with Telangiatasia

OS-OM Combined Case Conference Pleomorphic Rhabdomyosarcoma

with Telangiatasia

報告醫師：孫章唐、 林育如 指導醫師 : 黃逸岳 、陳玉昆

94-06-23

Case Data

• Name : 鍾X X

• Gender : Male

• Age : 39

• Marital status : Yes

• Native : 屏東縣

• Occupation : 商

• Attending V.S. : 黃逸岳醫師

• First visit : 900614

Chief Complaint

• Swelling over right cheek and preauricular to temporal area for half a year.

Present Illness

• This 39 y/o male patient found swelling over right cheek and preauricular to temporal area for four years.

• 900614 He noted the swelling and mouth open limitation when he took food and felt discomfort over bilateral faces, so he

came to 屏基 H for help.

• Referred to KMU OM department for further exam.

– R/O sialolithiasis or TMD

– referred to OMS department --- op suggestion – patient think – ~901119 OPD F/U

• 9307 patient found the lesion got larger.

• 940226 came back to our OPD

Past History

• Past medical history

• Denied systemic disease

• Denied drug allergy

• Past dental history

• OD , prosthesis : acceptable

Personal History

• Oral habit : A(-)B(-)C(-)

• Occupation :

• Education :

• Marital status :

• Economic state :

• Psychic status :

Family History

• 家族無系統性疾病史

• No contribution to this problem

• Family support : well--住院期間將由父母及妻子 照顧

Review of System (nothing particular)

• Cardiovascular system

• Central nervous system

• Respiratory system

• Digestive system

• Endocrine system

• Hematopoietic system

• Genitourinary system

• Bone and joint

Physical Examination

( Essentially normal )

• Conscious : clear , vital sign : stable

• Head & Face : asymmetry

• Eye : conjunctiva : not pale sclera : not icteric, EOM : normal

• Ear : no hearing impairment

• Nose : patent

• Throat : no discharge, tonsil : not injected

• Neck : LAP (-), JVE (-), supple, free extension

• Chest : symmetric expansion H.S. : no murmur, RHB B.S. : clear

• Abd. : soft, no palpable mass, normative bowel sound

• L/L : free extension, no pitting edema

• CN12 : N.P.

Lab. Data

( Essentially normal )

( Essentially normal )

• RBC : 4.17 x10

/ul PLT : 26.2 x10

/ul

• WBC : 4.31 x10

/ul HGB : 12.4 g/dl

• HCT : 38.8%

• PT: 11.1/ 11.1 PTT :34.2/ 27.5

• GOT / GPT : 23/23

• BUN / Cr. : 20/1.0

• NA / K / Cl : 142 / 3.6 / 108

OMF Examination

• Swelling mass over right temporal region

• Soft to rubbery in consistency

• Size : 4.5 x 4.0 x 1.5 cm

• Pain ( + )

• Tenderness (-)

• LAP (-)

• MMO : 43mm from 11 to 41

• TMJ function : OK

Image 940503

Image Findings – CT scan940304

Image Findings – CT scan

• 1) Prominent soft tissue involving right

infratemporal fossa and right periauricular corresponding destruction and involving of right temporal joint.R/O metastasis,

recommend biopsy.

• 2) Retention cysts in bilateral maxillary sinuses.

• 3) Bilateral ethmoid sinusitis.

Image Findings – MRI 940314

Image Findings – MRI

• 1) Interval enlargement of the soft tissue mass lesion involving right infratemporal fossa and right periauricular region with cystic component and adjacent bony remodeling of temporal bone.

Interval development of hemorrhage in the cystic component of lesion.

– D/D: hemangioma and metastasis.

– Recommend biopsy for further evaluation.

• 2) Retention cysts in the bilateral maxillary sinuses.

Other Survey

• CXR : no active cardiopulmonary disease

• Bone scan : low probability of local bone invasion from parotid gland tumor

• SCC marker : pending

Impression and D.D.

• Pleomorphic adenoma near right parotid gland

• Warthin’s tumor

• Hemagioma

• R/O Malignant tumor

Treatment Plan

OP :

Wide excision + bone trimming

Treatment Course

• 940510 Admission

• GA and ENT dept. -- consultation

• OP on 940512

OP Procedure

• Preauricular incision was made then dissected to expose the lesion

• Well encapsulated at superficial portion

OP Procedure(cont.)

• Aspiration : yellowish fluid followed by dark reddish fluid

• Ruptured during dissection : dark reddish jelly- like mass

OP Procedure (cont.)

• Bony destruction of zygoma, pterygoid plate, zygomatic arch

• Bone trimming was done

• Closed the wound layer by layer after placed of penrose

Histopathological

findings

• Infiltrative margin

• Small blue polygonal tumor cells with eosinophilic cytoplasm

• Prominent pleomorphism,

hyperchromatism and abnormal mitosis

• Numerous multinucleated giant cells

• Chondroid, myxoid substance and metaplastic bone formation

• Numerous small blood vessles

Masson tricon Brown S-100 -

PTAH + Desmin -

CK - Myogenin +

Vimentin + Myoglobin +

CD31 - Alcian blue +

CD34 - CD68 -

EMA - P53 Focal +

SMA - Ki67 Focal +

Classification of rhabdomyosarcoma

•Embryonal ^(ERMS)

•Alveolar ^(ARMS)

•Pleomorphic ^(PRMS)

Morphologic variants of PRMS

• Classic

• Round-cell

• Spindle-cell

Histopathological diagnosis

Pleomorphic

rhabdomyosarcoma with telangiatasia

Right pre-auricular area

Treatment Course (cont.)

