivity have been reported in
paraganglioma but immunopositivity for neuroendocrine
markers, with S100 highlighting sustentacular cells, helps
differentiate them from ASPS 3. PAX8, pan cytokeratin,
CD10 negativity helps in ruling out renal cell carcinoma
which is further substantiated by the absence of a renal mass
on radiology. Negative immunostaining for vimentin and
Melan-A ruled out an adrenocortical carcinoma. S100-P,
HMB45, and Melan-A negativity in tumor cells ruled out
a melanoma. Focal desmin positivity was seen in two of
our cases but the lack of nuclear positivity for MyoD1 and
myogenin ruled out a rhabdomyosarcoma. PEComa is differentiated
from ASPS due to its reactivity for HMB45 but
recently aberrant expression of HMB45 was also reported
in ASPS, though both tumors are TFE3 rearranged, diagnosis
of ASPS was favored based on presence of PAS-D
needle crystals in ASPS 21. Translocation analysis can
be performed, when necessary, and is the diagnostic ‘gold’
standard but one should be aware of other TFE3 rearranged
tumors while interpreting the results 3,21.
None of our cases showed extensive mitosis or necrosis
which are considered classical features of high-grade sarcoma.
Despite thatbiological behavior of ASPS is aggressive,
hence FNCLCC Histological Grading System isn’t used for
them, all ASPS by definition are considered high grade 1.
The management of ASPS typically involves surgical resection
for localized disease, which was performed in 8 cases
and was curative. Anthracycline-based chemotherapy with
or without radiotherapy was given for disseminated tumors
with metastases in 10 cases and for localized disease in one
case. It was largely ineffective with no response in any case
and rather progression of disease was noted in all cases in
present series. A search for novel therapies and their evaluation
is being done in clinical trials. Molecular targeted
treatment has been increasingly utilized. Vascular endothelial
growth factor receptor-targeted TKIs such as pazopanib,
crizotinib, sorafenib, anlotinib, sunitinib, and cedirranib
and MET kinase inhibitors have been explored in clinical
trials for metastatic disease with promising results 5,22.
We argue against the future of immunotherapy in ASPS
since a very focal intratumoral inflammatory host response
was seen in only two cases and only one case showed minimal
lymphocytic response at the tumor edge.
ASPS has a morphological and immunohistochemical
overlap with many mesenchymal and non-mesenchymal
tumors. Diffuse strong nuclear TFE3 positivity is sensitive
for ASPS in an appropriate clinicoradiological context.
Awareness of TFE3 positivity in other tumors is vital. It is
imperative to employ a panel of markers in order to identify
an alveolar soft part sarcoma from its differential diagnoses.
Due to the high propensity for early metastasis even at the
time of presentation in ASPS, complete metastatic workup
and long term follow up is recommended. ASPS is associated
with slow progression and resistance to conventional
Ethics Approval and Consent to Participate
The study has been approved by the institute research committee of
GCRI assuring legal and ethical criteria fulfilment in the study with
review number IRC/2022/P-79.
Authors received no financial support for the research, authorship
and/or publication of this manuscript
Conflict of Interest
The authors declare that they have no competing interests.
Availability of Data and Material
Available on request from the corresponding author.
Concept: KK, Design: KK, Data collection or processing: KK,
Analysis or Interpretation: KK, AG, Literature search: KK, Writing:
KK, AG, Approval: AG, AS, JG, PT.
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