Subcutaneous Leiomyosarcoma Metastasized to the Lymph Nodes Involved with Small Lymphocytic Lymphoma / Chronic Lymphocytic Leukemia
Ayfer KAMALI POLAT1, Atilla SORAN2, Amal KANBOUR-SHAKIR3, Howard EDINGTON2
1Department of General Surgery, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY
2Departments of Surgical Oncology, Pittsburgh University, UPMC, Pittsburgh PA, USA
3Departments of Pathology, Pittsburgh University, UPMC, Pittsburgh PA, USA
Keywords: Leiomyosarcoma, Chronic lymphocytic leukemia, Lymph node, Metastasis
Herein, we present a case of a 76-year-old Caucasian man with
a very large fungating, ulcerating mass, involving the right neck
and parotid area, which developed while he was being treated for
chronic lymphocytic leukemia/small lymphocytic lymphoma.
Resection of the fungating right neck tumor, right modified radical
neck dissection, and right superficial parotidectomy with flap
reconstruction were performed. The final pathological diagnosis
was high-grade leiomyosarcoma of the skin and the subcutaneous
tissue, with invasion into the skeletal muscle, skin, and soft tissue.
Additionally, the sarcoma had metastasized to the lymph nodes
that were involved diffusely by lymphoma. The most interesting fact
for this case is coincidence of three rare occurrences which were
soft tissue sarcomas of subcutaneous leiomyosarcoma form and its
metastasis to same lymph nodes that were involved with lymphoma.
Leiomyosarcomas (LMS) are rare malignant tumors originating
in the smooth muscles, and constitute 3–7% of all
soft tissue sarcomas1-3
. The LMS arising in the dermis
and subcutis are referred to as cutaneous and subcutaneous
. LMS usually appear as solitary
nodules and commonly occur on the lower extremities (50–
70%), followed by the upper extremities (20–30%), trunk
(10–15%), and head and neck (1–5%). Soft tissue sarcomas
of the head and neck in adults are rare. They account for approximately
10% of all soft tissue sarcomas and less than 1%
of all head and neck tumors1
. In this report, we present a
case of two different malignancies in the same lymph node,
in addition to the rare localization of LMS.
A 76-year-old Caucasian man with chronic lymphocytic
leukemia/small lymphocytic lymphoma (CLL/SLL) was
being treated with two cycles of chemotherapy. During
treatment, an enormous fungating 12,0 cm tumor covering
much of the right auricular and upper neck area with
black ulcerated nodules, which caused bleeding at the
postauricular area, had developed. He had palpable lymph
nodes on both sides of his cervical area, bilateral bulky
nodes on the axillary areas, as well as trochlear nodes and
groin lymph nodes. Right axillary lymph node excisional
biopsy disclosed small lymphocytic lymphoma /chronic
lymphocytic leukemia (CLL/SLL); and cells positive for
CD20, CD5, CD23, and CD38; negative for CD3, CD10, Cyclin D1and ZAP-70. Bone marrow aspirate and biopsy
disclosed SLL/CLL/SLL without any phenotypic evidence
of transformation to a higher grade lymphoid neoplasm.
Cytogenetics disclosed 80% abnormal B cells. Fluorescence
in situ hybridization results revealed a karyotype of 43XY
with monosomy 13 and absence of MYB (myeloblastosis)
gene deletion, loss of ATM (ataxia telangiectasia mutated),
p53 (protein 53) tumor gene suppression, trisomy 12, and
IgH (immunoglobulin heavy locus1) gene rearrangement,
which is equal to no abnormality of chromosome 17. This
pattern, in general, suggests a favorable prognosis for
CLL/SLL. Fluorodeoxyglucose (18F, FDG) scan of several
bulky FDG-avid cervical nodes were obtained. Within the
subcutaneous soft tissue of the posterior neck, innumerable
enlarged bilateral axillary lymph nodes; mediastinal,
abdominal, inguinal, and internal and external iliac lymph
nodes; cardiophrenic and costophrenic lymph nodes; an
intermediate 4-mm right middle lobe pulmonary nodule;
and mild splenomegaly demonstrating increased metabolic
activity were seen. Positron emission tomographycomputed
tomography showed no evidence of malignancy.
The obtained findings gave an initial impression of locally
advanced skin cancer, melanoma versus squamous cell
carcinoma, but most findings were clinically closer to the
appearance of melanoma. The first biopsy was performed
using a 3-mm punch biopsy, which failed to confirm
the exact histology. Thus, a misdiagnosis of atypical
fibroxanthoma was made. Since this lesion was growing
despite chemotherapy with bendamustine and rituximab,
the diagnosis of the first biopsy was thought to be a sampling
error. At this point, surgical extirpation was recommended.