• Post-OP ^:

– No significant complaint

– No evidence of facial nerve injury – Reinforce change dressing

• 940519 - Discharged

Diagnosis of Surgical Specimen

• Pleomorphic Rhabdomyosarcoma with

Telangiatasia

Treatment Course (cont.)

• OMS OPD F/U

• 940525~0605 chemotherapy 血液腫瘤內 科

‧Dacarbazine + Epirubicin + Ifosfamind +Mesna

術後一個月

術前

Discussion

Introduction

• A malignant neoplasm of skeletal muscle origin

• 8~19% of all soft tissue sarcoma

• 35~45% occur in head and neck

• Primary in children and young adults

• Most cases arising in patient under 12 y/o and 43% seen in patient less than 5 y/o

• The most common sarcoma in children

Clinical features

• A painless, infiltrative mass that may grow rapidly

• In the head and neck region, the

orbit is the most frequent location, followed by the nasal cavity and

nasopharynx.

• The palate is the most frequent intraoral site.

• 60% occur in male

ERMS ARMS PRMS Incidence

of RMS 60% 20~30% <5%

Age < 12 y/o 15~25 y/o 40~60 y/o Occur in

head and neck

70~90% 18% 7%

Regional

nodal meta 10~38% 33%

Distant:

75~85%

9%

Image features

• A densly cellular infiltrating neoplasm

• Skeletal muscle is always seen as the site of origin

• On both CT and MRI, masses are typically homogeneous, with

destruction of adjacent bone.

• Necrosis can be seen, but

intratumoral hemorrhage is uncommon

and calcification is typically absent.

Image features

• Tumor is isointense to muscle on T1- weighted images and hyperintense on T2-weighted images.

• Just over half of the cases enhance homogeneously, and the degree of

enhancement is similar to that of normal skeletal muscle.

• In most cases, tumor margin are

poorly-defined.

Histopathological features

EMRS

• Small round or oval cells with

hyperchromatic nuclei and indistinct cytoplasm.

• Resemble the developing muscle cells of 6~8 w/o fetus.

• Cross striations are rarely found

Histopathological features

AMRS

• Aggregates of poorly differentiated round to oval cells separated by

fibrous septa.

• Resemble the developing muscle cells of 10 w/o fetus.

• Mitoses are common, and

multinucleated giant cells are also may

be seen.

Histopathological features

PMRS

• Classic: sheets of large, atypical and often

multinucleated polygonal

pleomorphic rhabdomyoblasts

Histopathological features

PMRS

• Round cell: clusters of large, atypical PRMB throughout the lesion with a back-ground of slightly pleomorphic medium- sized, round, blue

rhabdomyoblasts

Histopathological features

PMRS

• spindle cell: a predominance of pleomorphic spindled RMB

arranged in a storiform

growth pattern with scattered

large PRMB

Immunoprofiles of PRMS

• At least one skeletal muscle-specific marker positive (myoglobin, nuclear MyoD1, nuclear myf4, and fast

myosin)

• Myoglobin (95%) and fast skeletal

muscle myosin (80%) appeared to be

the most sensitive markers for PRMS.

Related article

• Pleomorphic rhabdomyosarcoma, classic variant, with reactive

osteoclast-like giant cells ⁽

)

• Infiltrative margin

• Small blue polygonal tumor cells with eosinophilic cytoplasm

• Prominent pleomorphism,

hyperchromatism and abnormal mitosis

• Numerous multinucleated giant cells

• Chondroid, myxoid substance and metaplastic bone formation

• Numerous small blood vessles

Histopathological features of

the present case

Discussion

• general feature

– (1) the most common soft tissue sarcoma of children ( > 50

% of pediatric sarcoma )

– (2). > 250 new cases in USA

– (3) 25 % of pediatric rhabdomyosarcoma 發生在head and neck

• 以 soft palate 最多，其次為 alveolar ridge ( maxilla > mandible )

• 亦可出現在無肌肉處，如 gingiva.

• 常在初診斷即已 meta.

Discussion (cont.)

• age: 10 歲以下最多

• painless muscle

• if masticatory muscle s are involved Æ trismus is a common finding

• infratemporal fossa and orbital are common

sites

Discussion (cont.)

• prognosis:

– (1) 1970, 5-year overall survival rate < 10 % – (2) 1991, 5-year overall survival rate =70 %

– (3) 早期診斷加上combination therapy Æ 80 % – (4) 50 %

Discussion (cont.)

• 治療

– 先看是否有轉移

– 看 resectability of the tumor

• Group I: localized disease, completely resected Æ surgery only

• Group II: compromised or regional resection ( microscopic residual tumor ) Æ surgery + 45 Gy of radiation o the tumor base

• Group III incomplete resection Æ surgery + 50-55 Gy of radiation o the tumor base

• Group IV : distant metastasis – multiagent chemotherapy

Discussion (cont.)

• Chemotherapy

– multiagent chemotherapy: decrease toxicity and improved survival

– Ex :

‧Dacarbazine + Epirubicin + Ifosfamind +Mesna

Discussion (cont.)

• adult

– rare case

– alveolar type 為主

– similar protocol of children – surgery + RT + chemotherapy – 5 year survival rate : 20 %

Thanks for your attention!