After 2 cycles of bendamustine and rituximab, resection of the fungating right neck tumor, right modified radical neck
dissection, and right superficial parotidectomy with flap
reconstruction were performed.
Histologically, a highly pleomorphic infiltrative neoplasm
with vague fascicular pattern that abutted but did not
involve the epidermis was observed. The tumor cells were
predominantly large, round to rhomboid with abundant
eosinophilic cytoplasm and large ovoid vesicular nuclei
with prominent irregular nucleoli (Figure 1A). Necrosis
with a mitotic rate of approximately 5/10 in high-power
fields was identified. Immunohistochemistry revealed
tumor cells to be strongly and diffusely positive for desmin
and caldesmon. CD68 was positive within intratumoral
macrophages. The tumor cells showed weak cytoplasmic
positivity for S100, but were negative for cytokeratin AE1/3,
AMA, CD34, tyrosinase, HMB45, and Melan A (Figure
1b). These findings supported the diagnosis of LMS.
Click Here to Zoom
|Figure 1: a) Leiomyosarcoma involving the skin (H&E; x2);
b) Upper (H&E; x20), lower right (desmin and caldesmon, x20),
lower left (CK AE1/AE3, x20).
The tumor size in greatest dimension was 12.0 cm,
followed by an additional dimension of 7.0 cm. The final
pathological diagnosis was high-grade LMS of the skin
and the subcutaneous tissue with invasion into the skeletal
muscle, as well as metastatic LMS in multiple lymph nodes.
The LMS metastasized to multiple lymph nodes that were
also involved with CLL/SLL (Figure 2A-C). The mitotic rate
was 5/10 in high-power fields. There was no macroscopic
necrosis but microscopic necrosis was present in 15%
of the nodes. The grade was set to be 3 according to the
French Federation of Cancer Centers Sarcoma Group. The
pathologic staging was pT2a, pN1 (3+/15) Mx, G3. The
margin was uninvolved by sarcoma, and the closest margin
was 0.1 cm.
Click Here to Zoom
|Figure 2: a, b) Metastatic leiomyosarcoma to lymph node involved
with CLL/SLL (right-H&E, x20; left-H&E, x4); c) Leiomyosarcoma
infiltrating skeletal muscle (H&E, x20).
Soft tissue sarcoma of the head and neck in adults is rare.
Sarcomas are characterized by local invasion and their
pattern of metastasis is mostly hematogenous. Lymph node
metastases are uncommon. The rate of regional lymph
nodes metastasis of LMS was reported to be 2.7–15%1-4
Few cases of synchronous lymph node involvement of different
kinds of tumors concomitant with lymphoproliferative
disorders or other malignancies have been reported in the
literatüre5-9. Most of them involve CLL/SLL and different
kinds of cancers such as cutaneous malignant neoplasm
expressing the Langerhans cell phenotype, dendritic cell
tumor, malignant melanoma, oropharyngeal squamous cell
carcinoma, colon carcinoma, and squamous cell carcinoma
of the uterine cervix6-8,10-13. Immunosuppression
may predispose CLL/SLL patients to a high risk of second
malignancies11,14. Irradiation, alkylating agent chemotherapy,
and antimetabolite use have been identified as risk
factors that potentiate second malignancies15,16. The
monoclonal antibody rituximab has been previously linked
to second malignancies17,18, and rituximab was one of
the medications of our patient. Congyang et al. reported a
case of synchronous histiocytic sarcoma (HS) and diffuse
large B cell lymphoma (DLBL) involving the stomach19.
According to immunohistochemical results of their study;
HS and DLBL co-expressed Oct-2, Bcl-2, and Bcl-6, they
suggest that HS may share a common origin with DLBL,
or be transdifferentiated from DLBL19. Bonetti et al. described
two distinctive malignancies, cutaneous neoplasm
and chronic lymphocytic leukemia, in a single lymph node
at the inguinal region8. Demellawy et al. reported a case
of synchronous malignant melanoma and CLL/SLL in the
same lymph node20. To our knowledge, this is the first
report of cutaneous LMS and CLL/SLL existing synchronously
at the same lymph nodes.
Accurate diagnosis and histological classification with
adequate sampling is critical for treating soft tissue tumors
as cytological and architectural features may be inadequate
to distinguish the different sarcomas, particularly if they are
poorly differentiated. In the present case, the initial clinical
impression was locally advanced skin cancer, leading to a
misdiagnosis of atypical fibroxanthoma based on punch
biopsy. The correct final diagnosis was made after wide
surgical excision and lymph node dissection.
In conclusion, we reported here our encounter with a case
of synchronous occurrence of three rare phenomena: soft
tissue sarcoma presenting as LMS, involvement of the head
and neck region and the metastasis of the leiomyosarcoma
to the same lymph node affected by lymphoma.
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